APPENDIX A Anesthesia Implications of Syndromes and Unusual Disorders 1

This Appendix contains brief descriptions of common and rare syndromes; their associated anesthetic considerations for safe pediatric anesthesia practice are outlined. Many of these shared features make precise identifi cation diffi cult; the reader should consider all the information provided for informing anes- thetic considerations. Whenever possible, the referenced literature should be con- sulted before anesthesia is undertaken. A brief list of several excellent resources that are up-to-date follows this introduction. In some rare syndromes, the only references are in foreign language journals; we have listed these when an English abstract was appended. Very occasionally, the reference refers to the disease in adults; we still include it when no published pediatric literature was available and where we thought that the adult information provided might be useful. Space limits a complete literature review for each condition, but we have attempted to list the most important and recent publications or case reports. There are now more than 10,000 medical syndromes recorded, so it is inevi- table that this list is incomplete. Although the number of syndromes has increased and existing syndromes have become better understood in part because of genetic studies, anesthesiologists may still encounter unreported dif- fi culties and complications. When in doubt as to the identity and implications of a particular syndrome, the anesthesiologist should make preparations that take into account all possible associated disorders. Recurring challenges that are common in many of these syndromes infl uence the choice of an anesthetic technique, specifi cally the diffi cult airway and con- genital heart defects. The reader is encouraged to consult the chapters that pro- vide approaches to management of the diffi cult airway and specifi c congenital heart defects, which may be adapted to the particular syndrome presented and in accordance with the practice and experience of the anesthesiologist. Those conditions that include impaired renal function require special care when administering radiologic contrast media. Classic descriptions of the clinical

1 Originally adapted from Jones EP, Pelton DA: Can Anaesth Soc J 23:207, 1976 and exten- sively augmented and revised.

© Springer International Publishing Switzerland 2016 523 J. Lerman et al., Manual of Pediatric Anesthesia, DOI 10.1007/978-3-319-30684-1 524 Appendix A: Anesthesia Implications of Syndromes… problems of a syndrome may allow the reader to decide when newer drugs and techniques can be used safely in individual cases.

References Kliegman RM, editor. Nelson textbook of pediatrics. 19th ed. Philadelphia: Elsevier- Saunders; 2011. http://www.nelsonpediatrics.com . Baum VC, O’Flaherty JE, editors. Anesthesia for genetic, metabolic, & dysmorphic syn- dromes of childhood. 2nd ed. Philadelphia: Lippincott, Williams & Wilkins; 2007. Bissonnette B, Luginbuehl I, Marciniak B, et al., editors. Syndromes: rapid recognition and perioperative implications. New York: McGraw-Hill Medical Publishing Division; 2006. Fleisher LA, editor. Anesthesia and uncommon diseases. 5th ed. Philadelphia: Elsevier- Saunders; 2005. Jones KL, Jones MC, Del Campo Casanelles M, editors. Smith’s recognizable patterns of human malformation. 7th ed. Philadelphia: Elsevier; 2013. Appendix A: Anesthesia Implications of Syndromes… 525 Table continues on the following page. continues on the following Table cation: A case report. J Anesth. Anesth. J A case report. cation: cult but usually is not. Tracheal Tracheal cult but usually is not. lish abstract]. Anaesthesist. Anaesthesist. lish abstract]. cult due to excess lax skin. High incidence of High incidence lax skin. excess cult due to Preoperatively check electrolytes and ensure that electrolytes and ensure check Preoperatively even administered are supplementary corticosteroids for surgeryif by (i.e., anesthesia is unaccompanied MRI or other investigations) Intubation may be diffi may Intubation and depth oftube size insertion best judged by are a smaller tube than weight (not age)—most require Caution with neck their age. by indicated is IV access extension. excessive movements—avoid diffi on in the sitting position when operated complications Anesthesia implications Caution with muscle relaxants and residual weakness. weakness. Caution with and residual relaxants muscle other special recommendations No cation broblastic broblastic uid (CSF) shunts sm. Defective fi Defective sm. child with Aicardi syndrome undergoing laparoscopic Nissen’s fundopli Nissen’s undergoing laparoscopic syndrome Aicardi withchild n therapy in congenital adrenogenital syndrome with salt loss. [German-Eng with syndrome salt loss. adrenogenital n therapy in congenital Most common form of common Most dwarfi syndrome and Saethre-Chotzen See Carpenter syndrome See Apert syndrome virilization of hydrocortisone; synthesize Inability to supplementation, steroid All need perioperative females. ifeven not salt losing See leukodystrophy growth factor 3 (FGFR3) at chromosome 4. Defective bone Defective 4. 3 (FGFR3) at chromosome growth factor formation with rate of decreased endochondral ossifi leads to shorter tubular bones. Foramen magnum or spinal Foramen shorter tubular bones. leads to brain to be related Sleep apnea may occur. may stenosis craniectomy, need suboccipital May compression. stem fl or cerebrospinal laminectomy, Description spasms. Marked myotonia and drowsiness. Repeated Repeated and drowsiness. myotonia Marked spasms. aspiration pneumonia and infantile chorioretinopathy, callosum, corpus Absent 2011;25:123–6 Name Achondroplasia 2003;13(6):547–9 Anaesth. Paediatr management ofAnaesthetic a patient with achondroplasia. G. Korula N, Eipe BS, Krishnan Acrocephalopolysyndactyly Acrocephalosyndactyly Adrenogenital syndrome substitutio Perioperative W. Petrykowski von M, Abel Adrenoleukodystrophy syndrome Aicardi 2007;17(12):1223 Anaesth. Paediatr syndrome. Aicardi with in a child Anesthesia J. Mayhew management ofAnesthetic a Y. Mizuno T, Miwa Y, Terakawa 1984;33(8):374–6 526 Appendix A: Anesthesia Implications of Syndromes… level ++ cult ow (HBF); (HBF); ow ciency) and vitamin K level le, diffi cult airway; have oxide (pneumocephalus) (pneumocephalus) oxide e Reports. 2012;pii: bcr2012006901. bcr2012006901. 2012;pii: e Reports. urane has least effect on HBF. Maintain Maintain urane has least effect on HBF. uid therapy uid Ensure that anti-seizure medications will be given the medications will that anti-seizure be given Ensure Check hemoglobin and Ca morning of surgery. Assess cardiac status (echocardiogram) preoperatively. status (echocardiogram) preoperatively. cardiac Assess profi coagulation Bilirubin, preoperatively. should be checked a regurgitation; encourages Hepatosplenomegaly be necessary prevent to induction may rapid sequence Caution with drugs the liver. aspiration. handled by drugs hepatic blood fl that decrease Avoid isofl Epidural HBF. preserve to volume intravascular but check opioids, over be preferred anesthesia may X-rays). (check anatomy and vertebral clotting state Caution with transport osteoporosis and positioning; (vitamin be present D defi may Check and correct electrolytes to normal values. Renal Renal normal values. electrolytes to Check and correct drugs and caution with excreted impairment; renally fl airway cart at hand. Nasal airway unreliable; may may unreliable; airway Nasal cartairway at hand. and airways prepare obstruct when anesthetized; mobility Limited N.B. laryngeal (LMA). mask airway nitrous Avoid of joints. preoperatively. Care in moving, positioning, and use positioning, in moving, Care preoperatively. diffi of Beware restraints. Anesthesia implications supplementation + eumocephalus may occur. occur. may eumocephalus Disorder of osteoclasts and bone overgrowth. Infantile Infantile ofDisorder and bone overgrowth. osteoclasts ofmalignant at less than 1 year form presents age with because of and seizures thrive hypocalcemia. to failure and obligate bossing, frontal macrocephaly, Lethargy, nasopharyngeal (overgrowth bone) are mouth breathing and pn Hydrocephalus common. marrow from Anemia pathologic fractures. Brittle bones, hepatosplenomegaly sclerosis; with alkali and K Treated calculi. renal (97 %), musculoskeletal (inc. vertebral), ocular, facial, and facial, ocular, vertebral), (inc. musculoskeletal %), (97 of presentation an Variable abnormalities. neurologic cases Severe dominant inherited condition. autosomal transplantation liver necessitate Description rickets, osteomalacia, hypokalemia, tubular acidosis, Renal

cardiac have of May Disorder the bile ducts with cholestasis. 10.1136/bcr-2012-006901 10.1136/bcr-2012-006901 Marshall L, Mayhew JF. Anesthesia for a child with Alagille syndrome. Paediatr Anaesth. 2005;15:256–7 Anaesth. Paediatr Alagille with for a child syndrome. Anesthesia JF. Mayhew L, Marshall disease Albers-Schönberg (marble bone disease; osteopetrosis) ofAdministration BMJ Cas a paediatric general anaesthesia to patient with et al. osteopetrosis. I, Demirel Erhan OL, AB, Ozer doi: Albright-Butler syndrome 2001;94(5):221–5 Soc Med. J Royal tubular acidoses. The renal Capasso G. RJ, Unwin Name Alagille syndrome Appendix A: Anesthesia Implications of Syndromes… 527 culty—but usually culty—but Table continues on the following page. continues on the following Table a. 2006;61(4):394–8 a. ure. Am J Med Gen. 1997;69(1):13–6 Gen. J Med Am ure. syndrome: case report. Sao Paulo Med J. J. Med Sao Paulo case report. syndrome: nesth. 2011;21:1269–70 nesth. Preoperatively check ECG and electrolytes. Avoid Avoid ECG and electrolytes. check Preoperatively hyperventilation and respiratory alkalosis (exacerbates QT ECG for an increased Monitor hypocalcemia). caution with Extreme defects. interval or conduction weakness possible residual relaxants, muscle Use caution with drugs excreted by kidneys. Check kidneys. by caution with drugs excreted Use occur defects may conduction AV ECG as and obesity Diabetes require function. Check liver by Caution with drugs excreted special consideration. Great assessment required. cardiac Echo kidneys. tachycardia caution with avoid inhalation agents; Assess airway, use in-line stabilization during airway, Assess for diffi prepare laryngoscopy, Rx recommended Antiemetic routine. Anesthesia implications ndings. Int J Cardiol. 2013;167(4):1257–63 J Cardiol. Int ndings. Ectopic bone formation, developmental delay. delay. developmental bone formation, Ectopic defects, possible ECG conduction Hypocalcemia: for present May convulsions. problems, neuromuscular cataract surgery puberty, hepatic dysfunction, and glomerulosclerosis. and glomerulosclerosis. hepatic dysfunction, puberty, cardiomyopathy Dilated function. renal liver Decreased Renal failure in second to third decade. May present for present May decade. third to in second failure Renal transplantation renal psychiatric symptoms, and impaired pulmonary and impaired function symptoms, psychiatric Short stiffand infections. unstable cervical spine Description abnormalities, and muscle skeletal delay, Developmental after diabetes hearing loss, 7 years, blindness by Obesity, See Wiskott-Aldrich syndrome syndrome Wiskott-Aldrich See See leukodystrophy leukodystrophy See pathology renal variable. and nerve deafness; Nephritis

(pseudohypoparathyroidism) (pseudohypoparathyroidism) Alpha-mannosidosis Name Albright hereditary osteodystrophy Anaesthesi a series of of presentation cases and an unusual three ocular tetany. Pseudohypoparathyroidism: Singh M. RA, Sunder Aldrich syndrome Alexander disease A for patients withAnesthesia alpha-mannosidosis a case series of Pediatr et al. 10 patients. J, Roed LG, Borgwardt P, Hallas Alport syndrome atrioventricular transplantation block duringAlport’s in a patient with renal Complete PT. Vianna P Jr, Nascimento Ferrari F, Alström syndrome case report and review of syndrome: Alstrom sibs with the literat in two dysfunction Hepatic et al. S, Sato T, Tanaka M, Awazu 2001;119(5):184–6 fi magnetic resonance cardiac syndrome: Alstrom et al. V, Bettini R, Razzolini Corbetti F, 528 Appendix A: Anesthesia Implications of Syndromes… cartcult airway release and cardiac and cardiac release + cult. Have diffi Have cult. 5):475 09–18 review. Eur J Pediatr. J Pediatr. Eur review. ):921–3 nsitive to thiopental, propofol, and respiratory propofol, thiopental, to nsitive at hand. Assess respiratory status. Observe respiratory precautions Assess status. at hand. succinylcholine Avoid for long Q-T syndrome. Possible airway problems; mask ventilation and mask ventilation problems; airway Possible be diffi intubation may Check baseline ventilatory status (spirometry). Do not status (spirometry). Check baseline ventilatory possible K use succinylcholine: Check coagulation factors; treat excessive bleeding excessive treat factors; Check coagulation Risk of under plasma. hypoglycemia with frozen fresh values and infuse dextrose glucose measure anesthesia; perioperatively depressants (due to reduced muscle mass). Avoid Avoid mass). muscle reduced (due to depressants unpredictable possible: where relaxants muscle non-depolarizing to relaxants response (e.g., propofol, thiopental, vecuronium) vecuronium) thiopental, propofol, (e.g., Anesthesia implications arrest. Use minimal doses of thiopental, propofol, and minimal doses of propofol, thiopental, Use arrest. and Rocuronium (cisatracuriumrelaxants preferred). Monitor also be used. may suggamadex respiratory Avoid blockade. neuromuscular regional analgesia Consider depressants. relative relative mandibular → mia biochemical and clinical implications. A case report and literature and clinical implications. mia biochemical ciency of glucosyltransferase Early (brancher enzyme). prognathism; abnormal structure and angleprognathism; of mandible kyphoscoliosis (triangular facies), Degeneration of motor neurons. Progressive muscular muscular Progressive Degeneration of neurons. motor aspiration to Prone and respiratoryweakness failure. pneumonia Defi splenomegaly; failure; hepatic cirrhosis; liver severe hemorrhagic tendency severe midfacial hypoplasia midfacial hypoplasia severe Anterior horn cell degeneration hornAnterior cell Se Description disease) See gangliosidosis GM2 (Tay-Sachs disease See Tangier

features; dysmorphic interval, long QT paralysis, Periodic (glycogen (glycogen of level low Extremely serum albumin (4–100 mg/dL) drugs albumin to that bind to sensitive Very

Amyotrophic lateral Amyotrophic sclerosis 2004;163:664–70 Koot BG, Houwen R, Pot DJ, et al. Congenital analbumine Congenital et al. DJ, Pot R, Houwen BG, Koot Analphalipoproteinemia disease Andersen disease typestorage IV) 2010;20( Anesth. Paediatr Disease IV. Storage with management in a child Anesthetic Glycogen JF. Mayhew V, Patel A, Armendi De syndrome Andersen 2005;15(11):1019–20 Anaesth. Paediatr syndrome. Andersen’s with for the child Anesthesia DA. Young (infantile muscular atrophy) atrophy) (infantile muscular Name familial idiocy Amaurotic congenita Amyotonia 2004;21(11 Anaesthesiol. J Euro General disease. anaesthesia in a patient with neuron motor et al. M, Lechner Lirk P, B, Moser Analbuminemia Prabhakar A, Owen CP, Kaye AD. Anesthetic management of the patient with amyotrophic lateral sclerosis. J Anesth. 2013;27(6):9 Anesth. J management of sclerosis. lateral Anesthetic the patient with amyotrophic AD. Kaye CP, Owen A, Prabhakar Appendix A: Anesthesia Implications of Syndromes… 529 ed C1 ed treatment uid status, Table continues on the following page. continues on the following Table . 2012;34:623–30 . –11 1):50–5 cult airway. Prophylactic Prophylactic cult airway. ) and muscle relaxants (myopathy). Propofol Propofol (myopathy). relaxants ) and muscle αβ Check complement assay, Hct, fl Hct, assay, Check complement history, previous drug reactions. Note voice change or change voice Note previous drug reactions. history, for dental (e.g.., Prophylaxis dysphasia. (C1INH) inhibitor C1 esterase manipulation): Otherwise therapy should be used. fresh replacement Continue preoperatively. plasma for 1–3 days, frozen Danazol and postoperatively. IV perioperatively EACA epinephrine, attack: Acute is useful. (androgen) antihistamine (in case diagnosis is a true steroids, or purifi plasma, frozen fresh anaphylaxis), If pharyngeal tracheal edema develops: inhibitor. if for 24–72 h); in place this is not intubation (leave anesthesia Regional perform tracheotomy. possible, when care extreme Otherwise, when possible. instrumenting airway Usually uncooperative. Give anti-seizure medications anti-seizure Give uncooperative. Usually Caution with IV hypnotics on the morning of surgery. (GABA Anesthesia implications may be useful. Normal response to inhaled agents and to response Normal be useful. may diffi Possible opioids. limit vagal overactivity anticholinergic to edema. edema. → Movement toward an international treatment consensus. Clin Ther treatment an international consensus. toward Movement with hereditary angioedema. Allergy Rhinol. (Providence.) 2015;6( (Providence.) Allergywith Rhinol. hereditary angioedema. subunit receptors may alter alter may subunit receptors β A increased vascular permeabilityincreased → brinolytic and hormonal agents ciency of C1 esterase inhibitor, reduced to 20 % of 20 to reduced ciency of C1 esterase inhibitor, Usually painless; may have prodromal focal tingling or prodromal have may painless; Usually trauma May by or vibration. induced Often “tightness.” bouts of diarrhea; abdominal pain, have shock, hypotension, leading to hemoconcentration deaths Most pharyngeal slowly). edema (usually develops Treatment %. laryngealfrom mortality edema; 33 rate up to with antifi response to anesthetic drugs. Vagal hypertonia Vagal anesthetic drugs. to response Episodic brawny edema of extremities, face, trunk, airway airway trunk, face, edema of brawny Episodic extremities, on Mutation 1 week. lasts 4 h to abdominal viscera, Onset in childhood 11 responsible. chromosome Etiology: idiopathic form. (1) this from differentiates defi normal or (2) normal of levels levels type dysfunctional of of vasoactive Accumulation inhibitor. C1 esterase substances ataxia, seizures, paroxysmal laughter, muscle atrophy. atrophy. muscle laughter, paroxysmal seizures, ataxia, % of in 75 15q chromosome Genetic defect in maternal cases affecting GABA Description drooling, craniofacial anomalies, delay, Developmental (hereditary Williams AH, Craig TJ. Perioperative management for patients Perioperative TJ. Craig AH, Williams Name syndrome Angelman 2014;26:75–9 Anesth. J Clin syndrome. Angelman in the patient with considerations Perioperative AD. Kaye Bevinetto CM, Angioedema edema) angioneurotic 1989;71(2):309 A reviewAnesthesiology. of 25 patients with M. hereditary Hahn angioedema requiring surgery. M, Frank RT, Wall Riedl M. Hereditary angioedema therapies in the United States: States: angioedema Hereditary therapies in the United Riedl M. 530 Appendix A: Anesthesia Implications of Syndromes… cult cult supraglottic have cult; cartcult airway available. syndrome. Paediatr Anaesth. Anaesth. Paediatr syndrome. diatr Anaesth. 2011;21(1):72–7 diatr Anaesth. because of narrowed nasal passages. Ensure that a leak Ensure because of nasal passages. narrowed be ICP may the tracheal tube. around is present of High incidence respiratory increased. ifcomplications—caution history of URI recent Mask anesthesia may be diffi anesthesia may Mask intubation is almost Orotracheal devicesairway ready. be diffi intubation may Nasotracheal easy. always Potential respiratory and diffi problems Potential intubation. Have the diffi Have intubation. Extremity with deformitiesCare may positioning. easy vascular access preclude Anesthesia implications Preoperatively check serum electrolytes check if chronic Preoperatively Caution with excreted renally present. failure renal during Hyperkalemia surgery drugs. can produce treatment necessitating ECG changes Developmental delay. Hypoplastic maxilla and Hypoplastic delay. Developmental possibly with Craniosynostosis, Syndactyly. exophthalmos. be may Trachea fused cervical vertebrae. ICP; increased be CHD may with fused ringsnarrow (“bamboo trachea”) present See Christ-Siemens-Touraine syndrome syndrome See Christ-Siemens-Touraine abnormalities: craniosynostosis, midface hypoplasia, hypoplasia, midface craniosynostosis, abnormalities: cardiac, have May and joint contractures. atresia, choanal Respiratory abnormalities. and renal gastrointestinal, (including early intervention require obstruction may major cranial surgery Need to in neonates tracheostomy). craniosynostosis relieve Description and psychomotor retardation. Renal dysfunction or failure or failure dysfunction Renal retardation. and psychomotor hepatic failure also have May because of kidneys. polycystic syndrome See Klippel-Trénaunay-Weber and cartilaginous with bony condition Recessive ofMalformation the brainwith amaurosis stem congenital

syndrome See Marfan

Anhidrotic ectodermal Anhidrotic dysplasia Name Angio-osteohypertrophy 2007;17(5):497–8 Anaesth. Paediatr syndrome. Antley-Bixler for an infant withAnesthesia J. Mayhew D, Boswell Apert syndrome (acrocephalosyndactyly) a review of Pae Apert with syndrome: 509 anesthetics. in children complications Perioperative Bingham R. C, Moloney Barnett S, Arachnodactyly Arima syndrome hyperkalaemiaArima in an infant with QRS-interval caused by changes Intraoperative et al. H, Nemoto K-I, Nishikawa S, Koizuka 1998;8:425–8 Antley-Bixler syndrome syndrome Antley-Bixler Appendix A: Anesthesia Implications of Syndromes… 531 required; muscles required; Table continues on the following page. continues on the following Table tomy. J Anaesth Clin Pharm. Clin Pharm. Anaesth J tomy. surgery. [Japanese] English [Japanese] abstract. surgery. cartcult airway available. may be required in post-op period be required may Anesth. 2012;22:256–62 Anesth. 2 problem cult intubation and airway Check Hb and Hct levels and pulmonaryCheck Hb and Hct levels function as if antibiotic prophylaxis Use anemia. Treat needed. isolation). (reverse sterile technique Use indicated. O Supplemental Minimal Minimal thiopental/propofol Sensitivity non-depolarizing to muscle fat. by replaced Diffi relaxants. because of limitation of temporomandibular diffi have movement; often temperature in body and increase Tachycardia MH). to (not prone observed for unclear reasons for cooling and be prepared temperature body Monitor consider be used, analgesia may Regional measures. ultrasound guidance Anesthesia implications Preoperatively assess cardiac status (echocardiogram), status (echocardiogram), assess cardiac Preoperatively if use sterile technique, indicated; SBE prophylaxis Do not use cardiodepressants; isolation. reverse recommended and fentanyl midazolam, ketamine, rioperative risk in the patient with Ataxia-Telangiectasia. Pediatr riskAtaxia-Telangiectasia. rioperative in the patient with recurrent pulmonary recurrent infections; and sinus → bronchiectasis. Severe anemia may be present. RES be present. anemia may Severe bronchiectasis. % of cases malignancy in about 10 about 10 % of cases. Intraoperative tachycardia, tachycardia, % ofIntraoperative cases. about 10 but and hyperthermia occur may hypermetabolism, for MH without or genetic markers classic biochemical decreased serum IgA or IgE. Defective Defective or IgE. serum IgA decreased immunity Description complex cardiovascular anomalies (i.e., single ventricle); anomalies (i.e., cardiovascular complex Increased cases. and heart in many cyanosis failure infection overwhelming susceptibility to stiffness of CHD in joints; contractures, congenital Multiple telangiectasia; skin and conjunctival ataxia, Cerebellar Very malposition of spleen; Absent abdominal organs. Name Arthrogryposis multiplex management ofAnesthetic with a neonate arthrogryposis KK. Asim for emergency laparo congenita multiplex S, Samui R, Chowdhuri syndrome Asplenia management ofAnesthetic an infant with a single for noncardiac Okuda C et al. (asplenia syndrome) ventricle T, Ando K, Uchida telangiectasia Ataxia and pe Anesthetic et al. Bembea M, AJ, Iskander Lockman JL, 2011;27(2):244–6 1992;41(11):1793–7 Masui. 532 Appendix A: Anesthesia Implications of Syndromes…

uid status. status. uid 10.1155/2010/239239 d epiglottis d 1973;126:119–26 :1131–6 : 239239. doi: doi: 239239. : cult airway cart available. IV access IV access cartcult airway available. cult to correct. Hemodynamic instability; invasive instability; invasive Hemodynamic correct. cult to diffi have cult; Preoperatively assess cardiac status (echocardiogram), status (echocardiogram), assess cardiac Preoperatively and fl endocrine, (BUN/creatinine), renal material contrast Use if indicated. SBE prophylaxis bifi have May with caution. electrolyte abnormalities Check acid-base status: diffi to attention Careful be indicated. monitoring may anesthesia is Regional status. electrolytes and volume suitable be very and intubation may maintenance Airway diffi Caution with skin changes. by be complicated may use in-line stabilization if indicated. neck movement; with the eyes close or ointment and tape to Protect postoperatively ventilation Monitor shields. use eye Anesthesia implications hild with Bardet-Biedl syndrome. Case Reports in Medicine. 2010;pii in Medicine. Case Reports syndrome. hild with Bardet-Biedl of a patient with Beare-Stevenson syndrome. Paediatr Anaesth. 2005;15(12) Anaesth. Paediatr of syndrome. a patient with Beare-Stevenson obesity, hypogenitalism, diabetes, and hypertension. and hypertension. diabetes, hypogenitalism, obesity, is syndrome, typical(Spastic paraplegia, in Laurence-Moon heart abnormalities and congenital renal have absent.) May defects Chloride reabsorption but hypovolemic. Normotensive defect with cell urinary Juxtaglomerular potassium loss. prostaglandin hyperaldosteronism, hyperplasia, of and activation the renin-angiotensin- overproduction system aldosterone cutis gyratum, and cleft palate, atresia, choanal proptosis, cervicalAssociated spine and foramen abnormal genitalia. magnum abnormalities Description hydrocephalus, withCraniosynostosis cloverleaf skull, polydactyly, pigmentary retinopathy, delay, Developmental metabolic alkalosis. hypochloremic Hypokalemic, Name syndrome Bardet-Biedl J Dis Child. Am of less than 3 years age. children of Report unrelated two syndrome. Laurence-Moon-Biedl GR. Hogan Bauman ML, general anaesthesia for a c care Day S. Crowe S, Chittoodan syndrome Bartter’s 1995;42:808–12 Anaesth. Can J syndrome. management ofAnaesthetic with a child Bartter’s HS. Moseley Y, Delph S, Kannan syndrome Beare-Stevenson care Perioperative JD. Tobias M, Bothwell S, Upmeyer Appendix A: Anesthesia Implications of Syndromes… 533 Table continues on the following page. continues on the following Table cult because of scarring in 14;30:562–4 –5 cart cult airway available cult intubation because of have large tongue; be large for may Trachea cartcult airway available. mandible may cause airway problems perioperatively perioperatively problems cause airway mandible may and postoperatively Preoperatively assess cardiac status (echocardiogram) status (echocardiogram) assess cardiac Preoperatively and problems Airway rule out CHD. to in neonate diffi diffi blood Monitor age—use a cuffed tracheal tube. slow by hypoglycemia and treat frequently glucose cause rebound infusion of (bolus dose may dextrose useful for airway Nasopharyngeal hypoglycemia). require May obstruction. airway postoperative high hematocrit reduce to phlebotomy Preoperatively assess cardiac status (echocardiogram status (echocardiogram assess cardiac Preoperatively sterile Use involvement. rule out cardiac and ECG) to history of therapy; have steroid May technique. Tracheal be very poor. nutritional status may be very diffi intubation may diffi pharynx; have Anesthesia implications urgically of maxilla and wiringAdvancement of maxilla and rst sign; may extend to to extend rst sign; may cult intubation in a patient with Behcet’s disease. Anaesthesia. 2002;57(6):620–1 Anaesthesia. disease. cult intubation in a patient with Behcet’s cult airway in a child with Beckwith-Wiedemann syndrome. Paediatr Anaesth. 2009;19(7):696–8 Anaesth. Paediatr syndrome. with in a child cult airway Beckwith-Wiedemann See Duchenne muscular dystrophy dystrophy muscular See Duchenne genetic defect with disease caused by variableRare Birth than 4000 g, weight greater inheritance patterns. Omphalocele, and exophthalmos. macroglossia, umbilical hernias, hyperviscosity syndrome, visceromegaly, common are and hypoglycemia heartcongenital disease, be associated, may Cleft palate (see neonatal hypoglycemia). to be indicated may reduction tongue and if this is repaired, obstruction airway severe prevent esophagus) and genital area; uveitis, iritis, conjunctivitis, conjunctivitis, iritis, uveitis, esophagus) and genital area; vasculitis, have May arthritis. nonerosive skin lesions, risk of of sepsis at sites and CNS involvement; myocardial, etc. skin punctures, Description culties associated with Behçet’s syndrome. Br J Anaesth. 1972;44:100–2 Anaesth. J Br syndrome. withculties associated Behçet’s

s be corrected may if dysplasia; severe, Maxillonasal of ulceration Gross mouth (usually fi Batra M, Valecha UK. Anesthetic management of tongue reduction in a case of Beckwith-Wiedemann syndrome. J Anes Clin Pharm. 20 Clin Pharm. Anes J management ofAnesthetic in a case of reduction syndrome. tongue Beckwith-Wiedemann UK. Valecha Batra M, Name Becker syndrome Beckwith syndrome 1996;6:231–3 Anaesth. Paediatr syndrome. and the Beckwith-Wiedemann Anaesthesia et al. JL, Garcia-Perla Ojeda R, C, Suan 2008;22(1):93 Anesth. J management ofAnesthetic cases of two syndrome. Beckwith-Wiedemann et al. S, Kimura KamadaY, Y, Kimura syndrome Behcet diffi Anaesthetic ME. Turner syndrome Binder 2012;23(4):986–90 Surg. J Craniofac syndrome. Binder et al. M, Nugent NR, McLean S, Chummun (Beckwith-Wiedemann (Beckwith-Wiedemann infantile syndrome, gigantism) for management of GlideScope the diffi JB. Tuchman R, Atiles J, Eaton diffi after Oral aphthous ulcers et al. S, Demiroluk Y, Dikmen Salihoglu Z, 534 Appendix A: Anesthesia Implications of Syndromes… cult airway airway cult cartcult airway available. g. 2007;104(6):1348–9 g. ediatr Anaesth. 1996;6:65–8 ediatr Anaesth. ann-de Lange syndrome. Paediatr Paediatr ann-de Lange syndrome. urane administration urane Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively artery Left as for coronary Anesthesia disease. aggressive require may dysfunction ventricular CPB from wean therapy to Monitor heart rate. Episodic bradycardia requiring bradycardia Episodic heart rate. Monitor during occur or epinephrine may atropine sevofl Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively anemia and treat studies preoperatively; Coagulation additional steroids. Give available. platelets have ofConsiderations hemochromatosis status (echocardiogram), assess cardiac Preoperatively diffi anticipate pulmonarycheck status, diffi have and intubation; if indicated SBE prophylaxis Anesthesia implications frequent ux and aspiration leads to stulae, hearing loss, pre-auricular pits, pits, pre-auricular hearing loss, stulae, hocardiography in adult-type Bland-White-Garland syndrome. Anesth Anal Anesth in adult-type syndrome. hocardiography Bland-White-Garland e-Garland syndrome: clinical picture and anaesthesiological management. Pa and anaesthesiological management. clinical picture e-Garland syndrome: urane in siblings with Branchio-oto-renal syndrome. Paediatr Anaesth. 2007;17(1):80–3 Anaesth. Paediatr urane syndrome. in siblings with Branchio-oto-renal Coronary artery malformation with arising left coronary leading to ischemia the pulmonary Myocardial from trunk. if Lethal early heart not corrected acute failure. Branchial cysts or fi Branchial abnormalities renal ear malformations, external Congenital idiopathic RBC aplasia. Liver and spleen Liver idiopathic RBC aplasia. Congenital Craniofacial thrombocytopenia. hypersplenism, enlarged: with steroids Treatment be present. defects may and cardiac develop. may transfusions; hemochromatosis and repeated Increased be successful. transplant may Bone marrow ofincidence malignancy (leukemia) and GI cardiac with craniofacial, delay Developmental and strabismus. hirsutism, malformations, refl Gastroesophageal pulmonary infections Description (BOR) (BOR)

syndrome See cerebrohepatorenal

(Melnick-Fraser (Melnick-Fraser Bland-White-Garland Bland-White-Garland syndrome Minkovich LL, Brister SJ, Slinger PD. Transesophageal ec Transesophageal Slinger PD. SJ, Brister LL, Minkovich syndrome syndrome) Name Blackfan-Diamond syndrome The Bland-Whit G. Molter V, Grueness S, Kleinschmidt syndrome Bowen Lange Brachmann-de syndrome management withAnesthetic laryngeal with mask in a child Brachm et al. A, Gutierrez-Jodra T, Mencia Perez R, Fernandez-Garcia Branchio-Oto-Renal with sevofl Bradycardia NC. Wilton MH, Taylor Anaesth. 2006;16(6):698–700 Anaesth. Appendix A: Anesthesia Implications of Syndromes… 535 channels (local channels cartcult airway + Table continues on the following page. continues on the following Table 23(3):210–4 thorac Vasc Anesth. 2013;27(6):1334–6 Anesth. thorac Vasc nesth Soc J. 2004;51(9):946–7 nesth Soc J. ndrome: a retrospective database a retrospective ndrome: diffi cult airway; have brillator pads intra-op. Monitor ECG Monitor brillator pads intra-op. available. Care with Caution Care moving and positioning. available. with relaxants Possible Possible diffi Preoperatively assess cardiac status (echocardiogram), status (echocardiogram), assess cardiac Preoperatively Caudal epidural anesthesia for arrhythmias. monitor during surgery general anesthesia for noncardiac has successfully been employed Anesthesia implications Avoid parasympathetic stimulation (give parasympathetic (give stimulation Avoid agents) and caution with reversal anticholinergic, caution with drugs that affect Na analgesics). Thiopental, propofol, and inhaled agents propofol, Thiopental, analgesics). Apply normothermia. Maintain OK. probably defi as indicated postoperatively a patient with Cantrell’s pentalogy diagnosed prenatally. Can A Can pentalogy diagnosed prenatally. a patient with Cantrell’s administration of in patients with propofol established Brugada sy of a 7-year-old child with Brugada syndrome. J Intens Care Med. 2008; Med. Care J Intens with child of Brugada syndrome. a 7-year-old channel defect in the myocardium. ST defect in the myocardium. channel brillation + Cortical thickening of long bones and skull. Skeletal pain, pain, ofSkeletal Cortical thickening long bones and skull. and cranial nerve compression. weakness, muscle fractures Pathological Results from Na Na from Results umbilicus, agenesis of pericardial and diaphragm, umbilicus, sternum septal and cardiac malformations: defect and cardiac respiratory severe develop to Prone defects present. valvular and hypoxemia distress Description segment elevation in precordial leads and incomplete RBB leads and incomplete segment elevation in precordial ventricular to with Prone normal heart. anatomically and fi tachycardia Asia. in Southeast common but more in Occidentals Rare of the muscles Defect in the recti the abdominal wall above See leukodystrophy leukodystrophy See (progressive (progressive Flamee P, De Asmundis C, Bhutia JT, et al. Safe single-dose et al. JT, Bhutia C, Asmundis De Flamee P, analysis. Pacing & Clinical Electrophysiology. 2013;36:1516–21 & Clinical Electrophysiology. Pacing analysis. during arrest J Cardio general cardiac anesthesia in an undiagnosed Sudden Brugada patient. et al. N, Takai S, Nakao M, Fuyuta Cantrell pentalogy Name Brugada syndrome management Perioperative JD. Tobias J, Hollister Baty L, Camurati-Engelmann disease dysplasia) diaphyseal 464–5 2013;23: Anesth. Pediatr with for a child Anesthesia Camurati–Engelmann disease. Almenrader N. M, Passariello disease Canavan management ofAnesthetic et al. O, Takahata A, Suzuki T, Saito 536 Appendix A: Anesthesia Implications of Syndromes… ) 6 cult airway airway cult diffi have cult; 167–73 :329–41 ediatr Anesth. 2008;18:1235–7 ediatr Anesth. ):1653–7 ardiothorac Vasc Anesth. Anesth. Vasc ardiothorac Preoperatively assess cardiac status (echocardiogram), status (echocardiogram), assess cardiac Preoperatively mandible Hypoplastic if indicated. SBE prophylaxis intubation diffi make may post-op airway Monitor cart available. to Sensitive assess respiratory status. Preoperatively, and respiratory depressants: propofol, thiopental, may ventilation (postoperative relaxants muscle avoid all MH triggers Avoid if used). be required relaxants (see Chap. appropriately workstation and prepare Anesthesia implications Preoperatively assess cardiac status (echocardiogram) status (echocardiogram) assess cardiac Preoperatively induction and stress-free Slow disease. for valvular drugs that stimulate Avoid of maintenance anesthesia. or release ketamine) the sympathetic (i.e., system meperidine). atracurium, histamine (morphine, is the drugOctreotide of for perioperative choice of manifestations control carcinoid heart be (carcinoid disease) may brosis ushing, hypotension, etc.) is very in rare etc.) hypotension, ushing, diagnosis management of and perioperative Semin C heart carcinoid disease. See Kasabach-Merritt syndrome syndrome See Kasabach-Merritt peculiar facies, oxycephaly, delay, developmental Obesity, defects, cardiac deformed extremities, syndactyly, hypogenitalism risk ofIncreased MH Description common in adults but may occur in children, often in the often in children, occur in adults but may common GI bronchus, testis, (i.e., appendix but also in other sites Carcinoid the diagnosis is made at histology. Usually tract). (fl syndrome especially with occur malignant carcinoid but may children fi Cardiac tumors. present syndrome See Jervell-Lange-Nielsen

without wasting. hypotonia muscle dystrophy; Muscular more peptides) are vasoactive tumors (secrete Carcinoid

Name Capillary angioma with thrombocytopenic purpura syndrome tumors Carcinoid 2011;23 Anesth. J Clin anesthetic considerations and perioperative syndrome Carcinoid et al. JP, Boudreaux AD, Kaye K, Mancuso in concepts Current Solis J. G, Silvay Castillo JG, 2013;17(3):212–23 Cardioauditory syndrome Carpenter syndrome (acrocephalopolysyndactyly) type P implications ofAnesthetic II). (Acrocephalopolysyndactyly Carpenter syndrome G. Grover S, Nishtala Rajeev S, YK, Batra 2014;25(5 Surg. J Craniofac a review for the craniofacial Carpenter surgeon. syndrome: et al. NJ, Healy S N, Helman S, Kadakia disease Central core 2013;23:834–41 and malignantAnesth. hyperthermia susceptibility: myopathies a review Core Pediatr MC. Theroux Brislin RP, 2009;109(4):1 Analg. and risk of Anesth myopathies malignant hyperthermia. Core et al. F, Lehmann-Horn H, Rueffert W, Klingler Appendix A: Anesthesia Implications of Syndromes… 537 cult cartcult airway ed pulmonary infections Table continues on the following page. continues on the following Table . J Anesth. 2009;23(4):630–2 Anesth. J . 6(3):361–2 cult airway cart at hand. May have have May cartcult airway at hand. Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively diffi pulmonaryAssess have status; available. Treat hypoprothrombinemia, fresh-frozen fresh-frozen hypoprothrombinemia, Treat available. Drug surgical bleeding. decrease plasma may and relaxants titrate muscle metabolism is impaired; of reversal complete Ensure kidneys. by drugs excreted relaxants muscle may have impaired pulmonary function. Aggressive Aggressive pulmonary impaired function. have may is complications postoperative prevent therapy to supplemental steroids. Give required. transfusions platelet require may Thrombocytopenia Use sterile technique (reverse isolation). Use Use isolation). (reverse sterile technique Use Repeat disposable equipment. airway and intubation which worsens with age; have with have worsens age; and intubation which airway diffi function renal impaired Possible laryngomalacia. Assess cardiac status (echocardiogram). Diffi status (echocardiogram). cardiac Assess Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively are non-depolarizing relaxants to muscle Responses of Beware MH arrhythmias. usually normal. patients been used in many triggering agents have without problems Anesthesia implications ltration of ltration ciency, ciency, recurrent to ux predisposes Neonatal jaundice, hepatomegaly, polycystic kidneys, kidneys, polycystic hepatomegaly, jaundice, Neonatal and facies, dysmorphic coagulopathy, hypotonia, muscular and Hypotonia be present. CHD may delay. developmental refl gastroesophageal pneumonia multiple organs. Partial albinism, immunodefi albinism, Partial organs. multiple bacterial recurrent hepatosplenomegaly, pancytopenia, delay. and developmental disorders Neurologic infections. induce to be given drugs may therapy and cytotoxic Steroid remission choanal atresia, renal abnormalities, genital hypoplasia, and genital hypoplasia, abnormalities, renal atresia, choanal ear defects Hereditary polyneuropathy. Muscle weakness in legs and weakness Muscle polyneuropathy. Hereditary conduction arrhythmias, involvement: Cardiac arms. MH has been described in two cardiomyopathy. defects, a clear relationship however, patients with CMT syndrome; is not established Description histiocyte of infi Disorder function, neutrophil heart congenital disease, association ofAn Coloboma, See DiGeorge syndrome (Bowen syndrome, syndrome, (Bowen (peroneal (peroneal Name Cerebrohepatorenal syndrome syndrome) Zellweger Platis CM, Kachko L, Peled E, et al. Anesthesia for the child with Zellweger syndrome: a case report. Paediatr Anaesth. 2006;1 Anaesth. Paediatr a case report. syndrome: with for the child Anesthesia Zellweger et al. E, Peled L, Kachko Platis CM, 22 syndrome Catch Charcot-Marie-Tooth syndrome atrophy) muscular 1992;39(4):398–400 Anaesth. a review disease: of Can J 86 cases. for Charcot-Marie-Tooth Anaesthesia JF. Antognini association CHARGE 1991;46:582–5 Anaesthesia. association. and management of in the CHARGE Incidence problems airway RK. Wyse Stack CG, management with airway use of Successful a laryngeal syndrome K. in a patient with mask airway Fukuda CHARGE K, Hirota Y, Hara syndrome Chédiak-Higashi 1995;13(1):101–5 Anaesth. East J Mid report. syndrome—case in Che’diak-Higashi Anesthesia et al. Ovali E, N, Erciyes H, Ulsoy 538 Appendix A: Anesthesia Implications of Syndromes… cult; if cult; cult airway. Paediatr Paediatr cult airway. nol. 2013;9(2):153–60 nol. 8:647–50 cartcult airway at hand. cartcult airway at hand. English abstract. Masui. Masui. English abstract. sth. 1990;2(5):306–11 sth. the diffi have cult; Anesthesia implications Preoperatively assess respiratory status. Check assess respiratory status. Preoperatively isolation). (reverse sterile technique Use coagulation. Caution with induction. rapid sequence Consider drugs in the liver metabolized Hypoplastic mandible may make tracheal intubation make mandible may Hypoplastic diffi Monitor body temperature and be prepared to to and be prepared temperature body Monitor chest Use gases. inspired Humidify cooling. institute and postoperatively preoperatively physiotherapy there is acute respiratory distress, tracheotomy may be may tracheotomy respiratory is acute distress, there the diffi Have required. during occur surgery bleeding may ofProfuse the disease mass Tracheal intubation may be extremely diffi be extremely intubation may Tracheal view: diffi for nasal intubation in a predicted broscopic a new technique ammation. Poor pulmonary Poor ammation. ciencies: current status and future prospects. Drugs. 2014;74(9): 963–9 2014;74(9): Drugs. prospects. status and future current ciencies: persistent respiratorypersistent infections → The absence of sweating and tearing. Heat intolerance due intolerance of Heat The absence and tearing. sweating mucus Poor sweating. by temperature inability control to to formation Description Inherited disorder of leukocyte function: recurrent of disorder recurrent Inherited function: leukocyte infections with or nonpathogenic organisms (Bacteria infl fungi) and disordered involvement. organ system Multiple function. disease leads liver advanced % of cases, in 95 Hepatomegaly be present. may Thrombocytopenia portalto hypertension. Bone regurgitation and aspiration. to GI lesions predispose therapy be effective transplant may marrow masses may cause respiratory distress masses may syndrome See Saethre-Chotzen of dysplasia Fibrous mandible and maxilla with intraoral (anhidrotic (anhidrotic

2000;49(4):414–6 2005;1 Anaesth. East J Mid case report. syndrome—a in Christ Touraine Siemens problems Anesthetic Balakrishnan M. R, Kuriakose Gene therapy Gennery for primary R. immunodefi W, Qasim Expert Clin Immu Pulmonary Rev manifestations of granulomatous disease. et al. chronic N, Rezaei SA, Mohajerani SA, Mahdaviani Hotta M, Koitabashi T, Umemura N, et al. Anesthetic management of a patient with hypohidrotic ectodermal dysplasia. [Japanese] management ofAnesthetic a patient with ectodermal dysplasia. hypohidrotic et al. N, Umemura T, Koitabashi M, Hotta granulomatous Chronic disease Ane J Clin granulomatous disease. in patients with considerations Anesthetic chronic et al. TA, Epstein CA, Buzzanell RT, Wall Christ-Siemens-Touraine Christ-Siemens-Touraine syndrome ectodermal dysplasia) Name Cherubism under fi nasal tube exchange Oral to et al. Artes D, A, Garcés E, Monclus syndrome Chotzen Anaesth. 2008;18(7):663–6 Anaesth. Appendix A: Anesthesia Implications of Syndromes… 539 cult airway airway cult cult tracheal cult culties; have the have culties; Table continues on the following page. continues on the following Table with growth arrest. Paediatr Paediatr with growth arrest. position. cult to cart cult airway available ammatory state. TIVA with propofol TIVA ammatory state. the diffi have cult tracheal intubation; Risk of cartcult airway at hand. fat embolism Temporomandibular or cricoarytenoid arthritis may Temporomandibular and intubation diffi cause airway diffi or minor trauma. fracture, osteotomy, after therapy steroid Supplement Concern that stress of that stress Concern anesthesia and surgery may infl exacerbate Caution on this basis. recommended and remifentanil diffi Potential with drugs. relaxant the diffi have intubation; cart available. Associated subglottic stenosis may subglottic may stenosis Associated cart available. age tube (weight vs. a smaller diameter require be diffi May appropriate). and renal ofConsiderations cardiovascular associated ECG for evidence ofA preoperative disease. be indicated may ischemia/infarction myocardial Anesthesia implications Diffi brosis. Renal Renal brosis. ltration of muscle, ammation starting in ltration with fi sm, developmental delay, and premature and premature delay, developmental sm, exion contractures. Associated Associated contractures. exion repeated by ammatory characterized syndrome llagen disorders. Curr Opin Pediatr. 2014;26(1): 79–84 2014;26(1): Opin Pediatr. Curr llagen disorders. involvement common. Frequently receiving steroid therapy steroid receiving Frequently common. involvement Chronic infantile neurologic cutaneous articular syndrome. cutaneous articular infantile neurologic syndrome. Chronic Genetic autoinfl attacks of and skin joint infl fever wasting and muscle delay developmental Progressive infancy. be associated may dysmorphia Facial occur. may tissue diseases with variable systemic connective Systemic fatty infi Osteoporosis, involvement. pulmonary infi anemia, Description senescence; patients present in early childhood. Prominent Prominent in early childhood. patients present senescence; peripheral Ataxia, eyes. and sunken large teeth, maxillae, and fl neuropathy, Survival disease. and renal arteriosclerosis, hypertension, decade is unusual second beyond Dysmorphic dwarfi Dysmorphic

Name CINCA syndrome 2008;18(6):575–7 Anaesth. Paediatr in an infant withAnesthesia a CINCA syndrome. U. Burkhardt C, Hohne 2014;12:52 Online J. Rheumatol Pediatr treatment of Neonatal CINCA syndrome. et al. A, Scheven-Gete Von Berthet G, Y, Paccaud syndrome Cockayne of management of aging syndrome—recognition accelerated patients with Cockayne Perioperative N. Buu KA, Brown S, Raghavendran diseases Collagen (dermatomyositis; polyarteritis nodosa; arthritis;rheumatoid lupus systemic erythematosus) 1998;53(3):314 Anaesthesia. disease) arthritis chronic (Still’s for patients withAnaesthesia juvenile BL. Smith Orthopedic aspects of SM. co Doyle LE, LaMont Anaesth. 2008;18(4):360–1 Anaesth. 2013;113:1637–50 Clin Neurol. Handb repair. of Disorders excision nucleotide Rapin I. 540 Appendix A: Anesthesia Implications of Syndromes… urane ear review. Paediatr Anaesth. Anaesth. Paediatr ear review. 006;16(4):466–70 n with anhidrosis: case reports and reviewn with anhidrosis: Anesthesia is required for surgical procedures to block to for surgical procedures is required Anesthesia The and unpleasant sensations. tactile hyperesthesia doses in excessive use of prevent may the BIS monitor for validated but the BIS is inadequately teenagers, monitoring of Careful body children. younger of and maintenance normothermiatemperature of anticholinergics not essential. Avoidance required. induction rapid sequence Consider Preoperative consultation with is the cardiology consultation team Preoperative Because of the possibility ofstrongly recommended. arrhythmia intraoperative or increased insertion of preoperative atrioventricular a block, is usually temporary pacemaker transvenous via be achieved the umbilical this may recommended; transcutaneous Alternatively, in the neonate. vein Transesophageal be considered. pacing via pads may Sevofl in infants. not effective pacing is often increase to be useful for anesthesia as it tends may heart rate Anesthesia implications cient nerve cient growth factor. Insensitivity to pain and temperature, lack of pain and temperature, Insensitivity to growth factor. Hyperpyrexia delay. and possible developmental sweating, gastric emptying Delayed has been reported occur. may Rare autosomal recessive disorder due to defi due to disorder recessive autosomal Rare associated with other CHD lesions. Defect of withassociated other CHD lesions. conduction atrioventricularbetween node and bundle of or within His occur, arrhythmias may Supraventricular bundle of His. heart and failure congestive to % progress 20 and up to poorly less than 55 are rates Heart attacks. Stokes-Adams drugs chronotropic to infants and the response by tolerated is usually minimal Description be % of may heart congenital disease, Comprises less than 1 (CIPA) (CIPA) Name heart Congenital block heart management of a 16-y Anesthetic block: withcomplete the neonate congenital Thiagarajan RR et al. DR, Madril BD, Kussman insensitivity Congenital to pain and anhidrosis 2 Anaesth. Paediatr of with insensitivity in a child pain with congenital BIS monitor to Use anhidrosis. Stuth EA. Brandes IF, 2005;15(12):1059–66 Risk of et al. aspiration during anesthesia in patients with insensitivity pai I, congenital to Rozet SE, Gruenbaum A, Zlotnik 2010;24(5):778–82 Anesth. J of the literature. Appendix A: Anesthesia Implications of Syndromes… 541 cult cult cult, and cult, Table continues on the following page. continues on the following Table diffi cult airway; have m. 2015;37:1–3 m. ion of the clinical picture. Am J Dis Child. J Dis Child. Am ion of the clinical picture. cult airway cart at hand. Monitor Monitor cartcult airway at hand. Preoperatively assess cardiac status (echocardiogram), status (echocardiogram), assess cardiac Preoperatively diffi Possible if indicated. airway cart airway available airway obstruction develops easily; have the diffi obstructionairway easily; develops have midazolam Caution with (avoid) cartairway available. Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively be diffi may Intubation withCare asepsis. Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively Caution with the airway;Check endocrine status. have the diffi blood glucose Monitor ventilation. postoperative during long procedures Anesthesia implications in 20 %. Endocrine problems Endocrine problems %. in 20 cans congenita (dysplasia epiphysealis punctata): recognit punctata): epiphysealis (dysplasia cans congenita et al. Cornelia de Lange syndrome. Clin Genet. 2015;88(1):1–12 Clin Genet. de Lange syndrome. Cornelia et al. ng, congenital cataracts. CHD and renal anomalies in CHD and renal cataracts. congenital ng, Chondrodystrophy with contractures, saddle nose, saddle nose, with contractures, Chondrodystrophy delay, developmental and micrognathia, microcephaly dwarfi some other cases disease Gierke See von Short or dysmorphic extremities, hypoplastic nipples, rib nipples, hypoplastic Short extremities, or dysmorphic and downturned thin lips, hairline, Low defect. and sternal %. CHD in 30 growl. Cry is low-pitched mouth. (“cod”) infections to susceptible Pulmonary aspiration is common, is one case report of There defect). system (immune respiratory following midazolam depression redundant skin (neck, palms, soles), and papillomata (oral, and papillomata (oral, soles), palms, skin (neck, redundant %, CHD in 30 is common. involvement Cardiac anal). nasal, hypertrophic cardiomyopathy include hypopituitarism, hypothyroid, and hypoadrenal and hypoadrenal hypothyroid, include hypopituitarism, problems airway Potential occur. may Hypoglycemia states. and macroglossia, atresia, choanal include short neck, laryngeal papillomata Description hirsute. delay, developmental microcephaly, Short stature, facies, coarse growth, and delayed delay Developmental

koala cans congenita; Name syndrome Conradi calcifi Chondrodystrophia AP. Gold AR, Mastri WG, Tasker Cori disease Cornelia de Lange syndrome 1998;8(2):181 Anaesth. Paediatr implications ofAnaesthetic de Lange syndrome. Cornelia JF. Mayhew B, Tsusaki J Clin Phar Int with in a child de Lange syndrome. drug Cornelia reaction Unpredictable et al. Bokun Z, Milojevic I, Stevic M, syndrome Costello 2003;13(3):257–62 Anaesth. Paediatr implications ofAnaesthetic syndrome. Costello JD. Tobias M, Bothwell K, Katcher (chondrodysplasia epiphysealis epiphysealis (chondrodysplasia chondrodysplasia punctata; calcifi bear syndrome) 1970;119:122–7 2011;52(1):6–34 J Lipid Res. of synthesis. disorders caused by cholesterol syndromes Malformation GE. Herman FD, Porter K, Brondum-Nielsen C, Jespersgaard MI, Boyle 542 Appendix A: Anesthesia Implications of Syndromes… cult cult, cult, cult cult xation devices xation the diffi have cult; nestesiol. 2010;60(6):630–1 nestesiol. Endocrinol Metab. Endocrinol Metab. Anaesth. 2001;11(2):228–32 Anaesth. what level? Arch Dis Child. Dis Child. Arch what level? 95;5(2):139–41 airway cart at hand. Monitor body temperature; use temperature; body Monitor cartairway at hand. Do not use hot air warming blankets. forced carefully; Transfuse depressants. myocardial because ofovertransfusion is poorly tolerated contractility myocardial decreased Correct hypothyroidism and anemia preoperatively if and anemia preoperatively hypothyroidism Correct be useful. may triiodothyronine Intravenous possible. diffi have large tongue; due to problems Airway Preoperatively assess cardiac status (echocardiogram) status (echocardiogram) assess cardiac Preoperatively laryngomalacia. stridor, problems: Airway if indicated. be diffi intubation may Tracheal Eye protection important. Mask ventilation diffi ventilation Mask important. protection Eye the diffi oral airway; have ± thrust jaw requires intubation usually easy. Tracheal cartairway at hand. elective obstruction airway is common; Postoperative of Beware additional be indicated. may tracheostomy fi external to related problems airway postsurgery for maxillary limit distraction may which trismus the mouth and lead to to access airway cart at hand. A small size tube may be required. be required. tube may A small size cartairway at hand. Risk of croup postextubation Anesthesia implications of a patient with Cri Du Chat syndrome. Case report. Rev Bras A Bras Rev of Case report. a patient with Cri Du Chat syndrome. retention common. common. retention 2 yroid dysfunction: Achievements and challenges. Best PractRes Clin Best PractRes and challenges. Achievements yroid dysfunction: derations in a hypothyroid infant with hepatic haemangioma. Paediatr infant withderations hepatic haemangioma. in a hypothyroid Hypoglycemia, hyponatremia, hypotension, low cardiac cardiac low hypotension, hyponatremia, Hypoglycemia, is essential to Early treatment with levothyroxine output. delay developmental prevent Goiter; hypothyroidism secondary to defective synthesis of secondary defective to hypothyroidism Goiter; very Respiratory center Large tongue. hormone. thyroid CO depression; to sensitive 5p abnormalityChromosome delay, causing developmental face, round microcephaly, cry, abnormal catlike micrognathia, ears abnormal, some, In hypertelorism. be present CHD may and larynxepiglottis, small. hypoplastic maxilla, and exophthalmos because of and exophthalmos maxilla, hypoplastic of closure defect causing premature bony chromosomal and intracranialcranial sutures hypertension Description cult tracheal intubation induced by maxillary distraction devices in craniosynostosis syndromes. Paediatr Anaesth. Anaesth. maxillary Paediatr by cult tracheal intubation induced distraction devices syndromes. in craniosynostosis beak nose, parrot hypertelorism, Craniosynostosis, (congenital (congenital dos Santos KM, de Rezende DC, Borges ZD. Anesthetic management Anesthetic Borges ZD. DC, de Rezende KM, dos Santos Name Cretinism hypothyroidism) consi Perioperative et al. EA, Eldredge BV, Koka KP, Mason syndrome Cri du chat 19 Anaesth. Paediatr for the patient with considerations Anaesthetic cri syndrome. du chat RE. Schwartz SA, Stayer Brislin RP, syndrome Crouzon 1995;5:331 Anaesth. Paediatr syndrome. with in a child Crouzon’s problems airway Postoperative AJ. Cranston JF, Payne Diffi A. Heggie G, Frawley J, Roche 2002;12(3):227–34 2015;29(3):399–413 Grosse SD, Van Vliet G. Prevention of intellectual disability through screening for congenital hypothyroidism: how much and at much how of hypothyroidism: for congenital disability intellectual screening through Prevention Vliet G. Van SD, Grosse 2011;96(4):374–9 of th congenital Treatment Biebermann H. P, Kuhnen H, Krude Appendix A: Anesthesia Implications of Syndromes… 543 cult cult airway airway cult Table continues on the following page. continues on the following Table ;21:326–30 y. J Cardiothorac Vasc Anesth. Anesth. Vasc J Cardiothorac y. exes exes 17(1):88–9 pulmonary undergoing stenosis ; an infusion may be required. be required. an infusion may ; ++ culty poor maintaining IV line due to cult airway and intubation; have the diffi have and intubation; cult airway airway cart available. A smaller diameter tracheal tube A smaller diameter cartairway available. short-acting drugs for rapid Use be required. may ofrecovery refl airway Diffi Preoperatively assess cardiac status (echocardiogram) status (echocardiogram) assess cardiac Preoperatively (reverse sterile technique Use cardiology. and consult be previously Donor blood must irradiated isolation). Check graft-versus-host reaction. prevent to (30 Gy) Ca calcium levels Preoperatively assess pulmonary status. Use sterile Use assess pulmonary status. Preoperatively Diffi technique. larynx cause soft tissues around may Excess tissues. the diffi obstruction; have upper airway cart at hand Caution with intubation; may have laryngomalacia have Caution with may intubation; and short trachea Anesthesia implications ux and aspiration ciency: susceptibility ciency: ulous skin, frequent hernias. hernias. frequent ulous skin, nt of generalized congenital cutis laxa syndrome associated withnt of associated cutis laxa syndrome generalized congenital ement of surger patients with undergoing cardiac DiGeorge syndrome pend ber degeneration: with developmental delay, trigonocephaly, dysmorphic trigonocephaly, with delay, developmental cardiac stenosis, choanal cleft palate, small mouth, facies, refl Gastroesophageal disease. and renal leading to repeated pulmonary repeated infections leading to Aortic arch and cardiac abnormalities. Thymus and Thymus abnormalities. and cardiac arch Aortic serum Ca low hypoparathyroidism, absent, parathyroids with associated Often and stridor. in tetany resulting defi Immune 22 defect. chromosome to fungal and viral infections; recurrent chest infections. infections. chest fungal and viralto recurrent infections; transplants thymic by Treated Recurrent pulmonary infections, emphysema and cor and cor emphysema pulmonaryRecurrent infections, arterial fragilitypulmonale, Description (page 237) See hydrocephalus deletion ofPartial short arm of 9 is associated chromosome (Catch (Catch disease See collagen Elastic fi Elastic inguinal hernia repair. Pediatr Anesth. 2008;18:907–9 Anesth. Pediatr inguinal hernia repair. Yeoh TY, Scavonetto F, Hamlin RJ, et al. Perioperative manag Perioperative et al. RJ, Hamlin F, Scavonetto TY, Yeoh Pandey R, Garg R, Manikandan R, et al. Perianesthetic manageme Perianesthetic et al. R, Manikandan Garg R, R, Pandey syndrome Dandy-Walker 2007; Anaesth. Paediatr with management in a child Anesthetic deletion 9p syndrome. et al. Altintas F, Bakan M, OS, Cakmakkaya Dermatomyositis DiGeorge syndrome deletion 22q 22 syndrome, Velocardiofacial syndrome, and fourth third syndrome, brachial arch/pharyngeal syndrome) pouch 1997;11:811 Anesth. Vasc J Cardiothorac syndrome. for DiGeorge’s Anesthesia et al. S, Sanyal Agarwal RC, VP, Singh 2000 J Otolaryngol. Am Structural review. a current anomalies in patients with airway NL. DiGeorge syndrome: Shapiro RY, Huang Deletion 9p syndrome 2014;28(4):983–9 Name laxa Cutis 544 Appendix A: Anesthesia Implications of Syndromes… rophy. Paediatr Anaesth. Anaesth. Paediatr rophy. osures. Pediatr Anesth. Anesth. Pediatr osures. atients with muscular Duchenne tr Anaesth. 2006;16(2):105–8 tr Anaesth. Preoperatively assess cardiac status (echocardiogram) status (echocardiogram) assess cardiac Preoperatively Succinylcholine particularly in adolescents. cause hyperkalemic cardiac (may contraindicated leading be undiagnosed in infancy, DMD may arrest). use of elective avoid to recommendation to of less than 6 years in boys age. succinylcholine TIVA cause rhabdomyolysis; agents may Inhalational easily: occurs Respiratory depression is preferred. titrate limit cardiorespiratory drug dosage to non-depolarizing relaxants muscle Give depression. local analgesia Use block. judiciously and monitor IPPV support be needed may possible. whenever postoperatively Anesthesia implications dation for muscle biopsies in patients with dyst dation for muscle muscular Duchenne diac MRI versus echocardiography in the pre-operative assessment of in the pre-operative p echocardiography diac MRI versus Progressive pseudohypertrophy of pseudohypertrophy with muscles Progressive in occurs Predominantly in most cases. cardiomyopathy in also occurs syndrome, Becker a milder form, males; in mutation recessive X-linked Genetic cause: females. be subclinical May 21. gene at chromosome dystrophin of 20 years die before age and many until 2–6 years Description See leprechaunism leprechaunism See

) 6

dystrophy. Eur J Paediatr Neurol. 2015;19(4):395–401 Neurol. J Paediatr Eur dystrophy. 2014;24(8):851–6 The value of et al. car F, Muntoni E, Parish A, Brunklaus Yemen TA, McClain C. Muscular dystrophy, anesthesia and the safety [Editorial] Paedia of again. inhalational agents revisited; dystrophy, Muscular C. McClain TA, Yemen review of dystrophy: muscular 117 anesthetic exp or Becker and Duchenne Anesthesia et al. TN, Weingarten JD, Lorenz Segura LG, 2013;23:855–64 se and ketamine Dexmedetomidine J. Kean M, Corridore H, Kako Name syndrome Donohue Down syndrome (see Chap. Duchenne muscular muscular Duchenne dystrophy Appendix A: Anesthesia Implications of Syndromes… 545 cult; have have cult; cult with cleft palate; Table continues on the following page. continues on the following Table maintain IV line and cult to C syndrome. Anaesthesia. Anaesthesia. C syndrome. cult airway cart available. Be prepared to to Be prepared cartcult airway available. cult airway cart available. Use caution with Use cartcult airway available. the diffi kidney by drugs excreted Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively be diffi and tracheal intubation may Airway Diffi ECG. Preoperative tissues of Poor complications IV infusions. prevent surgical increased lead to and clotting defect may occur. Spontaneous pneumothorax may bleeding. Caution abnormalities. for ECG conduction Monitor anesthesia relatively Neuraxial with neck movement. (riskcontraindicated of bleeding) Anesthesia implications Assess nutrition and anemia. Preoperative chest chest Preoperative nutrition and anemia. Assess anticholinergics (i.e., avoid advised; physiotherapy with care skin Extreme [effect on sweating]). atropine eyes. Protect position and pad carefully. required; be diffi intubation may Tracheal the diffi have maintain normothermia using heating/cooling etc. blankets, Developmental delay and dysmorphic changes, changes, and dysmorphic delay Developmental renal %, CHD in 95 hypotonia. %, in 80 micrognathia die in infancy Most %. malformations in 50–80 abnormality:Collagen hyperelasticity and fragile tissues; dissecting aneurysm of fragility aorta, of other blood Bleeding diathesis; abnormalities. ECG conduction vessels: and gastrointestinal lung, heart, have May hernias. malformations Description Congenital anomaly complex. Lobster claw deformity, deformity, claw Lobster anomaly complex. Congenital ofdysplasia all ectodermal elements (including central control temperature with disordered nervous system), tearing, Decreased plus central defect). (hypohidrosis respiratory Cleft lip and palate, blepharitis. conjunctivitis, and malnutrition, genitourinarytract anomalies, infections, % in 8 delay Developmental anemia. See Trismus-pseudocamptodactyly Trismus-pseudocamptodactyly See

(trisomy (ectrodactyly, (ectrodactyly, EEC syndrome EEC syndrome Name syndrome Dutch-Kentucky the EE and cleft lip palate: ectodermal dysplasia with in a child ectrodactyly, problems Anaesthetic M. Satoyoshi A, Mizushima syndrome Edwards 18[E]) 2003;13(3):267–9 Anaesth. Paediatr syndrome. management for Edward’s Anaesthetic D. Lecoutre Nieuviarts R, P, Courreges Ehlers-Danlos syndrome (cutis hyperelastica) 2006;34(4):501–5 Care. Intens implications ofAnaesth Anaesthetic vascular type Ehlers-Danlos syndrome. Lane D. 1992;47:137–40 ectodermal dysplasia, and ectodermal dysplasia, cleft lip and palate) 546 Appendix A: Anesthesia Implications of Syndromes… cult; cult; tracardiac shunts and tracardiac shunts ;42:904–9 uid therapy, hypovolemia is not hypovolemia uid therapy, cart cult airway available cantly. Caution with controlled ventilation to to ventilation Caution with controlled cantly. Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively but airway intubation can be routine, Tracheal intubation diffi make may and peg teeth problems the diffi have Assess cardiac status (echocardiogram) and consult and consult status (echocardiogram) cardiac Assess shunt shunt; cardiac severityAssess of R-L cardiology. or acidosis. hypercarbia, with increase hypoxia, may induction with anesthetic agents has Inhalation potent a slow Alternately, withbeen utilized caution. great be performed as rapid induction may intravenous drugs or Avoid occur. IV agents and may effect from hypercarbia, PVR (i.e., increase that may events airway high-dose SVR (i.e., acidosis) or decrease hypoxemia, vasodilators) intravenous thiopental or propofol, signifi intrathoracic but minimize maintain lung volume with Care fl pressure. well tolerated, and overtransfusion may lead to R lead to and overtransfusion may tolerated, well viscosity increases but Polycythemia failure. ventricular if oxygen caution required hemodiluting as it decreases problems, all the potential Despite carrying capacity. anesthetic. a well-conducted tolerate children many Epidural anesthesia has been used successfully Anesthesia implications with pulmonary hypertension and in nger sm, cardiac cardiac sm, ement and outcomes for patients ement and outcomes ement of the child with Eisenmenger’s syndrome. Can J Anaesth. 1995 Anaesth. Can J syndrome. ement of with the child Eisenmenger’s Ectodermal defects causing skeletal dwarfi Ectodermal defects causing skeletal anomalies (50 %), chest wall defects, and poor lung wall defects, chest %), anomalies (50 nails. and hypoplastic polydactyly, Short limbs, function. peg teeth, cleft lip, cleft palate, abnormal maxillae, have May die in infancy often Patients hepatosplenomegaly. (pulmonary hypertension) and an intracardiac or fi cyanosis, fatigue, Dyspnea, shunt. R-L extracardiac clubbing, and cardiac failure. Often associated with associated Down Often failure. and cardiac clubbing, syndrome Description

ofAssociation high pulmonary vascular resistance See Williams syndrome syndrome Williams See n facies syndrome Eisenmenger syndrome: a review of institutional experience. J Clin Anesth. 2014;26(4):286–93 Anesth. a review J Clin of syndrome: Eisenmenger institutional experience. Ellis-van Creveld syndrome syndrome Ellis-van Creveld (chondroectodermal/ mesoectodermal dysplasia) Name syndrome Eisenmenger The anaesthetic manag et al. W, Casey C, Motherway B, Lyons Elfi 2008;20:618–21 Anesth. J Clin syndrome. implications ofAnesthetic Ellis-van Creveld JD. Tobias AI, Abeles Bennett JM, Ehrenfeld JM, Markham L, et al. Anesthetic manag Anesthetic et al. L, Markham JM, Ehrenfeld Bennett JM, Appendix A: Anesthesia Implications of Syndromes… 547 culty: Table continues on the following page. continues on the following Table iatr Anaesth. 2002;12(5):388–97 iatr Anaesth. ement. J Anaesthesiol Clin Anaesthesiol J ement. andation or a well-padded Antibiotic prophylaxis perioperatively to prevent to perioperatively prophylaxis Antibiotic Check historysecondary of therapy. infections. steroid diffi Airway isolation). (reverse sterile technique Use intraoral adhesion of scarring;oral avoid lesions, tongue, tracheal intubation and/or instrumentation of the airway otherwise, lubricateif develop; possible as bullae may trauma to Prevent tube and laryngoscope generously. friction or especially from membranes, skin or mucous lubricated very generous Use shearing movements. insuffl Use padding. lubricated mask for inhalation anesthesia or use propofol with Care and use optical a tourniquet or ketamine. often tapes (patients/parents adhesive Avoid ointment. probe an oximeter tapes can be tolerated); which know with be held in place bandage. a lubricated gauze may with surgicalECG pads should be coated lubricant and be analgesia may Regional under the child. placed for limb surgeryappropriate Anesthesia implications Skin cleavage at dermal-epidermal junction, resulting in resulting at dermal-epidermal junction, Skin cleavage minor trauma skin or mucous from to and blisters erosions Simplex: forms: in several The disease occurs membrane. mild with Relatively 17. chromosome maps to dominant, maps to recessive, rapid healing and little scarring. Lethalis: Severe, epidermolysis bullosa. Junctional 12. chromosome scarring and death extensive leads to at birth, presents of 2 years sepsis) usually before age. from (often rare Very 12. chromosome maps to Recessive, Dystrophic: scarring. with lesions heal slowly extensive but severe; and larynx, the pharynx, form and involve Strictures may hand”). Digital (“mitten fusion occurs esophagus. and growth retardation deprivation leads to Nutritional common are Infections anemia. Description See histiocytosis X

Name Eosinophilic granuloma Paed anaesthesia and pain management. pathophysiology, Epidermolysis bullosa in children: et al. A, Goldman J, Denyer J, Herod Epidermolysis bullosa syndrome) (Herlitz principle for anesthesia manag monitoring and no touch Careful Epidermolysis bullosa: et al. SK, Gore NJ, Mandawade SarafV, S 2013;29:390–3 Pharmacol. 548 Appendix A: Anesthesia Implications of Syndromes… cult culty of intubation increases 013;57(6):603–5 996;83:430–2 2;22:490–2 (6):700–2 yses. Pediatr Anesth. 2013;23:824–33 Anesth. Pediatr yses. , glucose, and the ECG; maintain and the ECG; glucose, , + cult airway and intubation; have the diffi have and intubation; cult airway Preoperatively assess cardiac status (echocardiogram) status (echocardiogram) assess cardiac Preoperatively ECG for myocardial function; for myocardial function; for renal and creatinine BUN ischemia; the kidneys if by caution with renal drugs excreted is present dysfunction airway cart available. Diffi cartairway available. Caution with be limited. may IV access with age. be Epidural analgesia may padding and positioning. deformity despite appropriate Diffi normokalemia and normoglycemia. Avoid muscle muscle Avoid normokalemia and normoglycemia. maintain body Sch. hyperkalemia from relaxants; TIVA solutions. glucose excessive Avoid temperature. in has been successful with and remifentanil propofol be useful analgesia may Regional adult patients. Monitor serum K Monitor Anesthesia implications

+

+ ations for patients with myotonia congenita or periodic paral congenita ations for patients with myotonia ent in Escobar syndrome: A formidable challenge. Indian J Anaesth. 2 Anaesth. J Indian A formidable challenge. syndrome: ent in Escobar into muscles and thus decreased serum K decreased and thus muscles into + See Andersen syndrome secondary serum weakness K to muscle Periodic X-linked lipid storage disorder. Lipid deposition in blood disorder. lipid storage X-linked crises. pain and fever causes periodic veryvessels severe particularly around Dark telangiectasia, opacities. Corneal ischemia, myocardial hypertension, genitals and buttocks; ischemia and myocardial Hypertension failure. renal Autosomal recessive progressive disease; multiple joint multiple disease; progressive recessive Autosomal severe facial and genital anomalies, contractures, intellect Normal kyphoscoliosis. Description disturbance (hypokalemia or hyperkalemia). Muscle Muscle disturbance (hypokalemia or hyperkalemia). variety in the hypokalemic weakness massive is caused by ofuptake K congenita See paramyotonia syndrome See Riley-Day syndrome See Stevens-Johnson (multiple (multiple

Familial periodicFamilial paralysis Kachko L, Platis CM, Konen O, et al. Lumbar epidural anesthesia for the child with Escobar syndrome. Paediatr Anaesth. 2006;16 Anaesth. Paediatr epidural with syndrome. anesthesia for the child Escobar Lumbar et al. O, Konen Platis CM, L, Kachko 201 Anesth. Pediatr a patient with to pterygium multiple syndrome. approach Anesthetic (Escobar) S. Karaman H, Gunay Sertoz N, managem Airway et al. H, Arun Kumar Chaudhuri S, S, Mathew Familial osteodysplasia Kuzma PJ, Calkins MD, Kline MD, et al. The anesthetic management of patients with multiple pterygium syndrome. Anesth Analg. 1 Analg. The anesthetic management ofAnesth et al. patients with pterygium multiple syndrome. Kline MD, Calkins MD, PJ, Kuzma Fabry disease corporis(angiokeratomas diffusum) 2008;63(1):101–2 Anaesthesia. Anderson-Fabry disease. for considerations Peri-operative AC. Pichel J, Woolley dysautonomia Familial 2014;57:1–18 J Pediatr. Korean Channelopathies. JB. Kim and anesthetic consider Pathophysiologic PA. Iaizzo O, Bandschapp Name Erythema multiforme periodic Eulenburg paralysis syndrome Escobar pterygium syndrome) Appendix A: Anesthesia Implications of Syndromes… 549 urane, urane, cartcult airway Table continues on the following page. continues on the following Table ions. J Clin Anesth. Anesth. J Clin ions. . 1994;80:206–9 . cult intubation. Have the diffi Have cult intubation. ciency. [English abstract.] Masui. 2008;57(2):200–2 [English abstract.] Masui. ciency. Treat electrolyte and acid-base abnormalities: Caution electrolyte and acid-base abnormalities: Treat Cisatracurium is the kidneys. by with drugs excreted of possibility Be aware of relaxant. muscle preferred other metabolic or endocrine defects status. (ECHO) and renal assess cardiac Preoperatively and larynx; pharynx, possible Deposits in oral cavity, diffi Do not give drugs that cause hemolysis (i.e., drugs that cause hemolysis (i.e., Do not give sulfonamides, phenacetin, acetylsalicylic acid, sevofl Midazolam, blue). methylene quinidine, nitrous oxide, and rocuronium are all acceptable. all acceptable. are and rocuronium oxide, nitrous transfuse ifAnemia; necessary available available Anesthesia implications cient patient: a review of literature. Anesth. Prog. Prog. Anesth. a reviewcient patient: of literature. oni syndrome for ocular surgery:oni syndrome anesthetic considerat perioperative cystectomy in a patient with glucose-6-dehydrogenase defi in a patient with glucose-6-dehydrogenase cystectomy Usually secondary to cystinosis. Proximal tubular defect: tubular defect: Proximal secondary cystinosis. to Usually dehydration. K loss, acidosis, function; renal impaired and pancreatic thyroid have may Older children present May deposition. secondary cysteine dysfunction to decade transplant in second for renal widespread visceral deposition: Sphingomyelin General systemic especially in CNS. lipogranulomas, failure renal cardiac, leading to involvement Diathesis for spontaneous/induced (drugs, fava beans, beans, fava (drugs, Diathesis for spontaneous/induced infection) hemolytic anemia Description (anemia

(glucose-6-phosphate (glucose-6-phosphate ciency) Wada R, Hino H, Ando Y. Case of chole laparoscopic Y. Ando H, Hino R, Wada Elyassi AR, Rowshan HH. Perioperative management of the glucose-6-phosphate dehydrogenase defi management of dehydrogenase the glucose-6-phosphate Perioperative HH. Rowshan AR, Elyassi 2009;56(3):86–91 2010;22(8):635–7 with tubular acidosis) renal (lipogranulomatosis) Name Fanconi syndrome 2004;14(10):878–85 Anaesth. Paediatr of care cystinosis. the patient with nephropathic Perioperative JD. Tobias TL, Ray with syndrome Fanc Lowe’s Chakravarty C. Garg R, R, Pandey Farber disease Anesthesiology lipogranulomatosis. The anesthetic implications of a patient with et al. Farber’s T, Terai S, Tatekawa A, Asada Favism dehydrogenase (G6PD) dehydrogenase defi 550 Appendix A: Anesthesia Implications of Syndromes… cult cult airway; cult 0–5 the diffi culty with have intubation; cart cult airway available culties with ventilation. Potential diffi Potential culties with ventilation. have the diffi have Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively diffi have May Airway may contain papillomas resulting in papillomas resulting contain may Airway diffi Anesthesia implications airway cart available. Evaluate renal function renal Evaluate cartairway available. fect), renal abnormalities, abnormalities, renal fect), defects cardiac at maxilla), cans ts and fetal alcohol spectrum disorders. Congenit Heart Dis. 2007;2(4):25 Dis. Heart Congenit spectrum ts and fetal alcohol disorders. Abnormalities of the infant due to maternal heavy alcohol of alcohol Abnormalities heavy maternal the infant due to impairment, intellectual growth retardation, consumption: microphthalmia, craniofacial abnormalities (microcephaly, fl upper lip, hypoplastic septal de (especially ventricular and inguinal hernia ossifi See myositis Multifarious features, including multiple papillomas of including multiple features, Multifarious and giant cell cysts, ontogenic skin, membranes, mucous tumors of bone Description

cans cult intubation associated with the fetal alcohol syndrome. Can Anaesth. Soc J. 1980;27:574–5 Soc J. Anaesth. Can with syndrome. cult intubation associated the fetal alcohol Name syndrome alcohol Fetal 1978;298:1063–7 N Engl J Med. syndrome. The fetal alcohol DW. Smith SK, Clarren Diffi BT. Finucaine heart Congenital defec et al. Rios R, Deal E, L, Burd Fibrodysplasia ossifi progressiva 1991;3:422–5 Anesth. J Clin and anesthesia management in Goltz syndrome. involvement Airway RS. Holtzman 2012;56(4):394–6 Anaesth. J Indian management in Gorlin-Goltz syndrome. Anaesthetic SD. Mundada KS, Gosavi Focal dermalFocal hypoplasia (Goltz syndrome) (Goltz syndrome) Appendix A: Anesthesia Implications of Syndromes… 551 exion Table continues on the following page. continues on the following Table

in Anesth. 2007;19(6):460–2 in Anesth. ;26(1):217–9 beroptic bronchoscopy and bronchoscopy beroptic limb fl cult due to cult intubation primarily microstomia due to Very Very diffi tight will facial muscles and micrognathia: not relax muscle and masseter blockade, with neuromuscular rigidity halothane or succinylcholine. follow may diffi access Venous Preoperatively assess metabolic state and cardiac and cardiac assess metabolic state Preoperatively drugs; with depressant Care cardiac status (ECHO). with and remifentanil propofol TIVA ECG. monitor appear normal. BIS responses has been recommended. if possible. avoid uncertain; relaxants to Response Otherwise cisatracurium with block monitor suggested contractures. Pulmonary function may be impaired be impaired Pulmonary function may contractures. Insertion of(late). an LMA (if is not microstomia fi facilitates severe) useful for analgesia maybe Regional intubation. MH trigger-free An surgery pain. and/or postoperative in these children be indicated may anesthetic technique Anesthesia implications of contracture exion limbs. ent of a patient with Freeman-Sheldon syndrome: case report. J Cl case report. ent of syndrome: a patient with Freeman-Sheldon brosis, leading to failure and serious failure leading to brosis, brosis of facial muscles. Hypertelorism, microstomia, microstomia, Hypertelorism, ofbrosis facial muscles. Strabismus and inguinal hernia common. Later, Later, Strabismus and inguinal hernia common. is a rare There lung disease. kyphoscoliosis causes restrictive association with malignant hyperthermia See Von Gierke Disease Gierke Von See with and dysplasia myopathy congenital Progressive tone Increased inheritance. recessive or X-linked autosomal and fi fl to Leads and micrognathia. and lateral degeneration of the cerebellum, Progressive myocardial scoliosis; ofposterior column spinal cord; degeneration and fi diabetic % are 10 intolerance; Glucose arrhythmias. Description

(glycogen (glycogen Name Forbes disease disease typestorage III) Freeman-Sheldon syndrome syndrome) (whistling face managem Anesthetic et al. D, Jawish C, EI-Hajj S, Madi-Jebara Friedreich ataxia 2005;15(5):433–4 Anaesth. Paediatr ataxia. management in Friedreich’s Anesthetic D. Renz C, Pancaro Hamilton T, Sathyamoorthy M. A case of Freeman-Sheldon syndrome. Anesthetic challenges. J Miss State Med Assoc. 2016;57(1):6–8 Assoc. Med State J Miss challenges. Anesthetic A case of syndrome. Freeman-Sheldon M. Sathyamoorthy T, Hamilton 2015 Surg. J Craniofac with child microstomia. hyperthermia Malignant in a 3-year-old et al. SS, Tholpady RL, Flores TA, Evans 552 Appendix A: Anesthesia Implications of Syndromes… the cult airway; have :910–12 tol. 2010;27(2):156–61 tol. rature reviewrature of 134 previously cult airway cart available. Nasal passages too small passages too Nasal cartcult airway available. Administer anti-seizure medications on the morning anti-seizure Administer status assess cardiac Preoperatively of surgery. loss leads to neurologic Progressive (echocardiogram). assess cardiopulmonaryrespiratory complications; status status (echocardiogram) assess cardiac Preoperatively large tongue, anomalies, Facial cardiology. and consult diffi and short lead to neck may diffi Airway preferred. oral approach for FOB intubation: in sitting position improved Anesthesia implications ltration common. common. ltration ory GM2 gangliosidosis: 21 new cases and lite of or subacute juvenile Invariably fatal. Supportive measures only treatment measures Supportive fatal. Invariably severe onset in infancy: rapid decline, neurologic Acute pulmonarybone abnormalities; infi of 2 years Death by age deterioration, psychomotor Onset in infancy: progressive Jewish Ashkenazi in Predominantly seizures. blindness, 5 years Death by heritage. Death from few somatic changes. Onset in early childhood: ofcardiopulmonary 10 year causes by age longer survival variants: same features; juvenile Rare large tongue, optic atrophy, alopecia, retardation, Growth with associated dilated teeth, and unerupted pulmonary glaucoma hypertension, cardiomyopathy, Description (growth retardation, alopecia, alopecia, retardation, and optic pseudo-anodontia, atrophy) Name Gangliosidoses typeGM1, 1 typeGM1, 2 disease: GM2 (Tay-Sachs Sandhoff disease) The natural hist et al. M, Tropak T, Stockley GH, Maegawa GAPO syndrome 2011;21 Anesth. Pediatr management ofAnesthetic a patient with for glaucoma surgery. GAPO syndrome A. Laha A, Trikha R, Sinha Nanda A, Al-Ateeqi WA, Al-Khawari MA, et al. GAPO syndrome: a report of two siblings and a review of literature. Pediatr Derma a report of GAPO syndrome: siblings Pediatr and a review two of et al. literature. MA, Al-Khawari WA, Al-Ateeqi A, Nanda reported. Pediatrics. 2006;118(5):e1550–62 Pediatrics. reported. 2002;38(1):98–100 Child Health. in infantile Sandhoff J Paediatr disease. involvement Cardiac SN. Joshi P, Venugopalan Appendix A: Anesthesia Implications of Syndromes… 553 ltration; have have ltration; Table continues on the following page. continues on the following Table zmann’s thrombasthenia for nasal thrombasthenia zmann’s atr. 2013;172(4):447–58 atr. . Can J Anaesth. 2005;52(8):845–7 Anaesth. Can J . 2010;51(6):727–32 described but anemia c anesthesia problems transfusions platelet c therapy for bleeding; Surgical bleeding cartcult airway available. infi or airway cult if neck, trismus, No No specifi status (echocardiogram). assess cardiac Preoperatively pulmonary ofAssess beware status; aspiration. be but may intubation usually routine Tracheal diffi No specifi is possible due to bleeding polypsis possible due to activated Therapy with recombinant disappointing. be VII plus antithrombolytic agents may factor history of therapy have steroid May helpful. the diffi disorders coagulation treat be a major problem; may anemia and correct Anesthesia implications 3). If nonneuronopathic 3). cient lysosomal enzyme glucocerebrosidase. Serum acid cient lysosomal enzyme glucocerebrosidase. If obvious signs: usually fatal in neurologic ciency. bromas. Associated with bowel adenocarcinoma and adenocarcinoma with bowel Associated bromas. Familial polyposis of colon; bone tumors, sebaceous cysts, cysts, sebaceous polyposisFamilial of bone tumors, colon; fi intussusception defi involvement. cardiac have May phosphatase increased. aspirationPulmonary (pseudobulbar palsy) disease from cause platelet may Hypersplenism and hepatosplenomegaly. defi typeinfancy (neuronopathic 2 and fractures, with bone pain, chronic is more (type course 1), therapy enzyme to replacement respond Some may etc. mild leading to function, platelet Abnormal purpura;thrombocytopenic abnormality of high-energy bleeding risk Considerable with mechanisms. phosphate surgical procedure any Description

Name Gardner syndrome Nutr. Gastroenterol J Pediatr polyposis and adolescents. adenomatous Familial in children P. Mamula JP, Franciosi Alkhouri N, disease Gaucher due to etc. spleen, liver, in CNS, accumulation Cerebroside anesthesia for pediatric after patients with disease outcome Gaucher Uncomplicated et al. A, Abrahamov A, Briskin A, Ioscovich Glanzmann disease (thrombasthenia) of acid treatment VII and epsilon aminocaproic a patient with Glan factor activated Recombinant S. Pezek Z, Ozkose B, Gunaydin Kaplan P, Baris H, De Meirleir L, et al. Revised recommendations for the management of Gaucher disease in children. Eur J Pedi Eur for the management of disease in children. recommendations Revised Gaucher et al. L, De Meirleir Baris H, P, Kaplan 2007;21(1):106–7 Anesth. J polypectomy. 554 Appendix A: Anesthesia Implications of Syndromes… culty for culty cart). cult airway be laryngoscope may beroptic cult (bilateral) or very easy (unilateral supplies (diffi cult airway and maintain an hold a mask in place cult to A GlideScope and fi A GlideScope with TIVA for a surgical airway. Ready very useful. rapid facilitate may and remifentanil propofol the trachea awake Extubate emergence. Preoperatively assess cardiac status (echocardiogram) status (echocardiogram) assess cardiac Preoperatively be extremely may problems; Airway if indicated. diffi intubation Tracheal anesthesia induced. once airway be very diffi may If rightor left-sided lesion). TMJ and mandible are diffi increased disease, or bilateral involved if ready available) an LMA (ProSeal Have intubation. plus all diffi Anesthesia implications ciency ciency See favism disease Gierke See von Unilateral mandibular hypoplasia; CHD in 35 %. %. CHD in 35 mandibular hypoplasia; Unilateral 22 trisomy. Embryonic chromosome malformation due to limit neck extension abnormalities may Vertebral Description

ciency Name Glucose-6-phosphate (G6PD) dehydrogenase defi Glycogen storage disease Glycogen I Type II Type Forbes III (Cori disease; Type disease) IV Type Type V disease) VI (Hers Type VII Type disease Gierke See von disease See McArdle disease See Pompe disease Gierke See von See Andersen disease defi phosphofructokinase See muscle Type VIII Type Goldenhar syndrome See hepatic phosphorylase kinase defi (oculoauriculovertebral (oculoauriculovertebral hemifacial syndrome; microsomia) Appendix A: Anesthesia Implications of Syndromes… 555 cult cult airway airway cult cult airway; cult the diffi have cult; Table continues on the following page. continues on the following Table ypical plasma cholinesterase. ypical plasma cholinesterase. ndrome. Eur Rev Med Pharmacol Pharmacol Med Rev Eur ndrome. . 1991;57(4):161–3 . Pediatr Anesth. 2012;22:600–1 Anesth. Pediatr cart cult airway available cart available. Increased ICP may be unrecognized be unrecognized ICP may Increased cart available. Tracheal intubation may be diffi intubation may Tracheal Asymmetry of cause a diffi the head may cervical in positioning and intubating; care Extreme the diffi Have be limited. may movement have the diffi have airway cart available. Cervical cartairway spine precautions available. drugs Caution with if protein-bound indicated. Avoid has caused hypoproteinemia. chylothorax fasciculations that could prevent to succinylcholine Caution with transport bones. break and positioning. be required may ventilation Postoperative Anesthesia implications cult intubation. Pediatr Anesth. 1999;9:393–8 Anesth. Pediatr cult intubation. beroptic bronchoscope. Paediatr Anaesth. 2008;18(8):793–4 Anaesth. Paediatr bronchoscope. beroptic management using the ProSeal laryngeal with in a child mask airway management using the ProSeal Goldenhar sy brosarcomas, kyphoscoliosis, incomplete segmentation of incomplete kyphoscoliosis, brosarcomas, See focal dermal hypoplasia and Gorlin-Goltz syndrome See focal dermal and Gorlin-Goltz syndrome hypoplasia syndrome See Turner Pathologic and lymphangiomatosis. osteolysis Massive deformities with and and bony neurologic fractures be kyphoscoliosis may Severe respiratory complications. cervical spine involvement: bony to relate Problems present. thoracic deformity respiratorysubluxation, leading to be present. may Pleural effusions or chylothorax failure. intellect Normal ductus arteriosus, patent dysostosis, Craniofacial of hypoplasia hypertrichosis, dental and eye labia majora, intelligence Normal anomalies. hypertelorism, carcinomas, basal cell nevoid Multiple cysts and jaw multiple mandibular prognathism, fi cervical hydrocephalus, and thoracic congenital vertebrae; etc. delay, developmental Description Sci. 2012;16(4):559–61 Sci. Name of prediction anatomical diffi microstomia: Hemifacial et al. RD, Bennun Ririe DG, C, Nargozian Goltz syndrome Gonadal dysgenesis Gorham syndrome (disappearing bone disease) 2000;55(2):157–9 Anaesthesia. anaesthetic management. Gorham syndrome: W. Habre Szabo C, Gorlin-Chaudhry-Moss syndrome ObservationAnesthesiol EnglishMinerva of[Italian] abstract. Gorlin-Goltz syndrome. a case. G. Mammana I, Tiberio Ortalli G, Gorlin-Goltz syndrome nevus syndrome) (basal cell The use of therapy pain control. anesthesia in photodynamic et al. subcutaneous infusion tumescent C, Girard Sleth JC, A, Debu Altintas F, Cakmakkaya OS. General anesthesia for a child with Goldenhar syndrome. Pediatr Anesth. 2005;15(6):529–30 Anesth. Pediatr General with anesthesia for a child Goldenhar syndrome. OS. Cakmakkaya Altintas F, with and at management using the pediatric in a child Airway Goldenhar syndrome GlideScope T. and Hackmann AM, Dower AD, Milne 2007;17(5):484–7 Anaesth. Paediatr and intubation with Goldenhar syndrome the fi et al. Alacakir H, S, Simsek Ozlu O, Airway et al. MA, Erdogan Begec Z, MS, Aydogan 556 Appendix A: Anesthesia Implications of Syndromes… release may may release in rst present + K ⇑ cartcult airway release). May have serious have May release). + D002063 K 7 ⇑ child with Hallervorden-Spatz with child Hallervorden-Spatz Indian J Anaesth. 2015;59(1):43–6 Anaesth. J Indian 999;9(6):539–542 exes, and other signs of polyneuropathy exes, diffi have cult intubation); cult to maintain IV cannula in situ. Prevent Prevent in situ. maintain IV cannula cult to (risk ofcit resolves Preoperatively assess cardiac status (echocardiogram status (echocardiogram assess cardiac Preoperatively artery as for coronary Manage disease. and ECG). Diffi and hypertension of (rupture aneurysms). tachycardia NG tube Avoid an arterial damage). line (vessel Avoid (bleeding) for at least 3 months after Do not use succinylcholine onset of neuron motor polyneuritis and until lower defi Inhalation assess pulmonary status. Preoperatively induction of of relaxation anesthesia leads to abnormal posturing and trismus and facilitates ( succinylcholine Avoid intubation. available. Responses to other anesthetics are normal other anesthetics are to Responses available. intensify rigidity)intensify induction (in case or rapid sequence of diffi Anesthesia implications the postoperative period with weakness, loss of period with weakness, the postoperative refl tendon hemodynamic instability. Disease may fi Disease may instability. hemodynamic cation; cation; nt of a child with panthothenate kinase-associated neurodegeneration. nt of neurodegeneration. with a child kinase-associated panthothenate Degeneration of elastic tissue in the skin, eye, and Degeneration of eye, elastic tissue in the skin, of rupture especially in system; arteries, cardiovascular arterial tract; hypertension; calcifi gastrointestinal ofocclusion arteries and coronary cerebral peripheral neuritis; progressive polyneuropathy; Acute cranial bulbar palsy with nerves; usually involving an infection follow May and hypotension. hypoventilation and plasma exchange Early treatment by or surgery. is highlyimmunotherapy limit the disease. desirable to support and ventilatory tracheotomy Some require of disorder basal ganglia: leading to recessive Autosomal and scoliosis, Torticollis, and chorea. dystonia, dementia, of Episodes obstruction airway and trismus develop. during Stereotactic posturing. occur desaturation may state the neurologic improve may thalamotomy considerably Description

(pseudoxanthoma (pseudoxanthoma (acute [idiopathic] (acute polyneuritis) Name Grönblad-Strandberg syndrome elasticum) 1981;60(5):344– Analg. Anesth elasticum. in pseudoxanthoma considerations Anesthetic LW. Fabian RC, Ramirez-Inawat SL, Krechel Guillain-Barré syndrome 1 Anaesth. Paediatr diagnosis following anaesthesia. delayed syndrome: Guillain-Barre et al. K, Sykes JM, Wilmshurst GD, Jones Hallervorden-Spatz disease in a thalamotomy stereotactic computer-assisted, management for two-stage Anesthetic et al. JY, Matsumoto Flick RP, MT, Keegan Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2014;9:C Rev. Database Syst Cochrane syndrome. immunoglobulin for Guillain-Barre Intravenous van Doorn PA. AV, Swan RA, Hughes 2000;12(2):107–11 Anesthesiol. J Neurosurg Disease. manageme Anaesthetic S. Bhattacharjee G, Biyani R, Sinha Appendix A: Anesthesia Implications of Syndromes… 557 cartcult airway Table continues on the following page. continues on the following Table nt after neck mass excision. neck mass excision. nt after esth. 1999;9(5):444–7 esth. during develop neck surgeryushing may mouth opening not limited due to cult airway No contraindications to routine anesthesia. anesthesia. routine to contraindications No fl Hemifacial with sympathetic ganglia interference due to Diffi diffi have obvious preoperatively; available on given medications are anti-seizure Administered assess cardiac Preoperatively the morning of surgery. and ECG) for cardiac status (echocardiogram Comprehensive function or evidence of myocarditis. electrolyte, Correct respiratory assessment required. have May abnormalities. and coagulation acid-base, requiring dysfunction) (gastrointestinal full stomach Short-acting anesthetics that do not require RSI. function recommended. normal hepatic or renal monitoring of continuous biochemistry Intensive and postoperatively needed intraoperatively Anesthesia implications unilateral ushing due to ciency. Central nervous ciency. lequin syndrome combined with Horner’s syndrome in a pediatric syndrome patie with combined Horner’s lequin syndrome cult airway management in a child with Hecht-Beals syndrome. Indian J Anesth. 2012;56(6):591–2 Anesth. J Indian syndrome. with management in a child cult airway Hecht-Beals See Histiocytosis X See Histiocytosis with line bisecting the changes demarcation Skin color and fl sweating Hemifacial body. sympathectomy and kyphoscoliosis, arachnodactyly, delay, Developmental joint contractures congenital multiple syndrome See Kasabach-Merritt (usually prodromal 2-year-olds; in 1- to occurs Usually sudden onset of by infection followed gastrointestinal) All and thrombocytopenia. hemolytic anemia, failure, renal system—severe cardiovascular be involved: may systems failure; cardiac and congestive myocarditis, hypertension, respiratory-pulmonary insuffi and seizures, drowsiness, to progressing depression system: with hepatic dysfunction, Hepatosplenomegaly coma. thrombocytopenia, Coagulopathy: and coma. seizures, time, prothrombin prolonged function, platelet decreased renal blood transfusion, is by Treatment and bleeding time. and symptomatic therapy for other disorders dialysis, Description Paediatr Anaesth. 2007;17(6):597–8 Anaesth. Paediatr Name Hand-Schüller-Christian syndrome Harlequin syndrome har postoperative Transient et al. Cho JE, WO, Kim HK, Kil Hecht-Beals syndrome Ana Paediatr management ofAnaesthetic paediatric syndrome. two patients with Hecht-Beals et al. R, Shiro A, Tateoka O, Nagata Hemangioma with thrombocytopenia Hemolytic syndrome uremic 1987;34:196–9 Anaesth. Can J The haemolytic and anesthesia. uraemic JK. syndrome Rosales GD, Johnson 2014;7:231–9 Dis. Renovasc J Nephrol Int of Management in children. hemolytic-uremicGrisaru syndrome S. Kumar A, Chandran R, Khanna P, et al. Successful diffi Successful et al. Khanna P, Chandran R, A, Kumar 558 Appendix A: Anesthesia Implications of Syndromes… uid cult; have have cult; ndings and literature review. Curr Curr review. ndings and literature ions during Caesarean section. Euro J Euro section. ions during Caesarean ice for the anesthesiologist. J Clin for the anesthesiologist. ice ciency. Metabolism. 1986;35(7):627–33 Metabolism. ciency. be diffi intubation may brosis; of Beware cartcult airway loose teeth at hand. c anesthesia complications reported. Monitor Monitor reported. c anesthesia complications Preoperatively assess metabolic status and history. No No assess metabolic status and history. Preoperatively specifi glucose-containing and administer levels glucose acid-base if check solution; perioperatively indicated Correct anemia and coagulation defects. Assess Assess defects. anemia and coagulation Correct Check electrolytes and fl cardiorespiratory status. history of therapy. have steroid May balance. Laryngeal fi Anesthesia implications the diffi Monitor coagulation. Careful preoperative assessment preoperative Careful coagulation. Monitor transfusion platelet require of May pulmonary status. during surgery ltration: ammation and ammation ciency revisited. Neuromuscul Disord. 2010;20(2):125–7 Disord. Neuromuscul ciency revisited. brosis brosis mia, purpura, hemorrhage; purpura, mia, by the Clot Signature Analyzer: a potentially valuable dev a potentially Analyzer: the Clot Signature by Hepatomegaly; increased liver glycogen concentration. concentration. glycogen liver increased Hepatomegaly; development. motor and delayed growth retardation Minor children Many occur. may hypoglycemia moderate to Mild on a special diet. asymptomatic and lead normal lives are with severe disease status is more Occasionally rarely a form of this Very and acidosis. hypoglycemia neonatal hypoglycemia cause severe disease may hepatic involvement. Pulmonary-diffuse hilar infi hepatic involvement. insipidus if with Diabetes loss of sella turcica necrosis, teeth. and/or radiotherapy be on chemotherapy May involved. See epidermolysis bullosa Abnormality platelet bleeding diathesis due to Albinism: pulmonaryand life-threatening fi disease Gierke See von Osteolytic lesions in bones and viscera lungs, (larynx, leukemia. acute similar to Clinical course and spleen). liver, ane pancytopenia, Hypersplenism, Gingival infl pulmonale. cor respiratory failure, Description (glycogen (glycogen (eosinophilic ciency storage disease type VIII) disease typestorage Name Hepatic phosphorylase kinase defi Clinical and laboratory observations et al. with in a child hepatic phosphorylase BA, Burke kinase defi BI, Brown M, Tuchman Herlitz syndrome Hermansky-Pudlak syndrome of Evaluation hemostasis et al. Sevarino FB, CM, Ayoub AS, Haddadin Hers disease Histiocytosis X granuloma: Hand-Schüller- Letterer- Christian disease, disease) Siwe management ofAnesthetic a patient with histiocytosis X and pulmonary et al. complicat C, Greim O, Eichelbroenner J, Broscheit Echaniz-Laguna A, Akman HO, Mohr M, et al. Muscle phosphorylase b kinase defi Muscle et al. M, Mohr Akman HO, A, Echaniz-Laguna 2004;21(11):919–21 Anaesth. 2015;41(4):320–31 Rev. Treat Cancer review. a systematic sarcoma: Langerhans cell et al. L, Masterson RC, Dwivedi JE, Howard Monit Comp. 1999;15(2):125–9 Comp. Monit fi tomography computed high-resolution syndrome: Hermansky-pudlak TL. Mohammed S, Mohammed Faheem Saeedan M, Bin 2015;44(4):383–5 Diagn Radiol. Probl Appendix A: Anesthesia Implications of Syndromes… 559 cult cult cartcult airway Table continues on the following page. continues on the following Table

position BP cuffcult to osis type II). Paediatr Anaesth. Anaesth. osis type Paediatr II). cult airway cart at hand. Delayed Delayed cartcult airway at hand. Anaesth. 1994;41:423–6 Anaesth. with Consult maintain urineuids to output. cult venous cannulation. Potential diffi Potential cannulation. cult venous available. May be diffi May available. intubation, check airway; have the diffi airway; check have intubation, intubation due to large tongue. Attempts to secure secure to Attempts large tongue. intubation due to been not always using the LMA have airway diffi have successful; anesthesia and postobstructive from recovery pulmonary edema has been reported Preoperatively assess cardiac status (echocardiogram assess cardiac Preoperatively be abnormal. may system limb venous Upper and ECG). Diffi Give fl risk of reduce hematologist for treatment options to blood reduce Dextran 40 may thromboembolism. and increase viscosity adhesiveness and platelet be may stockings Pneumatic peripheral perfusion. and dextrose Infuse stasis. venous prevent to indicated (impairs oxide nitrous Avoid levels. glucose monitor of methionine and to homocysteine conversion level) increases Diffi syndrome. as for Hurler Management Anesthesia implications ning from general anaesthesia in a patient with Hunter syndrome. Can J syndrome. general anaesthesia in a patient withning from Hunter Upper limb abnormalities; CHD in 80 % (usually ASD) but % (usually CHD in 80 limb abnormalities; Upper with occur anatomy; arrhythmias normal cardiac may possibility of pulmonary arrhythmia, sudden death from occlusion coronary embolus, intimal thickening; phenomena due to Thromboembolic Hypoglycemia kyphoscoliosis. osteoporosis, lentis, ectopia thrombosis, precipitate Angiography may occur. may especially cerebral (see page syndrome than Hurler but less severe Similar, See also mucopolysaccharidoses 561). Description

(heart-hand syndrome) Name Holt-Oram syndrome 1998;80(6):856–7 Anaesth. J Br syndrome. Holt-Oram et al. K, Kumano K, Higa Shono S, 2013;16:157–8 Anaesth. Cardiac Ann Syndrome. implications in Holt-Oram Anesthetic et al. Girotra S, VS, Pathania A, Singh Homocystinuria 1994;6(2):142–4 Anesth. implications ofAnesthetic J Clinical with the child homocystinuria. JD. Tobias DA, Johnson S, Lowe 1995;7(2):176 Anesth. J Clinical and administration of Homocystinuria oxide. nitrous DD. Koblin 2012;22(11):720–2 Pak. Surg Physicians management of J Coll Anaesthetic patient with homocystinuria. a young Ali FM. A, Asghar Hunter syndrome (mucopolysaccharid syndrome of Failure the laryngeal in a patient with the airway secure Hunter’s G. Fognani mask to P, Busoni (mucopolysaccharidosis (mucopolysaccharidosis type II) 1999;9:153–5 awake Delayed et al. P, Crespi Boorin MR, A, Kreidstein 560 Appendix A: Anesthesia Implications of Syndromes… cult beroptic scope scope beroptic Caution withltration. ndings. J Ped J Ped ndings. ew fi and cisatracurium agents ofurane, are intubation, especially in older children, due to due to especially in older children, intubation, of movement and limited short neck, micrognathia, airway advanced Have joint. temporomandibular and fi as GlideScope adjuncts such Preoperatively assess cardiac status (echocardiogram status (echocardiogram assess cardiac Preoperatively and chest prophylaxis Antibiotic and ECG). artery If coronary severe preoperatively. physiotherapy tachycardia, avoid dysfunction, disease or myocardial obstruction airway due to Upper atropine. do not give lymphoidprofuse tissue infi of hypoplasia the odontoid, neck movement; Diffi occur. subluxation may atlantoaxial not or oral may airways Nasal at hand. immediately be successfully LMA may An obstruction. relieve obstruction. relieve but does not always placed sevofl Propofol, lymphoid fail (due to Epidural analgesia may choice. deposition) Anesthesia implications cult airway in Hurler’s syndrome. J Anaesthesiol Clin Pharmacol. 2014;30(4):558–61 Clin Pharmacol. Anaesthesiol J syndrome. in Hurler’s cult airway ng, pectus excavatum, kyphoscoliosis. Abnormal Abnormal kyphoscoliosis. pectus excavatum, ng, Developmental delay, gargoyle facies, deafness, stiff deafness, joints, facies, gargoyle delay, Developmental dwarfi artery coronary cartilages; severe disease tracheobronchial involvement. and myocardial valvar at early age, respiratory and cardiac die from Most Hepatosplenomegaly. of after 10 years sudden death common before age; failure of See mucopolysaccharidoses 7 years age. See Scheie syndrome syndrome See Scheie See Progeria disease See Urbach-Wiethe Description

(type I HS) Surg.1993;28(3):403–8 of Management diffi an anticipated et al. S, Kishan Shailaja S, T, Gurumurthy Name Hurler syndrome and n mortality, morbidity, care, perioperative with Children mucopolysaccharidosis: et al. Carpenter BL, W, Krivit Belani KG, Hurler-Scheie compound syndrome (mucopolysaccharidosis type(mucopolysaccharidosis formerly classed as I H; type I) Hutchinson-Gilford Hutchinson-Gilford syndrome Hyalinosis, cutaneous-mucosal Appendix A: Anesthesia Implications of Syndromes… 561 jaw limited cult due to Table continues on the following page. continues on the following Table of idiopathic thrombocytopenic c anesthesia recommendations. c anesthesia recommendations. immediately scope beroptic ventilatory support from wean cult to May have history of steroid therapy. Platelet counts counts Platelet history of therapy. have steroid May transfusions but platelet are be very low, may intramuscular Avoid NSAIDs. Do not give ineffective. do not give injections (if is performed, splenectomy Preoperative until the spleen is out). platelets Prophylactic be indicated. may vaccine pneumococcal be indicated. may splenectomy antibiotics after Preoperatively assess cardiac status (echocardiogram status (echocardiogram assess cardiac Preoperatively intubation Tracheal and ECG) pulmonary status. diffi maintenance and airway stiffness of Caution neck and rib cage. movement, use in-line stabilization ifwith neck movement; as adjuncts such airway advanced Have indicated. and fi GlideScope specifi No available. be diffi May Anesthesia implications -globulin is γ gy Task Force. Guidelines for the investigation and management for the investigation Guidelines Force. gy Task ciency common. Atlantoaxial Atlantoaxial common. ciency cult intubation management in a child with I-cell disease. Saudi J Anaesth. 2010;4(2):105–7 Anaesth. Saudi J with disease. cult intubation management in a child I-cell

6 See stiff syndrome baby See Chap. is factor an antiplatelet disease in which Autoimmune in destruction resulting of in the spleen platelets present, May for bleeding. with and the potential thrombocytopenia or intracranial gastrointestinal severe or chronic; be acute in a few weeks. most recover in children; rare bleeding are of 10 year age. over in children likely ITP is more Chronic with and high-dose steroids Treatment effective in raising the platelet count (i.e., for a surgical (i.e., count in raising the platelet effective in is very recommended rarely Splenectomy procedure). children Description Developmental delay, Hurler-type bone changes, severe severe bone changes, Hurler-type delay, Developmental pulmonary cardiac chronic disease; joint limitation, insuffi valvar involvement, but some common Death in early childhood subluxation. survive 1 or 2 decades

Name Hyperekplexia Hyperpyrexia/ hyperthermia, malignant Diffi et al. Al-Bahlani SS, George G, AK, M Mahfouz Idiopathic thrombocytopenic purpura General in Haematology Haematolo for Standards British Committee 2003;120(4):574–96 J Haematol. Br and in pregnancy. children purpura in adults, I-cell I-cell disease (mucolipidoses) 562 Appendix A: Anesthesia Implications of Syndromes… cult airway airway cult -blockers to to -blockers β

gingivalcult intubation due to -blockade β urane. Propofol may improve rhythm improve may Propofol urane. brillation. Watch for hypoglycemia as a for hypoglycemia Watch brillation. lidocaine and to respond brillation may Preoperatively assess cardiac status (ECG); consult consult status (ECG); assess cardiac Preoperatively General anesthesia may cardiologist. with the child’s with arrhythmias; pretreat precipitate decrease risk. Avoid atropine, halothane, and halothane, atropine, Avoid risk. decrease desfl with and remifentanil propofol TIVA disturbances. Left be the optimal technique. (or other opioid) may in ganglion block has been recommended stellate Ventricular interval. the QT decrease adults to fi defi ofcomplication Check preoperatively for evidence ofCheck preoperatively other organ Diffi involvements. hyperplasia motion at neck and and limited the diffi have joints; temporomandibular cart at hand. Careful positioning and padding Careful cart at hand. required Anesthesia implications bromatosis. Paediatr Anaesth. 2000;10(6):682–4 Anaesth. Paediatr bromatosis. urane anesthesia on QTc interval and cardiac rhythm in children. Paediatr Anaesth. Anaesth. Paediatr intervalrhythm in children. urane and cardiac anesthesia on QTc urane anesthesia in a child with congenital long QT syndrome. Paediatr Anaesth. Anaesth. Paediatr syndrome. urane with long QT anesthesia in a child congenital bromatosis. Br J Anaesth. 1996;76(1):163–6 Anaesth. J Br bromatosis. urane and desfl urane on QT parameters in children with congenital sensorineural hearing loss. Anaesthesia. 2009;64(1):3–8 Anaesthesia. with sensorineural congenital in children hearing parameters loss. urane on QT brillation) under anesthesia. Acquired long QT syndrome syndrome long QT Acquired brillation) under anesthesia. of radiolucent contractures exion large and small joints, See asplenia syndrome See asplenia syndrome defects: conduction deafness and cardiac Congenital be misdiagnosed as arrhythmias attacks (may and syncopal interval. QT prolonged large T waves, ECG shows epilepsy). Serious arrhythmias (ventricular occur. death may Sudden fi of be a result may drug therapy Autosomal recessive disease; multiple subcutaneous nodules, subcutaneous nodules, multiple disease; recessive Autosomal fl bone destruction and humerus), (especially femur involve manifestations may Systemic hypertrophic gingiva. Entrapment of system. and digestive renal, lung, pleura, normal Intelligence occur. may nerves and vessels Description (Romano-Ward (Romano-Ward bromatosis bromatosis Name Ivemark syndrome and Lange-Nielsen Jervell syndrome long congenital syndrome, syndrome) QT 2007;17(6):563–7 Curry TB, Gaver R, White RD. Acquired long QT syndrome and elective anesthesia in children. Paediatr Anaesth. 2006;16(4):471–8 Anaesth. Paediatr anesthesia in children. and elective syndrome long QT Acquired RD. White R, Gaver Curry TB, during de pointes sevofl Torsade et al. Raczka F, I, Massad Saussine M, 2006;16(1):63–5 Effects of et al. sevofl CS, Kim JT, Kim HS, Kim The effects of et al. sevofl S, Ozer AH, Karagoz E, Aypar Juvenile hyaline fi fi hyaline and juvenile Anaesthesia N. Madden Soni N, B, Norman fi hyaline gingival hyperplasia in juvenile intubation for massive Fibreoptic B. Padwa JH, Ko Seefelder C, Appendix A: Anesthesia Implications of Syndromes… 563 c cult Table continues on the following page. continues on the following Table immediately scope beroptic ngle-institutional case series. Pediatr Pediatr ngle-institutional case series. me. Rev Bras Anesth. 2012;62(3):424–31 Anesth. Bras Rev me. but no other specifi cult airway Extreme care with in-line stabilization care intubation; Extreme adjuncts airway advanced Have be indicated. may and fi as GlideScope such (hypoplastic ventilation high-pressure Avoid available. necessitate Surgery enlarge thorax may to lungs). with Care periods ofprolonged ventilation. assisted kidneys by drugs excreted respiratory perioperatively. problems Life-threatening anesthetic agents and opioids. to sensitive Very and ventilation, controlled induction, Inhalational monitoring Apnea local or regional analgesia advised. be useful caffeine may postoperatively; status (echocardiogram). assess cardiac Preoperatively diffi have May anesthesia problems reported; have the diffi have reported; anesthesia problems airway cartairway at hand Anesthesia implications rst year of life. Arch Dis Child. 2015;100(2):158–64 Dis Child. Arch of rst year life. patients with Joubert syndrome: a retrospective analysis of a si a retrospective patients with Joubert syndrome: rt: anesthesia in patients with asphyxiating thoracic dystrophy: Jeune syndro Jeune rt: thoracic anesthesia in patients with dystrophy: asphyxiating congenital syndromes: clinical issues. Spine. 2009;34(17):1815–27 Spine. clinical issues. syndromes: congenital Severe thoracic malformation leading to neonatal asphyxia. thoracic neonatal asphyxia. malformation leading to Severe A high of rate cervical and instability spine stenosis is forms Milder pulmonaryAssociated hypoplasia. present. changes, renal Cystic in older children. present may failure renal to progressing vermis Cerebellar disorder. recessive autosomal Rare Hypotonia, cysts. or agenesis and brain stem dysplasia protrusion. and tongue jerky movements, eye ataxia, alternating respiration: Abnormal delay. Developmental be lethal in early May and apneic spells. tachypnea childhood % have 50 and craniofacial anomalies; delay Developmental hypotonia Muscular disease. renal % have CH D and 25 Scoliosis normal. biopsies are but muscle be present may in many develops Description (Mohr (Mohr

(asphyxiating thoracic (asphyxiating dystrophy) Case repo et al. D, Tonelli Grigio TR, Saletti D, Name Jeune syndrome 1987;66(1):86–8 Anesthesiology. thoracic dystrophy). (asphyxiating syndrome with Jeune’s for children Anesthesia Borland LM. Spine deformities in rare Jr. Campbell RM, Joubert syndrome management ofAnesthetic et al. S, Jena B, Vinay Sriganesh K, Kabuki syndrome 2007;17(9):900–1 Anaesth. Paediatr syndrome. with for a child Anesthesia Kabuki JF. Mayhew G, Johnson clinical and molecular diagnosis in the fi syndrome: Kabuki et al. Lepri FR, A, Di Pede Dentici ML, syndrome variant, familial variant, syndrome agenesis) vermis cerebellar 2014;24:1180–4 Anesth. 564 Appendix A: Anesthesia Implications of Syndromes… ol. 2012;66(4):291–4 ol. 7–19 3;57(3): 292–4 3;57(3): . Paediatr Anaesth. 2003;13(8):714–7 Anaesth. Paediatr . If any surgery is planned, prepare for major blood prepare surgeryIf any is planned, Correct be indicated. monitoring may invasive losses; FFP defects. and coagulation hypovolemia, anemia, help may Steroids transfusionsand platelet required. Order respiratory physiotherapy preoperatively. Use Use preoperatively. respiratoryOrder physiotherapy Assess isolation). (reverse aseptic technique careful be need to lung lobes may Affected respiratory status. for lobectomy isolated Anesthesia implications purpura, purpura, → brinolytic activity. Treated by by Treated brinolytic activity. brinogenemia cient mucociliary clearance; sinusitis, bronchiectasis. bronchiectasis. sinusitis, cient mucociliary clearance; radiotherapy (surgery may precipitate disseminated radiotherapy disseminated (surgery precipitate may with embolization, Treated coagulation). intravascular and sirolimus. and everolimus chemotherapy, steroids, destruction of follows tumor Recovery bleeding, anemia, increased fi increased anemia, bleeding, Dextrocardia, situs inversus. Immotile abnormal cilia, abnormal cilia, Immotile situs inversus. Dextrocardia, defi Defective surgery. sinus undergo endoscopic Often immunity in size; that suddenly increases Hemangioma hypofi thrombocytopenia, Description Name Kartagener syndrome syndrome with child Kartagener’s in an 8-year-old General anaesthesia for lobectomy et al. Thomas J, OP, Sanjay Sahajananda H, Kasabach-Merritt syndrome 1987;34(1):1 Prog. Anesth management ofAnesthetic syndrome. a patient with Kasabach-Merritt Akita S. T, Takeshita M, Kawahara Burduk PK, Wawrzyniak K, Kazmierczak W, et al. Kartagener’s syndrome—anaesthetic considerations for ENT surgery. Otolaryngol P for ENT surgery. considerations syndrome—anaesthetic Kartagener’s et al. W, Kazmierczak K, Wawrzyniak PK, Burduk 201 Anaesth. J Indian management of syndrome. Anaesthetic with a neonate Kasabach-Merritt et al. KN, Saxena B, Taneja S, Kumar Appendix A: Anesthesia Implications of Syndromes… 565 cult airway airway cult and lead II). Be prepared Be prepared and lead II). 5 Table continues on the following page. continues on the following Table the diffi have cult; h. 1996;6(3):235–8 h. p sedation. Paediatr Anaesth. Anaesth. Paediatr p sedation. r Pharm Des. 2008;14(13):1295–301 r Pharm Des. urane anesthesia has been used cart at hand. The use ofcart at hand. LMA is an option for securing calcium levels ionized Monitor the airway. perioperatively Administer anti-seizure medications on the morning anti-seizure Administer status assess cardiac Preoperatively of surgery. with cardiologist. consult and ECG); (echocardiogram as for a and anesthetize depressants myocardial Avoid for patient with artery Monitor coronary disease. (V changes ischemic cardiac be diffi may Intubation with vasoactive and antiarrhythmic drugs. Regional Regional and antiarrhythmic drugs. with vasoactive limb ischemic peripheral improve nerve blocks may Sevofl circulation. surgical increase may Salicylates satisfactorily. bleeding Anesthesia implications sm, macrocephaly, thoracic skeletal thoracic skeletal macrocephaly, sm, of the Kenny-Caffey syndrome using the laryngeal mask. Paediatr Anaest Paediatr using the laryngeal syndrome mask. of the Kenny-Caffey Acute febrile exanthematous disease secondary vasculitis febrile to exanthematous Acute dysfunction, valvular (pancarditis, with involvement cardiac artery and coronary arrhythmias, vasculitis with endemic in children, Seen in infants and young aneurysms). oral erythema, conjunctivitis, Signs include fever, Japan. Cardiac hands and feet. strawberry and red tongue, % of asymptomatic ranges from cases: in 20 involvement and massive failure congestive severe to ECG changes and used in treatment are Salicylates infarction. myocardial Biliary tract lesions. or bowel coronary reduce may in involvement Hepatic laparotomy. require symptoms may % ofCNS vasculitis with symptoms ranging patients. 10 and death reported seizures to headache from Dwarfi intellect. Normal aspiration recurrent hypocalcemia, anemia, abnormalities, mandibular hypoplasia and can have Description Name Kawasaki disease lymph node (mucocutaneous syndrome) general having anesthesia or dee syndrome review ofA 15-year with Kawasaki’s children et al. Chan KC M, Anderson JE, Morrison Kenny-Caffey syndrome management Anaesthetic Lewis IH. JE, Fletcher EL, Janke 2005;15(12):1053–8 Cur in treatment. in pediatric concepts current rheumatic diseases: Central involvement nervous system A. Bakkaloglu A, Duzova 566 Appendix A: Anesthesia Implications of Syndromes… beroptic beroptic cult because cult ndrome. J Clin Anesth. Anesth. J Clin ndrome. . 2011;113:98–102 . cart cult airway available laryngoscope, LMS, etc) Do not extubate until fully Do not extubate etc) LMS, laryngoscope, awake No anesthesia problem reported, except as related to to as related except reported, anesthesia problem No spinal cord prevent to very carefully Position diabetes. damage (osteoporosis) and (echocardiogram assess cardiac Preoperatively be very diffi may Intubation status). renal “awake/ of should be carried immobile neck: out if otherwise possible; inhalation induction sedated” airway advanced Have without relaxant. muscle fi devices at hand (GlideScope, immediately correct status (echocardiogram); cardiac Assess for major transfusion. Be prepared coagulopathy. have intubation usually not a problem; airway, Assess the diffi Anesthesia implications n and airway management: a case series management: ofn and airway sy Klippel-Feil highstulas and anemia lead to ned to males. Sex chromosome defect (47 XY. XXY). XXY). defect (47 XY. chromosome Sex males. ned to See maple syrup urine disease Confi reduced Tall, ambiguous genitalia at birth. have May vertebral hypogonadism, problems, behavior intelligence, mellitus. diabetes have May osteoporosis. due to collapse at puberty therapy is initiated replacement Testosterone fusion of cervicalCongenital or more two vertebrae, be may encephalomyelocele Occipital causing neck rigidity. Arnold-Chiari malformation in the neonate. associated and cardiac Rib defects, be associated. may and/or scoliosis related disease occasionally renal of with Hemangiomas bone; hypertrophy adjacent fi AV thrombocytopenia. with failure; possible cardiac output, cardiac in association withthrombocytopenia visceral and pectus scoliosis, Macrocephaly, hemangiomas. hemangiomas from occur bleeding may Severe excavatum. See leukodystrophy Description

(globoid cell

2008;20:447–51 Name Ketouria, branched-chain Klinefelter syndrome with(gonosomal aneuploidy tubular dysgenesis) 2005;72(11):2259–62 Phys. Fam Am syndrome. Klinefelter M. Menke DJ, Wattendorf Klippel-Feil syndrome cervical Congenital spine fusio GA. Mashour V, Vanaharam Stallmer ML, Klippel-Trénaunay-Weber syndrome (angio-osteohypertrophy) Analg Anesth a review of syndrome: 136 anesthetics. Klippel-Trenaunay for surgeryAnesthesia to related JL. Wilson Barbara DW, Krabbe disease leukodystrophy) leukodystrophy) Appendix A: Anesthesia Implications of Syndromes… 567 cult and cult xation the cult airway; have Table continues on the following page. continues on the following Table 1973;126:119–26 culty and fi with IV access cult airway cart at hand. An LMA will An be most cartcult airway at hand. beroptic laryngoscope, LMS, etc.). Caution with etc.). LMS, laryngoscope, beroptic Limited mouth opening, diffi mouth opening, Limited diffi thermal instability; to air use forced Prone helpful. Diffi heater. and (echocardiogram) assess cardiac Preoperatively be diffi may Intubation respiratory status. advanced Have be present. subglottic may stenosis (GlideScope, devicesairway available immediately fi cervicalneck; spine instability; use in-line stabilization ICP increased Possible during laryngoscopy. and (echocardiogram) assess cardiac Preoperatively status renal Anesthesia implications at face, face, at Disorder of Disorder scaling; with keratinization widespread severe dental disease likely severe hips. elbows, knees, dislocations: congenital Multiple fl cleft palate, hydrocephalus, Characteristic facies, tissue defect of cartilage Connective of upturned nose. ribs, Cervical arytenoids, and tracheomalacia. epiglottis, spine respiratory chronic kyphoscoliosis, abnormal and unstable, and CHD problems, pigmentary retinopathy, delay, Developmental and (Polydactyly and spastic paraplegia. hypogenitalism, absent.) May are typical syndrome, in Bardet-Biedl obesity, abnormalities and CHD renal have Description Name Lamellar ichthyosis 2012;22:492–4 Anesth. Pediatr in lamellar ichthyosis. challenges Anesthetic VV. Pai VM, Annigeri HV, Hegde Larsen syndrome 2002;12(7):632–6 Anaesth. Paediatr and its anaesthetic considerations. Larsen syndrome Choudhry DK. P, Malik Laurence-Moon-Biedl syndrome J Dis Child. Am of less than 3 years age. children of Report unrelated two syndrome. Laurence-Moon-Biedl GR. Hogan Banman ML, 568 Appendix A: Anesthesia Implications of Syndromes… cult airway airway cult the diffi have cult; diothorac Vasc Anesth. Anesth. diothorac Vasc r Anaesth. 2006;16(1):38–42 r Anaesth. i J Anesth. 2012;6:181–5 Anesth. i J cart on hand Preoperatively, assess pulmonary status and treat Preoperatively, give hydration, adequate Ensure infections. acute Use levels. glucose and monitor infusion, dextrose solution. Ringer’s lactated normal saline and avoid in the ventilation Monitor acidosis. Treat may and remifentanil Propofol period. perioperative procedures be useful for many and (echocardiogram assess cardiac Preoperatively and tachycardia Avoid ECG) and respiratory status. support to Be prepared drugs. depressant myocardial arrhythmias; have function and treat cardiac drugs and antiarrhythmic drugsvasoactive on hand. be diffi may Intubation Anesthesia implications etic management in complicated endoscopic procedures. Paediat procedures. endoscopic etic management in complicated A genetic neurologic and metabolic disease considered to be a to and metabolic disease considered A genetic neurologic in infancy or childhood. occur may myopathy mitochondrial optic atrophy, somnolence, hypotonia, develop Infants respiratoryAltered and pyramidal tractdeafness, signs. sudden infant death lead to and may occur may patterns intracellular metabolism secondary to Impaired syndrome. acute have Older children involvement. mitochondrial General deteriorationneurologic and respiratory failure. respiratory and death by failure be followed anesthesia may large freckles; multiple syndrome; A cardio-cutaneous progressive CHD, deafness. ptosis, eyelid hypertelorism, (pulmonary in stenosis hypertrophic cardiomyopathy serious ECG anomalies include aberrant %); conduction; 95 common; retardation Growth occur. arrhythmias may Genitourinary in some. etc., kyphosis, pectus carinatum, ovarian anomalies (hypospadias cryptorchidism, etc.) hypoplasia, Description (subacute (subacute Name Leigh disease Saud review. Case report and literature in Leigh disease: considerations Anesthetic et al. KM, Al-Shuaibi TM, Wani AS, Terkawi LEOPARD syndrome a review of J Car syndrome: forty-nine anesthetic exposures. and LEOPARD Anesthesia et al. TN, Weingarten ED, Wittwer TY, Yeoh necrotizing necrotizing encephalomyelopathy) anesth Leigh syndrome: et al. S, Shafran-Tikva Goldin E, D, Gozal 2014;28:1243–50 2004;14(4):352–6 Anaesth. Paediatr syndrome. implications ofAnaesthetic LEOPARD JD. Tobias P, Russo J, Torres Appendix A: Anesthesia Implications of Syndromes… 569 cult urane, and urane, the diffi have cult; Table continues on the following page. continues on the following Table tions. Nephron Physiol. 2014;10:24 Physiol. Nephron tions. Use drugs excreted by the kidney with caution. Beware Beware the kidney with by caution. drugs excreted Use of metoclopramide. regurgitation and give management. for behavior Benzodiazepines isofl thiopental, propofol, Midazolam, cisatracurium degradation (Hoffman independent of Caution with recommended. are dysfunction) renal catecholamines Assess for multiple organ disease. Administer Administer organ disease. for multiple Assess blood solutions and monitor glucose-containing be diffi may Intubation glucose. airway cart available. Use drugs excreted by kidneys by drugs excreted Use cartairway available. with caution Anesthesia implications impaired impaired renal → withn face mandibular hypoplasia. cult airway in Lesch-Nyhan syndrome. J Anaesthesiol Clin Pharmacol. 2012;28(2):239–41 Clin Pharmacol. Anaesthesiol J syndrome. in Lesch-Nyhan cult airway A severe insulin-resistance disease due to a mutant insulin a mutant disease due to insulin-resistance A severe gene with as such endocrine associated disorders receptor Elfi hypothyroidism. delay. developmental and severe thrive to Failure hyperplastic hyperinsulinism from due to Hypoglycemia islets of tubular defects renal Langerhans; ofin males. occurs Disorder purine metabolism, malnutrition, and growth retardation, delay Developmental basal ganglia volume. secondary reduced to choreoathetosis behavior. self-destructive with compulsive aggressive Very RBC damage, calculi, renal leads to Hyperuricemia gouty artery and coronary arthritis, hypertension, disease. Renal has been reported. tracheal diverticulum Associated age of by failure 20 years function with nephrocalcinosis due to hypercalciuria. hypercalciuria. function with due to nephrocalcinosis of 1 year die before age Most be dysmorphic. may Lungs Description (Donohue (Donohue Name Leprechaunism syndrome) 1965;66:372–9 J Pediatr. syndrome). (Donohue’s Leprechaunism Szijarto L. I, Lakatos A, Kallo INSR muta and the kidney: in patients with nephrocalcinosis recessive receptor Insulin et al. F, Cameron E, Cochran A, Simpkin Lesch-Nyhan syndrome 1985;63(2):197–9 Anesthesiology. syndrome. and the Lesch-Nyhan Anesthesia RG. Wilkins Larson LO, diffi unanticipated An et al. A, Tyagi Sharma C, Salhotra R, [Epub ahead of[Epub print] 570 Appendix A: Anesthesia Implications of Syndromes… 2007;17:64–9 strophy. Tex Heart Inst. Inst. Heart Tex strophy. p syndrome). Pediatr Anesth. Anesth. Pediatr p syndrome). hromatic Leukodystrophy patients. Mol Mol patients. Leukodystrophy hromatic Administer anti-seizure medications on the morning anti-seizure Administer assess pulmonary status and Preoperatively of surgery. If are oral secretions medications. anticonvulsant Danger of use an antisialagogue. pulmonary copious, antiemetics. and give suction the stomach aspiration; succinylcholine Avoid and pad carefully. Position risk of(theoretical Seizure hyperkalemia). to responses in altered result medications may glucose- Give relaxants. induction agents and muscle Ringer’s lactated solutions and avoid containing awake, Extubate temperature. body Maintain solution. Adrenal (N.B. closely postoperatively. monitor steroids. give in adrenoleukodystrophy; dysfunction for be appropriate epidural analgesia may Lumbar acceptable infusions are pain.) Propofol postoperative for MRI renal, (echocardiogram) assess cardiac Preoperatively for diabetes; Considerations status. and coagulation Caution with drugs blood glucose. monitor the kidneys by and those excreted liver by metabolized Anesthesia implications brotic liver leading to failure, failure, leading to liver brotic myocardial infarction secondary to congenital generalized lipody secondary congenital to infarction myocardial hild with congenital generalized lipodystrophy (Berardinelli-Sei hild with generalized lipodystrophy congenital with metachromatic leukodystrophy undergoing MRI. Pediatr Anesth. Pediatr undergoing MRI. leukodystrophy with metachromatic aspiration pneumonia. ux lead to acid–base balance and predisposition to metabolic acidosis in Metac to and predisposition acid–base balance ciency of progressive arylsulfataseA. See histiocytosis X of formation disorder lysosomal storage Inherited myelin a defi due to poor gait disturbance, disease with spasticity, degenerative extrapyramidal movements, seizures, development, motor swallowing and Disordered and choreoathetosis. refl gastroesophageal fi Generalized loss of fat, all body Malnutrition and anemia. Acid-base abnormalities may abnormalities may Acid-base and anemia. Malnutrition occur diabetes. nephropathy, portal splenomegaly, hypertension; anemia lead to may Hypersplenism failure. renal have May cardiomyopathy dilated develop May and thrombocytopenia. Description

(Alexander (Alexander 2008;35(2):196–9 2012;22:299–300 and cardiomyopathy Dilated Khalil J. MC, Mourtada WI, Khalife Name Letterer-Siwe disease Leukodystrophy disease, Canavan disease, Pelizaeus- disease, Krabbe disease, Merzbacher adrenoleukodystrophy, metachromatic leukodystrophy) 2003;13(8):733–4 Anaesth. Paediatr leukodystrophy. with metachromatic management in children Anaesthetic J. Hernandez-Palazon Sedation for children et al. Baldoli C, Gemma M, C, Mattioli with diabetes Lipodystrophy Berardinelli- (Seip syndrome, Seip syndrome) anesthesia in a c from emergence Delayed M. Allford Bennett T, Lorioli L, Cicalese MP, Silvani P, et al. Abnormalities of Abnormalities et al. P, Silvani MP, Cicalese Lorioli L, Genet Metab. 2015;115(1):48–52 Genet Metab. Appendix A: Anesthesia Implications of Syndromes… 571

++ ). Use reduced doses reduced Use ). ++ Table continues on the following page. continues on the following Table iatr Anesth. 2014;24:1302–4 iatr Anesth. Fanconi’s syndrome. Paediatr Paediatr syndrome. Fanconi’s eys-Dietz syndrome. J Bone Joint Surg Surg J Bone Joint eys-Dietz syndrome. ). Caution with drugs excreted by kidneys by Caution with drugs excreted ). cartcult airway at hand and use in-line + culty with intubation and unstable cervical spine; Assess for cervical spine subluxation preoperatively. for cervicalAssess spine subluxation preoperatively. Diffi diffi have and hypertension. tachycardia Avoid stabilization. complicate may antihypertensives Concurrent Drugs manage hyper/ to anesthesia unless withheld. regional Consider should be at hand. hypotension blocks when appropriate and correct Check electrolyte and acid-base balance serum Ca and low acidosis and hypokalemia Anesthesia implications of use a blockade non-depolarizing relaxants; muscle Avoid Caution with opioids. monitor. infusion (decreases glucose hyperventilation or excess serum K (treated with(treated vitamin D and Ca ative management of diagnosed have to ative syndrome a patient with Lowe See Farber disease This is an inherited defect in transforming growth aortic in dilated root, resulting receptors factor-beta easy bruising. joint laxity, tortuous vessels, hypertension, Cervical spine instability and focal kyphosis is common (Romano-Ward syndrome and Lange-Nielsen See Jervell syndrome) delay; developmental glaucoma, Cataract, males. Affects sodium and proteinuria, tubular acidosis, renal hypotonia, and rickets. osteoporosis, potassium wasting, failure renal leads to Glomerulosclerosis Description

(similar to Marfan’s) Marfan’s) (similar to Name Lipogranulomatosis Loeys-Dietz syndrome Ped instability a case report. duringwith anesthesia in an adolescent Loeys-Dietz Hemodynamic syndrome: Bould MD. AC, Bunting syndrome Long QT Lowe syndrome (oculocerebrorenal syndrome) and perioper Preoperative U. Aypar Demirtas F, Saricaoglu F, Fuhrhop SK, McElroy MJ, Dietz HC, III et al. High prevalence of High prevalence cervical III et al. deformity in Lo surveillance Dietz HC, and instability requires MJ, McElroy SK, Fuhrhop 2015;97(5):411–9 Am. Anaesth. 2004;14(6):530–2 Anaesth. 572 Appendix A: Anesthesia Implications of Syndromes… ely and postoperatively. ely and postoperatively. cart cult airway available Anesth. 2011;21:1269–70 Anesth. diffi culty; have Be alert and for for hepatic dysfunction perioperativ hypoventilation but intubation is usually without Check airway, diffi Transport and position carefully, protect protect and position carefully, Transport orthostatic show hypotension May hemangiomata. Careful drugs. vasodilator to and be sensitive Caution with fractures. avoid positioning to hemangiomata)intubation (airway Check acid-base endocrinologist. the child’s Consult Check plasma amino acids preoperatively. balance, anesthesia. and after during, serum before, glucose Start infusion (at least 10–15 mg/kg/min) glucose is until oral intake and continue preoperatively has been TIVA overhydration. Prevent reestablished. recommended Anesthesia implications -mannosidases A and α ciency of ciency

6 lysosomal accumulation of lysosomal accumulation mannose-rich substrates. → B immunologic function. neutrophil Abnormal and early infections, recurrent severe Hepatosplenomegaly, Hurler-like delay, developmental loss, Hearing death. clumsy motor facies, gargoyle-like changes, skeletal tissues connective weak function, See Treacher Collins syndrome syndrome Collins See Treacher Primary metabolic defi See collagen diseases See collagen and hemangiomas with malignantEnchondromatosis bleeding from gastrointestinal fractures, Pathologic change. orthostatic hypotension hemangiomas, See page Chap. and valine isoleucine, leucine, metabolize Inability to ofaccumulation amino acids and keto branched-chain damage and respiratory neurologic severe acids lead to diet by Treated of Episodes hypoglycemia. disturbances. episodes with due stress life-threatening Acute, birth. from and may infection or surgery ketoacidosis to lead to may transfusion dialysis or exchange peritoneal require Description

type I Mandibulofacial Mandibulofacial dysostosis Mannosidosis type II (milder) (severe), Name Lupus erythematosus disseminatus Maffucci syndrome 1998;26(5):586–9 Care. Intens Anaesth syndrome. implications ofAnaesthetic Maffucci’s et al. AM, Cho Ng SK, Chan SK, Malignant hyperpyrexia/ hyperthermia for patients withAnesthesia alpha-mannosidosis--a case series of Pediatr et al. 10 patients. J, Roed LG, Borgwardt P, Hallas syrupMaple urine disease branched-chain (MSUD; ketonuria) 1996;51(6):575–8 Anaesthesia. management in maple syrup Anaesthetic urine disease. et al. Akkus O, M, Ercan S, Kahraman Appendix A: Anesthesia Implications of Syndromes… 573 cult Table continues on the following page. continues on the following Table idoses. Pediatr Anesth. Anesth. Pediatr idoses. cult and the cervical spine is. Anesth Analg Analg Anesth 2002;95(2):331–2 is. Anesth. 2010;24(6):945–8 Anesth. cult airway cart available. May May cartcult airway available. nt. Int J Cardiol. 2013;167(1):1–10 J Cardiol. Int nt. . 2013;96(3):1070–2 . See mucopolysaccharidoses. Assess cardiac cardiac Assess See mucopolysaccharidoses. and ECG) pulmonary(echocardiogram status hemoglobin counts. Assess and platelet X-ray). (chest Spinal cord drugs. with depressant Care cardiac challenging have May occur. may compression diffi have intubation; postoperatively ventilation require Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively be diffi may Intubation Laryngoscopy joint. unstable at the atlantoaxial cervical prevent spine or should be gentle to The use of joint damage. advance temporomandibular devicesairway and in-line stabilization is diffi leading to Tracheomalacia recommended. to carefully Position has been described. ventilation depressants, myocardial Avoid dislocations. prevent hypertensive become the patient to but do not allow (danger of of Beware aortic dissection). pneumothorax ventilation with controlled Anesthesia implications ngers, ngers, broelastosis and broelastosis tissue brillin causes connective conduction defects; heart failure by 20 years of 20 years heart by defects; age failure conduction Tall, thin predominantly male patients with thin predominantly long fi Tall, with poor lung reserve; Kyphoscoliosis intellect. Normal anemia, respiratory hypersplenism, infections; chronic with heart involvement Myocardial thrombocytopenia. fi cardiomyopathy, thickening, valve long face, and high arched palate. Mutant gene at Mutant palate. and high arched long face, 15 for fi chromosome joint instability leading to and dislocation disorder dislocation of (including cervical spine), lens, High lung cysts. pectus excavatum, hernia, kyphoscoliosis, Aortic %) of spontaneous pneumothorax. (~4 incidence or aneurysm; aortic lead to incompetence dilation may root pulmonary artery be diseased may or mitral valve Description

(mucopolysaccharidosis (mucopolysaccharidosis type VI) Name Marfan syndrome (arachnodactyly) 2008;117(21):2802–13 Circ. management of syndrome. Medical Marfan RE. Pyeritz MG, Keane during of general syndrome anesthesia for correction tracheomalacia in Marfan scolios Unexpected et al. BK, Kim YH, Kim AY, Oh Maroteaux-Lamy syndrome J syndrome). VI (Maroteaux-Lamy type in a patient withAnesthesia mucopolysaccharidosis et al. M, Nakasuji Okutani R, SH, Suh Hiebert JD, Auld BC, Sasaki T, et al. Infant repair of massive aortic aneurysm with prosthetic valved conduit. Ann ThoracAnn Surg of aortic repair Infant aneurysm conduit. massive with et al. valved prosthetic Sasaki T, BC, Auld Hiebert JD, pulmonary with oedema during mucopolysacchar anaesthesia in children Postobstructive et al. DN, Robinson V, Colovic RW, Walker 2003;13(5):441–7 diagnosis guide to and treatme a cardiologist’s VI: type A Mucopolysaccharidosis et al. V, Opoka-Winiarska A, Jurecka A, Golda 574 Appendix A: Anesthesia Implications of Syndromes… cult rule out lms to cartcult airway available. 1998;45(7):660–3 lg. 2008;107(2):422–7 lg. atlantoaxial instability. Airway problems and diffi problems Airway instability. atlantoaxial the diffi have intubation; in-line stabilization during laryngoscopy. require May withAssociation laryngomalacia and tracheomalacia require May described as a cause of ventilate. to failure oropharyngeal or nasopharyngeal during airway Cautious use of muscle induction and recovery. Caution with ifrelaxants present. hypotonia fragilepositioning due to bones Flexion/extension lateral neck fi Flexion/extension and drugs cause mast cell stimuli known to Avoid treatment with Prophylactic degranulation. and ranitidine, diphenhydramine, antihistamines (i.e., has been and steroids (Singulair)) montelukast be safely anesthetics may Inhalation recommended. fentanyl, succinylcholine, rocuronium, Propofol, used. be used may and/or meperidine (Demerol) remifentanil, dextran, anticholinergics, amphotericin B, Avoid safely. atracurium, ketorolac, vancomycin, dextromethorphan, tetracaine, chloroprocaine, benzocaine, succinylcholine, Bleeding secondary to caution with NSAIDs. procaine; therapy protamine require heparin may release Anesthesia implications ic management of a child with Marshall-Smith syndrome. Can J Anaesth. Anaesth. Can J ic management of syndrome. with a child Marshall-Smith ux. Anesthesia for minor surgical procedures have led have for minor surgicalAnesthesia procedures ux. cult airway in a patient with Marshall-Smith syndrome. Paediatr Anaesth. 1998;8(5):429–32 Anaesth. Paediatr syndrome. in a patient withcult airway Marshall-Smith Skeletal dysplasia and dysmorphic facial features including facial features and dysmorphic dysplasia Skeletal Possible thrive. to and failure Hypotonia micrognathia. Respiratory tract anomalies lead to instability. atlantoaxial nontraumatic to fractures Prone complications. of aggregates Abnormal histamine- and heparin-containing maculopapular skin lesion is a brownish-red mast cells; degranulation cell with Mast rash mainly on trunk. with occur histamine and heparin may systemic release and drugs (including alcohol, changes, temperature trauma, vancomycin polymyxin, papaverine, morphine, salicylates, with Often a history ofand atropine). gastroesophageal refl but most are and death, generalized anaphylaxis to uneventful Description Name Marshall-Smith syndrome Anaesthet et al. F, Veyckemans P, Pendeville Dernedde G, Mastocytosis syndrome (urticaria pigmentosa) Ana Anesth disease. anesthetic management for a complex routine mastocytosis: Pediatric et al. LM, Scott A, Uzzaman Carter MC, Klein NJ, Misseldine S. Anesthetic considerations in pediatric mastocytosis: a review. J Anesth. 2013;27:588–98 Anesth. J in pediatric a review. considerations Anesthetic mastocytosis: S. Misseldine Klein NJ, Antila H, Laitio T, Aantaa R, et al. Diffi et al. R, Aantaa Laitio T, H, Antila Appendix A: Anesthesia Implications of Syndromes… 575 the have cult; Table continues on the following page. continues on the following Table maintain urineuids to output. tr Anesth. 2013;23:817–23 tr Anesth. cult airway cart at hand. Care with drugs excreted with Care drugs cartcult airway excreted at hand. Preoperatively assess cardiac status (echocardiogram status (echocardiogram assess cardiac Preoperatively maintain infusion Do not use tourniquets; and ECG). of during surgery; dextrose do not use IV fl succinylcholine. drugs; with depressant Care cardiac shivering. Prevent ECG monitor status (echocardiogram). assess cardiac Preoperatively be diffi and intubation may Airway diffi or kidneys liver by Administer anti-seizure medications on the morning anti-seizure Administer rhythm with cardiac ECGAssess of surgery. IV give fasting, prolonged Avoid preoperatively. blood glucose and check infusion, dextrose its high due to fat propofol Avoid perioperatively. solution and use Ringer’s lactated Avoid content. normal saline Anesthesia implications ciency ciency. Paediatr Anaesth. 2006;16(12):1293–4 Anaesth. Paediatr ciency. ciency; not increased serum lactate brosis, and polydactyly. Most die Most and polydactyly. brosis, Muscle Muscle phosphorylase defi fatigability; increased to progresses Initially, exercise. by affected), muscles cramps (all skeletal muscle and weakness may Myocardium failure. renal lead to may myoglobinuria Patients been reported. ECG abnormalities have be involved; for MH with test the in vitro contracture positive test may but no reports been reported of clinical MH have and micrognathia, microcephaly, encephalocele, Occipital hepatic dysplasia, cystic renal bilateral CHD, cleft epiglottis, and fi ductal proliferation in infancy of mitochondrial enzyme. Hypoglycemia, seizures, or coma or coma seizures, of Hypoglycemia, enzyme. mitochondrial treated been reported have Tachyarrhythmias result. may with carnitine A disorder ofA disorder fatty acid metabolism secondary defi to Description

ciency

Name McArdle myopathy V) disease type storage and anesthesia—a case report disease (glycogen Pedia and review of the literature. McArdle’s Bollig G. Meckel syndrome 1998;35(6):497–501 Genet. J Med syndrome. Meckel P. Paavola Salonen R, acyl-CoA chain Medium defi dehydrogenase defi with dehydrogenase in a child acyl-CoA Anesthesia medium-chain JF. Mayhew AC, Justiz (glycogen storage disease storage (glycogen V) type (dysencephalia splanchnocystica) (MCAD) 576 Appendix A: Anesthesia Implications of Syndromes… cult cult airway airway cult culties; have diffi have culties; e. Braz J Anesthesiol. Anesthesiol. Braz J e. sum, holoprosencephaly and choanal and choanal holoprosencephaly sum, ciency. Anesth Analg. 1991;73(4):499–501 Analg. Anesth ciency. the diffi have cult (but not usually); Assess for associated defects. Cleft nose, lip, and palate and palate lip, Cleft nose, defects. for associated Assess cause intubation diffi may Intubation assess respiratory status. Preoperatively be diffi may Administer anti-seizure medications on the morning anti-seizure Administer Risk of suction the acid aspiration; of surgery. succinylcholine Avoid hypothermia. to Prone stomach. interact may Anticonvulsants disease). (neurologic Postoperative with non-depolarizing relaxants. muscle be required may ventilation Avoid excessive fasting or accumulation of fasting or accumulation blood in excessive Avoid volume. intravascular Maintain tract. gastrointestinal and ammonia level. electrolytes, blood gases, Monitor metabolic exacerbate (May oxide. nitrous Avoid defect) cart at hand. Caution withcart nasal instrumentation; at hand. be present may nasal encephaloceles Anesthesia implications airway cart available. May be sensitive to opioids, opioids, to be sensitive May cartairway available. ventilation Monitor occur. apnea may central postoperatively rst ome in association with hydrocephalus, agenesis of callo the corpus ome in association with hydrocephalus, aspiration leads to ux commonly cause chronic culties and aspiration may Various degrees of cleft face; lipomas, and dermoids over and dermoids over degrees of lipomas, cleft face; Various Other intracerebraloften deformities are bone. frontal be present may Choanal atresia present. Onset in fi of disorder metabolism. X-linked copper paralyses ofCongenital cranial nerves sixth and seventh micrognathia. Limb deformities, in inability smile. results to diffi Feeding with associated MH although Not pulmonary problems. this one case suggested months of life; retarded growth and developmental delay, months of delay, growth and developmental retarded life; degeneration. cerebral progressive seizures, refl Gastroesophageal pneumonia. Death from seizures or pneumonia in a few seizures Death from pneumonia. years Autosomal recessive defect of protein metabolism. Protein Protein defect of metabolism. protein recessive Autosomal acid levels, highmetabolism leads to plasma methylmalonic ketonemia, acidosis, dehydration, vomiting, lethargy, producing plus intake, limiting protein by Treated and hyperammonemia. andAnesthesia supplemental bicarbonate and cobalamin. acidemia metabolism and lead to protein surgery increase may Description

(kinky

ciency atresia. Euro J Ped. 1987;146(3):301–2 J Ped. Euro atresia. hair disease) oculofacial (congenital paralysis) Name syndrome cleft face Median syndr cleft face Median T. Eusterbrock W, Lenz Bomelburg T, Menkes syndrome 2008;18:1225–6 Anesth. Pediatr disease. with for a child Anesthesia Menkes P. Pietropaoli Almenrader N, M, Passariello Methylmalonyl-coenzyme A defi mutase defi A mutase management ofAnesthetic with a child methylmalonyl-coenzyme et al. R, Jack CM, Haberkern Sharar SR, Moebius syndrome 2006;18:55–9 Anesth. J Clin implications ofAnesthestic Syndrome. Moebius JD. Tobias Gondipalli P, of recrudescence malignant Fatal hyperthermia syndrom et al. in an infant with Moebius Cezar LC, WA, Filho Pinto CR, Fernandes 2013;63(3):296–300 Appendix A: Anesthesia Implications of Syndromes… 577 beroptic beroptic Table continues on the following page. continues on the following Table tr Anesth. 2012;22(9):901–7 tr Anesth. Check platelet count and hemoglobin. Assess BUN/ Assess and hemoglobin. count Check platelet IM Avoid supplement. steroid Possible creatinine. induction and intubation (prevent Smooth injections. hypertension as might cause CNS bleed). risk (due to intubation contraindicated Nasotracheal kidneys. by withof Care drugs excreted bleeding). (may transfusionsPlatelet should be avoided transfuse disease); withexacerbate PRBCs and FFP Preoperatively assess cardiac (echocardiogram and (echocardiogram assess cardiac Preoperatively with Care ECG) and pulmonary status (X-ray). stability atlantoaxial Assess drugs. depressant cardiac and use in-line stabilization during preoperatively airway advanced Have laryngoscopy if indicated. fi (GlideScope, devices available immediately laryngoscope, LMA, etc.). Care with Care positioning and etc.). LMA, laryngoscope, analgesia Regional neck manipulation. excessive avoid for some children be appropriate may Same as type IV: preparation and anesthetic preparation Same as type IV: Anesthesia implications aorticng; incompetence; Hemolytic anemia and thrombocytopenia, arteriolar and anemia and thrombocytopenia, Hemolytic disease. renal damage, neurologic capillary disease, for splenectomy and steroids; plasmapheresis Treatment: for historyAssess of therapy steroid cases. resistant Normal intellect. Severe dwarfi Severe intellect. Normal kyphoscoliosis with poor lung function (cardiorespiratory symptoms and pulmonary decade). second hypertension by spinal cord joint leading to atlantoaxial Unstable See hernia common. Inguinal deafness. compression; mucopolysaccharidoses Severe developmental delay. Skeletal anomalies similar to anomalies similar to Skeletal delay. developmental Severe type IV Description

-glucuronidase β ( ciency) Moschcowitz Moschcowitz disease (thrombotic purpura)thrombocytopenic defi Name Morquio syndrome 2002;12(7):641–4 Anaesth. Paediatr and its anaesthetic considerations. syndrome Morquio’s RE. Schwartz MA, Rehman KA, Morgan Pedia syndrome. of with complications and perioperative Morquio children care Anesthetic et al. C, Ditro T, Nerker MC, Theroux Mucopolysaccharidosis 1994;79(6):1203–5 Analg. Anesth management ofAnesthetic purpura. a patient with thrombocytopenic thrombotic EG. Pivalizza (mucopolysaccharidosis (mucopolysaccharidosis type IV) type VII 578 Appendix A: Anesthesia Implications of Syndromes… urane culty of airway hesia. 1994;49(12):1078–84 hesia. copolysaccharidoses. Pediatr Anesth. Anesth. Pediatr copolysaccharidoses. cult to intubate. LMA may not relieve not relieve LMA may intubate. cult to Hypocapnia leads to severe cerebral ischemia: prevent prevent ischemia: cerebral severe leads to Hypocapnia Isofl maintain normocapnia. hyperventilation, may be useful as a cerebral vasodilator, propofol may may propofol vasodilator, be useful as a cerebral may hypothermia. Prevent protection. cerebral provide and maintain maintenance) × (1.5 hydration Maintain be Scalp blocks may perfusion pressure. cerebral useful during neurosurgery All may be diffi All may occur may compression Spinal cord obstruction. because of of thickening dura and odontoid MRI of preoperative spinal cord hypoplasia; and status (echocardiogram cardiac Assess suggested. All are assess severityECG) to of dysfunction. cardiac (Early pulmonary postobstructive edema. subject to the diffi reduce therapy may cell stem management later) management later) Anesthesia implications V ed as type ofne network vessels ciency (see Morquio syndrome) syndrome) ciency (see Morquio cations lippo syndrome syndrome lippo -Glucuronidase defi -Glucuronidase β Severe carotid artery carotid with stenosis a fi Severe Initially the basal ganglia (“puffaround of smoke”). ischemia manifests as transient attacks leading to ischemic is with Treatment hemiplegia. paroxysmal and later and by aspirin blockers or calcium channel low-dose surgical revascularization using a scalp artery the pial to ofsurface the brain bones and intellect Affects only intellect Affects bones only Affects See previous classifi syndrome Hurler I H: formerly classifi syndrome; Scheie I S: syndrome) (see Scheie compound Hurler-Scheie HS: syndrome Hunter II: III: Sanfi syndrome Morquio IV: syndrome Scheie Formerly V: syndrome Maroteaux-Lamy VI: VII: Description Name Moyamoya disease 2005;15(12):1111–5 Anaesth. Paediatr disease and anesthesia. Moyamoya et al. ZS, Ustalar Ozgen S, N, Baykan Mucopolysaccharidoses VII II, I HS, I H, Type Type III VI IV, I S, Type Anaest A review of in children. problems airway and mucopolysaccharidoses. Anaesthesia et al. P, Morris M, Darowski RW, Walker Frawley G, Fuenzalida D, Donath S, et al. A retrospective audit of anesthetic techniques and complications in children with mu in children audit of and complications anesthetic techniques A retrospective et al. Donath S, D, Fuenzalida G, Frawley 2012;22:737–44 Appendix A: Anesthesia Implications of Syndromes… 579 c Table continues on the following page. continues on the following Table . 1988;35:90–2 . Monitor glucose levels. Infuse dextrose. No specifi No dextrose. Infuse levels. glucose Monitor been reported have anesthesia complications Caution with all muscle relaxants. Succinylcholine Succinylcholine Caution with relaxants. all muscle in very highresults or hyperkalemia and CK levels should be avoided support be required may Ventilatory relaxants: of Possibility cholinergic crisis with postoperatively. therapy anticholinesterase Anesthesia implications Neither glycogen nor glucose can be used as metabolic nor glucose glycogen Neither muscle rhabdomyolysis, myoglobinuria, have May fuels. hemolytic anemia and intolerance, exercise cramps, RBC life span (13–16 days) Reduced hyperuricemia. Muscular dystrophy, eye disease (glaucoma, strabismus, strabismus, disease (glaucoma, eye dystrophy, Muscular muscle Severe delay. and developmental nystagmus), bedridden retention, secretion weakness, See page 364 gravis in older children. myasthenia to Similar Do not use respiratory or muscle depressants Description

(glycogen (glycogen ciency storage disease type VII) disease typestorage disease) (Tarui Name Multiple endocrine adenomatoses Type I II Type brain disease eye, Muscle, (MEB) Anaesth Can J and brain eye disease.” “muscle, with in children succinylcholine after kinase levels Serum creatine U. Karhunen Muscle phosphofructo- kinase defi syndrome See Sipple See Wermer syndrome 2007;26(2):105–7 Myologica. Acta clinical and genetic update. disease and distal glycogenoses: Tarui O. Musumeci A, Toscano Myasthenia congenita 2004;14(8):625–35 Anaesth. Paediatr gravis. with myasthenia in children for thymectomy Anesthesia Stoddart PA. MC, White Kose EA, Bakar B, Ates G, et al. Anesthesia for a child with Walker-Warburg syndrome. Brazil J Anesth. 2014;64:128–30 Anesth. Brazil J syndrome. Walker-Warburg with for a child Anesthesia et al. G, Ates Bakar B, EA, Kose 580 Appendix A: Anesthesia Implications of Syndromes… culty with culty c cartcult airway and beroptic intubation indicated. intubation indicated. beroptic lyses. Pediatr Anesth. 2013;23:824–33 Anesth. Pediatr lyses. cult due to contractures; have have contractures; cult due to xed; have diffi have xed; a review ofcans progressiva: 42 cases in cult airway cart at hand. Avoid succinylcholine succinylcholine Avoid cartcult airway at hand. Check respiratory function and history of steroid if and intubation problems Airway neck rigid therapy. and mouth fi Use short-intermediate-acting non-depolarizing Use succinylcholine; with avoid relaxants muscle caution; technique TIVA inhalation anesthetics; avoid use opioids with caution. recommended; due common respiratory complications Postoperative nerve blocks for analgesia Regional poor cough. to recommended positioning. Risks of MH and rhabdomyolysis are Risks of are positioning. MH and rhabdomyolysis unknown Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively be diffi may Airway diffi Diffi and caution with other relaxants. Anesthesia implications recommendations at www.IFOPA.org at www.IFOPA.org recommendations advanced airway devices available. Forced Forced devices airway available. advanced cause local progression manipulation of may the jaw of very disease; gentle fi all padding and prevent Careful IM injections. Avoid Specifi trauma joints and tissues. to brodysplasia ossifi brodysplasia cans progressiva. Br J Anaesth. 2006;97(5):701–3 Anaesth. J Br cans progressiva. brodysplasia ossifi brodysplasia ations for patients with myotonia congenital or periodic para congenital ations for patients with myotonia brillar myopathy. Pediatr Anesth. 2015;25:231–8 Anesth. Pediatr brillar myopathy. ltration of tendons, fascia, aponeuroses, and aponeuroses, ltration fascia, of tendons, Bony Bony infi thoracic greatly reduces Thoracic involvement muscle. Risk of respiratory any failure. progressive compliance: further minor trauma of causing progression disease Decreased ability to relax muscles after contraction; diffuse contraction; after muscles ability relax Decreased to dystrophica of myotonia hypertrophy (similar to muscle response Paradoxical benignbut more and nonprogressive). possible (generalized non-depolarizing relaxants to muscle (possible succinylcholine avoid spasms); muscle spasm) masseter hyperkalemia, Skeletal (including respiratory), cardiac, and smooth cardiac, (including respiratory), Skeletal and Contractures disease. in progressive involved muscle Risk ofrigidity respiratory muscles. skeletal in affected and sudden defects, conduction cardiomyopathy, failure, death Description

cans

cans brillar myopathy myopathy brillar brodysplasia ossifi brodysplasia progressiva) progressiva) (fi Name Myofi in myofi considerations Anesthetic Lopez G. Latham GJ, Myositis ossifi management ofAnaesthetic with a child fi G. Silvestri A, Moscatelli M, Tumolo Myotonia congenita and anesthetic consider Pathophysiologic PA. Iaizzo O, Bandschapp (Thomsen disease) Kilmartin E, Grunwald Z, Kaplan FS, et al. General anesthesia for dental procedures in patients with fi General anesthesia for dental procedures et al. FS, Kaplan Z, Grunwald Kilmartin E, 30 patients. Anesth Analg. 2014;118:298–301 Analg. Anesth 30 patients. Appendix A: Anesthesia Implications of Syndromes… 581 beroptic beroptic Table continues on the following page. continues on the following Table cartcult airway in OR and use cult. Have diffi Have cult. Preoperatively assess cardiac status (echocardiogram status (echocardiogram assess cardiac Preoperatively Do not use respiratoryAssess function. and ECG). %). in 50 causes myotonia (which succinylcholine Cautious use of cause inhalational agents as may ECG continuously. Monitor depression. myocardial poor produce drugs relaxant may Non-depolarizing medication with interact patient’s and may relaxation Caution with antagonizing muscle phenytoin). (i.e., myotonia; induce neostigmine may relaxants; and myotonia cause shivering halothane may is useful after Suggamadex postoperatively. respiratory to sensitive Extremely rocuronium. regional analgesia when possible. depressants—use pulmonary complications; postoperative Anticipate Caution with support be necessary. ventilatory may opioids function assess cardiac Preoperatively if indicated. SBE prophylaxis (echocardiogram); and intubation opening can be very limited Mouth very diffi advanced airway adjuncts such as LMA and fi adjuncts such airway advanced technique. Upper airway obstruction may necessitate necessitate obstruction airway may Upper technique. ventilatory Postoperative in the neonate. tracheostomy postoperatively Monitor occur; obstruction may Anesthesia implications shlike face, cleft palate (similar to Treacher Treacher (similar to cleft palate face, shlike Weakness and myotonia; eyelid ptosis, cataracts, frontal frontal cataracts, ptosis, eyelid and myotonia; Weakness defects and arrhythmias, conduction cardiac baldness; pulmonary function, Reduced possible cardiomyopathy. Esophageal respiratory to depressants. very sensitive GERD and to motility dysphagia and tendency disorder; Endocrine abnormalities (hypothyroidism, aspiration. in present May in older patients. be present may diabetes) with and hypotonia the neonate weakness Micrognathia, fi of be may Fallot Tetralogy limb deformities. Collins), Cervical spine anomalies associated. Description

Name Myotonia dystrophica 2003;13(2):94–102 Anaesth. Paediatr and paediatric anaesthesia. dystrophy Myotonic Bass SP. RJ, White 2013;23:794–803 Anesth. Pediatr type dystrophies anesthetic care. 1 and 2: Myotonic JL. Scholtes F, Veyckemans Nager syndrome 2002;12(4):365–8 Anaesth. Paediatr syndrome. implications ofAnaesthetic Nager et al. Braddock SR, JO, Johnson K, Groeper (myotonic dystrophy, Steinert dystrophy, (myotonic disease) 582 Appendix A: Anesthesia Implications of Syndromes… Brisk cartcult airway at hand. 167–73 th. 2007;54(10):835–9 th. 0;91(4):858–9 the diffi have cult; vagal responses noted; atropine or glycopyrrolate may may or glycopyrrolate atropine noted; vagal responses central sensitivity depressant to Possible be indicated. and (abnormal response) succinylcholine Avoid drugs. be may ventilation Postoperative inhalation agents. disease suggests central core Link to required. possibility of in nemaline reported MH but not yet be appropriate. analgesia may Regional myopathy. be the best alternative may technique TIVA Caution with intubation (fragile teeth). Prepare for Prepare Caution with intubation (fragile teeth). by with Care drugs excreted instability. vasomotor (abnormal muscle and pad carefully Position kidneys. insertions) and (echocardiogram) assess cardiac Preoperatively and physiotherapy Preoperative pulmonary status. may Intubation antibiotics for infection if indicated. be diffi Anesthesia implications Dysplasia of nails and absent or hypoplastic patellas. Fragile Dysplasia of patellas. nails and absent or hypoplastic abnormality of elbows, “lilac horns” have May teeth. excretion. mucopolysaccharide increased nephropathy, Distal sensory changes instability. Vasomotor disease. central core to be related may myopathy, Congenital cry, weak with as neonates hypotonia, present Commonly micrognathia, Dysmorphic features, and poor feeding. development Motor palate. high arched slender face, of weakness muscle trunk and limbs plus delayed, respiratory and pharyngeal; respiratory leads to failure, heart be Congenital disease may aspiration pneumonia. associated Description

(arthro- osteoonychodysplasia) Name Nail-patella syndrome Anaes Can J a case report and anesthetic implications. syndrome: Nail-Patella et al. SH, Meterissian Backman SB, TA, Hennessey rod myopathy Nemaline 1985;32(5):543–7 Soc J. Anaesth Can implications ofAnaesthetic myopathy. nemaline rod FA. Burrows M, Cunliffe 200 Analg. Anesth in an infant with nemaline myopathy. Spinal anesthesia for gastrostomy et al. I, Erez Sheffer O, Shenkman Z, Klingler W, Rueffert H, Lehmann-Horn F, et al. Core myopathies and risk of malignant hyperthermia. Anesth Analg. 2009;109(4):1 Analg. and risk of Anesth myopathies malignant hyperthermia. Core et al. F, Lehmann-Horn H, Rueffert W, Klingler Appendix A: Anesthesia Implications of Syndromes… 583 urane. Propofol is Propofol urane. Table continues on the following page. continues on the following Table ively ventilatory support. ventilatory support. ively hild Neurol. 2012;27:1541–6 hild Neurol. :806–808 culty (pulmonary with ventilation Preoperatively check cardiac (echocardiogram), (echocardiogram), cardiac check Preoperatively studies. and coagulation pulmonary X-ray), (chest diffi Anticipate and possible disease and ascites) restrictive for postoperat requirement Caution with drugs in the liver. metabolized of withPossibility seizures sevofl Start infusion (5–10 mg/kg/min on a pump; IV glucose blood glucose and monitor no bolus) preoperatively (Boluses would stable postoperatively. until condition be may The child hyperglycemia.) rebound precipitate Normal (N.B. and glucagon. diazoxide, steroids, receiving have be found to occasionally may neonates full-term <40 mg/dL) “hypoglycemia” asymptomatic temperature and maintain body Monitor usually OK. Anesthesia implications ltration of lungs ciency, pneumonia. May have have May pneumonia. ciency, coronary or valvular heart disease. Developmental delay. delay. Developmental heart or valvular coronary disease. (type year A) to the 3rd Death usually by ataxia. Epilepsy, Pulmonary disease intellect. (type Normal 15th year C). in alveoli) (foam cells leads to pulmonaryleads to insuffi Symptomatic hypoglycemia in infants: (1) small for in infants: hypoglycemia Symptomatic and (3) premature (2) diabetic mothers, gestational age, and lethargy, convulsions, If untreated: infants. insulinoma or Rarely, delay; no ketosis. developmental pancreatectomy. requiring subtotal hypertrophy pancreatic See also Beckwith syndrome See Gorlin-Goltz syndrome of and accumulation sphingomyelin Hepatosplenomegaly liver, Bone marrow, the body. and other lipids throughout anemia and leads to and spleen involvement infi Diffuse foam cell thrombocytopenia. Description (see ning neonatal hypoglycaemia: a continuing debate. Semin Fetal Neonatal Med. 2014;19:27–32 Med. Neonatal Semin Fetal debate. a continuing ning neonatal hypoglycaemia: Name Neonatal hypoglycemia, symptomatic 2014;21:45–50 Opin Endocrinol Obes. Diabetes Curr on neonatal hypoglycemia. Update PJ. Rozance Tin W. Defi carcinoma basal cell Nevoid syndrome Niemann-Pick disease disease) types Wolman also and type B (onset in D, C, A, infancy) 2002;12(9) Anaesth. Paediatr in anaesthesiological practice. problem a rare disease: Niemann-Pick P. Knapik G, Bujok LS, Bujok Miao N, Lu X, O’Grady NP. Niemann-pick disease type C: implications for sedation and anesthesia for diagnostic procedures. J C disease type Niemann-pick implications for sedation and anesthesia diagnostic C: procedures. NP. O’Grady X, Lu N, Miao 584 Appendix A: Anesthesia Implications of Syndromes… cult airway airway cult have cult intubation; 7 82 the diffi have cult intubation; culties due to nasal, oral, and mandibular oral, nasal, culties due to cart cult airway available Preoperative assess cardiac (echocardiogram and (echocardiogram assess cardiac Preoperative status. and coagulation creatinine) (BUN renal ECG), diffi Possible as for considerations Hemangioma carefully. Position syndrome Maffucci Airway Airway diffi Diffi Brittle teeth. defects. diffi cart at hand. Care with drugs excreted by kidneys by with Care drugscart excreted at hand. Anesthesia implications pertrophic cardiomyopathy. pertrophic cardiomyopathy. a review of anaesthetic problems. Paediatr Anaesth. 1994;4:179– Anaesth. Paediatr a review of anaesthetic problems. sm. 25 % risk for skeletal, visceral or brain % risk for skeletal, 25 sm. See Pfeiffer syndrome See Pfeiffer syndrome mild developmental hypertelorism, neck, web Short stature, anomalies: Cardiac syndrome. Turner to Similar delay. hy usually pulmonary stenosis, dysfunction platelet hydronephrosis, Micrognathia, See Goldenhar syndrome syndrome See Lowe syndrome See Moebius with (enchondromatosis syndrome See also Maffucci within chondromas Multiple hemangioma). cavernous Asymmetric pathologic fractures. usually unilateral; bones, dwarfi malignancy Microphthalmia and microcornea, small nose with and microcornea, Microphthalmia dental enamel dysplasia, cleft palate, nostrils, anteverted plus a generalized mandibular defect of modeling; bony abnormal long bones thick ribs, dysplasia, Description ndings in Noonan syndrome on long-term follow-up. Congenit Heart Dis. 2014;9(2):144–50 Dis. Heart Congenit follow-up. on long-term syndrome ndings in Noonan

(variant (ODOD) Name Noack syndrome of Pfeiffer syndrome) 2011;14:214– Anaesth. Cardiac Ann perspective. an anesthesiologist’s syndrome: Noonan U. Kiran PM, Kapoor V, Malik V, Aggarwal Oculoauriculovertebral syndrome dysplasia: Oculodento-osseous J. Dunphy Colbert S, F, Colreavy Oculofacial paralysis, congenital Noonan Noonan syndrome Oculocerebrorenal syndrome Oculodento-osseous dysplasia Ollier syndrome Colquitt JL, Noonan JA. Cardiac fi Cardiac JA. Noonan JL, Colquitt Appendix A: Anesthesia Implications of Syndromes… 585 cult to control. control. cult to cartcult airway Table continues on the following page. continues on the following Table aemia. Acta Anaesth Scand. Anaesth Scand. Acta aemia. neral anesthesia: a cohort from a a cohortneral from anesthesia: aesth. 2007;17(6):603–4 aesth. ):1189–93 cartcult airway immediately cult airway, small larynx small tracheal (prepare cult airway, cult to maintain IV due to fragile vessels. Check fragile maintain IV due to cult to vessels. diffi have cult intubation; available. Preoperatively renal function (BUN/ renal Preoperatively available. the by caution with drugscreatinine); excreted kidneys Diffi diffi have tubes); Danger of empty regurgitation; stomach available. induction before and problems Airway respiratoryAssess status. diffi Anemia; internal hemorrhage may occur hemorrhage occur internal Anemia; may Blood loss diffi perioperatively. Diffi may ventilation pressure Positive pulmonary status. in patients with pulmonaryAV oxygenation decrease malformation Anesthesia implications d Weber-Rendu syndrome with coexisting congenital methaemoglobin congenital with syndrome coexisting Weber-Rendu nt of patients with hereditary hemorrhagic telangiectasia undergoing ge potentially hazardous anaesthetic implications. Anaesthesia. 1985;40(12 Anaesthesia. anaesthetic implications. hazardous potentially hemoptysis possible massive stula; X-linked or autosomal dominant, affects males more than affects males more dominant, or autosomal X-linked and genital abnormalities Craniofacial (bifi females. achalasia, aspiration, Dysphagia and recurrent scrotum). and a high arched micrognathia, Hypertelorism, hiatal hernia. Laryngeal malformations (including laryngotrachealpalate. cleft and subglottic and pulmonary stenosis) hypoplasia mandible hypoplastic lobed tongue, Cleft lip and palate, polycystic digital hydrocephalus, anomalies; and maxilla, tracheomalacia and laryngomalacia. Possible kidneys. recovery in delayed result Corpus callosum anomaly (may anesthesia) from most commonly capillary dilation, and venous Multiple be organ may but any affects the skin and nasal mucosa, of High incidence pulmonaryAV affected. and hepatic fi Description

(hemorrhagic (Mohr syndrome) syndrome) (Mohr Name Opitz-Frias syndrome A case with syndrome. Opitz-Frias Gillbe C. Bolsin SN, Oral-facial-digital syndrome An management of Anesthetic a newborn Paediatr type (oro-facial-digital with MM. Ozek Mohr II) syndrome. A, Dagcinar A, Gercek Osler-Rendu-Weber syndrome telangiectasia) management ofAnaesthetic Osler- PK. Bithal MP, Pandia Sharma D, (G syndrome, hypospadias, hypospadias, (G syndrome, dysphagia syndrome) 2005;49(9):1391–4 Manageme et al. KO, Anusionwu JW, Hanson TN, Weingarten single academic center’s experience. J Anesth. 2013;27:705–11 Anesth. J experience. single academic center’s 586 Appendix A: Anesthesia Implications of Syndromes… cult intubation; intubation; cult ogenesis imperfecta. J Anesth. Anesth. J ogenesis imperfecta. 4;14(6):524–5 level. Unpredictable response to to response Unpredictable level. + cult airway cart at hand. Avoid neuraxial Avoid cartcult airway at hand. culty fragile in maintaining IV due to vessels. have diffi have regional analgesia if spine is abnormal. Assess cardiac status (echocardiogram and ECG); SBE and ECG); status (echocardiogram cardiac Assess diffi Possible if indicated. prophylaxis non-depolarizing muscle relaxants; avoid avoid non-depolarizing relaxants; muscle dystrophy) (See also myotonic succinylcholine. Check serum K Anesthesia implications Use extreme care in positioning (to prevent breaking breaking prevent in positioning (to care extreme Use easily broken. are Teeth bones) and intubating. Diffi hyperthermiaIntraoperative (not MH) has been inhaled anesthetics; described in patients receiving decrease or may unchanged remains temperature during TIVA lung pathology → kyphoscoliosis → fragile in carious, teeth ciency results ient with trisomy 13. Pediatr Anesth. 2006;16:213–5 Anesth. Pediatr ient with 13. trisomy cta: different anaesthetic approaches to two paediatric cases. Paediatr Anaesth. 200 Anaesth. Paediatr paediatric two cases. to anaesthetic approaches different cta: Osteoporosis Osteoporosis Fragility of in subcutaneous hemorrhage. results vessels Dentine defi may be high or low be high or low may + 1. 1. stillbirth Congenita—usually or rapidly fatal 2. deafness blue sclera, fractures, Tarda—pathologic See Albers-Schönberg disease serum weakness; paroxysmal cold; to on exposure Myotonia K cleft lip micrognathia, microcephaly, delay, Developmental anomalies (usually ventricular cardiac have May or palate. die in infancy. Patients septal defect and/or dextrocardia). spinal malformations Possible Description

(trisomy 13 (trisomy Ogawa S, Okutani R, Suehiro K. Anesthetic management using total intravenous anesthesia with remifentanil in a child with anesthesia with in a child oste remifentanil intravenous management using total Anesthetic K. Suehiro Okutani R, S, Ogawa 2009;23(1):123–5 (Eulenburg periodic (Eulenburg paralysis) Name Osteogenesis imperfecta (fragilitas ossium) imperfe Osteogenesis Capan Z. L, Karabiyik Osteopetrosis Paramyotonia congenita 2004;98(1):68–9 Analg. Anesth congenita. in a patient with paramyotonia Pyloromyotomy et al. VI, Dogan A, Gercek B, Ay Patau syndrome 1996;6:151–3 Anaesth. Paediatr syndrome). for patients withAnaesthesia 13 (Patau’s trisomy Beasley JM. RC, Pollard in a pat Caudal block complication IT. Cohen syndrome) syndrome) Appendix A: Anesthesia Implications of Syndromes… 587 cult Table continues on the following page. continues on the following Table beroptic scope, and diffi scope, beroptic

carotid arteries. Paediatr Anaesth. Anaesth. Paediatr arteries. carotid cult airway. Have advanced airway advanced Have cult airway. airway cart) available. If OSA, reduce opioids and reduce If OSA, cart)airway available. saturation and oxygen respirations closely monitor for proptosis care Eye postoperatively. Preoperatively ensure that patient is euthyroid, that patient is euthyroid, ensure Preoperatively otherwise as for cretinism Anticipate diffi fi devices (GlideScope, status (echocardiogram). assess cardiac Preoperatively Review vascular CVA. Risk of ischemia, cerebral and function. oxygenation cerebral Monitor anatomy. need therapy may Hypertension Anesthesia implications berscope” in a patient with Pfeiffer syndrome. J Bronchology Interv Pulmonol. 2011;18(4):374–5 Pulmonol. Interv J Bronchology in a patient with Pfeiffer syndrome. berscope” ls fi ls nding in a case of Pfeiffer syndrome and its management. J Anaesthesiol Clin Pharmacol. 2011;27(3):414–5 Clin Pharmacol. Anaesthesiol J nding in a case of and its management. Pfeiffer syndrome See leukodystrophy inheritance of sensorineural recessive deafness Autosomal May production. block of incomplete and goiter; thyroxine risk for thyroid Increased or hypothyroid. be euthyroid implants for cochlear Candidates cancer. congenita See familial periodic paralysis and paramyotonia proptosis, craniosynostosis), Cloverleaf skull (bicoronal may ankylosis of elbows, small mandible, hypertelorism, sleep obstructive associated have May hydrocephalus. have High mortality rate intelligence. Normal apnea. arterial hemangiomas, fossa brain malformations, Posterior of defects (PHACE). eye coarctation the aorta, anomalies, with be associated agenesis of bilateral May the carotid Airway perfusion via arteries. the vertebral cerebral arteries, artery may Renal stenosis be present. hemangioma may cause hypertension Description Gupta A, Ahmed M, Prabhakar C, et al. Unique airway fi airway Unique et al. C, Prabhakar Ahmed M, A, Gupta Name Pelizaeus-Merzbacher disease Pendred syndrome 1960;29:279 Queensland J Med. of The syndrome deafness. sporadic and congenital goitre WR. Trotter ME, Morgans GR, Fraser 2011;70:43–9 Otorhinolaryngol. Adv hearing Hereditary abnormalities. loss with thyroid et al. KA, King J, Muskett Choi BY, 2010;20(7):707–13 in the era Thyroid. of cancer with associated medicine. thyroid personalized syndromes Familial ML. Richards Periodic paralysis Pfeiffer syndrome “bonfi using a Intubation et al. R, Pallotto R, Giretti Caruselli M, PHACE syndrome and agenesis of syndrome internal bilateral with management in a child Anesthesia PHACE et al. Raisky O, O, Metton A, Javault 2007;17(10):989–93 2014;28:919–23 Anesth. J syndrome. for aortic withAnesthesia in a child PHACE reconstruction Y. Oda Okutani R, T, Imada 588 Appendix A: Anesthesia Implications of Syndromes…

ciency. Arch Surg. 1993;128(12):1391–5 Surg. Arch ciency. Induction and maintenance by inhalation technique. inhalation technique. by and maintenance Induction infusion and monitor dextrose Give ventilation. Control to (tendency perioperatively levels glucose and phenylalanine adequate Ensure hypoglycemia). hemoglobin (no megaloblastic anemia) concentration as it is contraindicated oxide Nitrous preoperatively. in vitamin the cobalt B12 and has oxidizes irreversibly to Sensitive degeneration. spinal cord subacute lead to body monitor opioids and other CNS depressants; anti- continue If patient has epilepsy, temperature. medicationsseizure Anesthesia implications cient diet must ciency. At risk for B12 At ciency. ciency. Vomiting, CNS irritability, developmental delay, delay, developmental CNS irritability, Vomiting, ciency. Phenylalanine hydroxylase defi hydroxylase Phenylalanine defi Phenylalanine-defi convulsions. hypertonia, be maintained; may present with present megaloblastic anemia if may be maintained; poorly controlled Description Name Phenylketonuria 2004;14(8):701–2 Anaesth. Paediatr management ofAnesthetic a strabismus patient with phenylketonuria. V. Celiker Dal D, 2007;17(9):824–30 Anaesth. Paediatr and pediatric anesthesia. oxide is no laughing nitrous matter: oxide nitrous When VC. Baum Flippo TS, Holder WD, Jr. Neurologic degeneration associated with nitrous oxide anesthesia in patients with vitamin with degeneration oxide associated B12 defi nitrous Neurologic Jr. WD, Holder Flippo TS, Appendix A: Anesthesia Implications of Syndromes… 589 beroptic beroptic cult airway cult cult. Diffi cult. Table continues on the following page. continues on the following Table ediatr Anaesth. 2008;18(1):77–80 ediatr Anaesth. ities: A valuable option with pectus ities: straction osteogenesis. Paediatr Paediatr straction osteogenesis. Preoperatively assess cardiac (echocardiogram) and (echocardiogram) assess cardiac Preoperatively Controlled function (BUN/creatinine). renal the chest due to is recommended ventilation deformity Anticipate airway obstruction for airway and potential Anticipate aspiration perioperatively Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively with growth. improve and airway Micrognathia diffi be extremely may Intubation or GlideScope intubation should be anticipated; topical topical intubation should be anticipated; or GlideScope analgesia withairway lidocaine will nebulized facilitate insertion anesthesia induce of used to awake an LMA, or infant. for intubation in the neonate and as a conduit extubation before should be fully awake The child adjuncts must be immediately available, and a fi available, be immediately adjuncts must Anesthesia implications rst Absent or hypoplastic pectoral muscles with muscles pectoral or hypoplastic chest Absent have May syndactyly Ipsilateral or microdactyly. deformity. form: Extreme anomalies. and gastrointestinal renal CHD, herniation on Lung has facial paralysis. syndrome Moebius ofcrying: wall on inspiration movement chest paradoxical Cleft palate, micrognathia, glossoptosis due to fi glossoptosis due to micrognathia, Cleft palate, and sixth retardation, psychomotor paralysis, cord Vocal and cyanotic respiratory distress, Stridor at rest, nerve palsy. spells or choking branchial arch embryologic defect. CHD may be present. be present. CHD may embryologicbranchial arch defect. and can occur obstruction upper airway may Neonates: prone nursing by airway Maintain pulmonale. cor lead to or intubation, suture, tongue require may on a frame: tracheostomy Description Poland Poland syndrome Name syndrome Pierre Robin tracheal the laryngeal intubation through Pa with mask in neonates obstruction. upper airway Awake Shingu K. A, Nagata T, Asai Plott syndrome 1998;45(3):277–9 Anaesth. Can J syndrome. in Poland Anaesthesia et al. Bala I, S, Kannan Sethuraman R, wall deform chest of and congenital implant for the correction silicone acquired Customized S. Worrollo DH, Parikh G, Soccorso Frawley G, Espenell A, Howe P, et al. Anesthetic implications ofAnesthetic with infants with treated mandibular di mandibular hypoplasia et al. P, Howe A, Espenell G, Frawley 2013;23(4):342–8 Anaesth. 2014;119(2):400–12 Analg. Anesth review. a perioperative Sequence: Robin Pierre et al. L, Grunwaldt A, Kumar Cladis F, 2015;50(7):1232–5 Surg. J Pediatr excavatum. 590 Appendix A: Anesthesia Implications of Syndromes… cult airway cart at hand. TIVA TIVA cartcult airway at hand. ation pressures. Prevent Prevent ation pressures. proach. Paediatr Anaesth. Anaesth. Paediatr proach. disease. Pediatr Anesth. 2007;17:697–702 Anesth. Pediatr disease. Preoperatively assess cardiac function assess cardiac Preoperatively if SBE prophylaxis (echocardiogram and ECG); with cardiac required care Extreme indicated. and maintain preload. tachycardia avoid depressants; ECG for rhythm and ST segment changes; Monitor relaxants muscle Use serious occur. arrhythmias may are and ketamine with etomidate caution; cause airway may Large tongue agents. recommended the diffi have problem; be useful in analgesia may Regional is recommended. some cases Preoperatively, assess renal function (BUN/ assess renal Preoperatively, pneumothorax; lead to cysts may Lung creatinine). high-peak infl avoid aneurysm) cerebral hypertension (possible associated Anesthesia implications -glucosidase enzyme (rhGAA) has proven effective in effective -glucosidase enzyme (rhGAA) has proven See Guillain-Barré syndrome syndrome See Guillain-Barré hypotonicity; large severe Deposits of in muscles, glycogen and conduction hypertrophic cardiomyopathy tongue; 2 years before cardiorespiratory failure Death from defects. human therapy with recombinant of Replacement age. α the life span extending See collagen disease See collagen bladder, lungs, spleen, pancreas, cysts in the liver, Associated % aneurysm cerebral in 15 (one-third); thyroid Description (glycogen (glycogen Pompe Pompe disease Polyneuritis, Polyneuritis, acute disease typestorage II) Name Polyarteritis nodosa Polycystic kidneys management ofAnaesthetic disease type infants with storage ap glycogen a physiological et al. II: Bengur RA, DR, Cook RJ, Ing 2004;14(6):514–9 general anesthesia for infants with to Pompe’s an alternative are anesthetic techniques Regional J. Bruce Briggs G, RW, Walker Appendix A: Anesthesia Implications of Syndromes… 591 urane, urane, O, O, sevofl 2 Table continues on the following page. continues on the following Table Avoid prolonged fasting and dehydration. Avoid Avoid fasting and dehydration. prolonged Avoid barbiturates (including thiopental) and certain other hydantoin, ketamine, etomidate, IV agents (i.e., or antipyretics, sulfonamides, derivatives, for drug et al. See Jensen agents). hypoglycemic been used The following have in porphyria. concerns (brief propofol glycopyrrolate, safely: atropine, N succinylcholine, exposure), vecuronium, atracurium, cisatracurium, fentanyl, fentanyl, cisatracurium, atracurium, vecuronium, neostigmine, epinephrine, barbiturates, morphine, chlorpromazine, diphenhydramine, chloral hydrate, and bupivacaine Anesthesia implications form Paediatr ciency: of porphyria. a severe intermittent acute with porphobilinogen homozygous deaminase defi Paralysis, psychiatric disorder, autonomic imbalance— autonomic disorder, psychiatric Paralysis, by abdominal pain precipitated tachycardia; hypertension, of incidence High diabetes etc. infection, stress, drugs, Description Name Porphyrias 1995;80:591–9 Analg. Anesth in porphyrias. considerations Anesthetic V. Striepe Fiddler DS, NF, Jensen in a child Anesthesia Dorman T. L, Sheppard Anaesth. 2005;15(5):426–8 Anaesth. 592 Appendix A: Anesthesia Implications of Syndromes… The small cult. the have cult; Paediatr Anaesth. 2006;16(7):790–3 Anaesth. Paediatr 4;24(4):457–9 may Low-grade pyrexia cartcult airway at hand. Danger of and blood glucose so monitor hypoglycemia period. solution in perioperative infuse IV glucose diffi cannulation Obesity venous makes mandible may make intubation diffi make mandible may diffi or hernia surgery. strabismus, duringoccur scoliosis, Sleep also occur. risk may Hypothermia ofNo MH. may ventilation or controlled assisted apnea common: or apneabe necessary during operation, and after opioid only low-dose Use monitoring postoperatively. opioids with regional blocks and postoperative or avoid obstruction; of airway Beware postoperative NSAIDS. analgesia Regional airway. improve may nasal CPAP in some patients for intraoperative be appropriate may pain management and/or postoperative Anesthesia implications hypotonia, poor feeding, poor feeding, hypotonia, exes absent. Second phase: hyperactive, uncontrollable uncontrollable hyperactive, phase: Second absent. exes Sporadic mutation. Cytogenetic deletion at chromosome deletion at chromosome Cytogenetic Sporadic mutation. Angelman father (same genetic defect in inherited from type Hypothalamic mother). is inherited from syndrome Neonate: “Pickwickian syndrome.” refl developmental polyphagia, disturbed, thermoregulation obesity cardiorespiratory Extreme failure. leading to delay. a small mouth or mandible have May Description Name Prader-Labhart-Willi syndrome 1998;8(3):267–71 Anaesth. Paediatr syndrome. and Prader-Willi Anaesthesia M. Super A, Dobson OR, Dearlove 201 Anaesth. at higher Paediatr risk syndrome for anesthetic complications? Willi with Prader children Are Landsman IS. Lam H, Mantadakis E, Spanaki AM, Geromarkaki E, et al. Near demise of a child with Prader-Willi syndrome during elective orchidopexy. during elective syndrome demise of with Near a child Prader-Willi et al. E, Geromarkaki AM, Spanaki E, Mantadakis Appendix A: Anesthesia Implications of Syndromes… 593 cult airway airway cult ation pressures; pressures; ation cartcult airway cult because of small Table continues on the following page. continues on the following Table diffi have cult; rative concerns. J Ped Neurosci. Neurosci. J Ped concerns. rative –26 rome. J Anesth. 2006;20:26–9 Anesth. J rome. O and high-peak infl 2 mouth and receding mandible; have diffi have mandible; mouth and receding as for adults withAnesthesia coronary cart available. avoid ischemia; artery disease and myocardial glucose- Administer and hypertension. tachycardia values glucose solutions and check containing perioperatively Assess cardiac status preoperatively particularly status preoperatively cardiac Assess evidence of artery coronary disease and myocardial be diffi may Intubation ischemia. If for cystic lesion. X-ray chest Check preoperative N avoid present, available. Postoperative airway obstruction may occur, occur, obstruction airway may Postoperative available. ventilation monitor possible pneumothorax. Caution with airway, Caution with airway, possible pneumothorax. be diffi intubation may Anesthesia implications espiratory complications caused by cystic lung malformation in Proteus synd espiratory cystic lung malformation in Proteus caused by complications Premature aging starts at 6 months to 3 years; cardiac cardiac aging starts 3 years; at 6 months to Premature may Diabetes cardiomegaly. hypertension, disease-ischemia, artery coronary occur Death from disease may be present. of Thin skin and fragile 10 year blood vessels before age. ofA highly overgrowth variable disease with progressive and abnormal skin lesions (nevi), bone, tissues, connective is common. Scoliosis lung lesions. distribution of Cystic fat. and twisted because of be elongated The neck may vertebral and be present, malformations may Vascular deformities. been pulmonary have thrombosis emboli without venous reported Description (Hutchinson- Name Progeria 2007;101(1):13 Res. Circ aging syndromes. of disease in accelerated cardiovascular Mechanisms EG. Nabel FS, Collins Capell BC, Proteus syndrome 2004;14(8):689–92 Anaesth. Paediatr syndrome. for proteus Anesthesia et al. B, Tuncer AZ, Kordan Cekmen N, r Perioperative et al. H, Hattori M, Sato M, Nakane Gilford syndrome) syndrome) Gilford 2001;11(5):611–4 Anaesth. Paediatr progeria. with in a child Anaesthesia Hutchinson-Gilford Liessmann CD. Periope syndrome: progeria Extradural surgery hematoma et al. with in a child Hutchinson-Gilford C, Patra Agarwal J, U, Hansda 2013;8:165–7 594 Appendix A: Anesthesia Implications of Syndromes… have cult in some; have cult intubation; 0 1;11:501–4 cult airway cart available. Use muscle relaxants relaxants muscle Use cartcult airway available. cartcult airway at hand Preoperatively assess renal function (BUN/creatinine). function (BUN/creatinine). assess renal Preoperatively and control intubate as for a full stomach: Treat be diffi may Intubation ventilation. diffi Thoracic kidneys with by caution. and drugs excreted analgesia and may epidural useful for postoperative respiratory compromise prevent possible diffi airway, Assess diffi Anesthesia implications Agenesis of abdominal musculature with renal anomalies. of with anomalies. Agenesis renal abdominal musculature respiratory risk cough; of atelectasis, postoperative Poor and respiratory failure infections, See Albright osteodystrophy See Grönblad-Strandberg syndrome cranial enlarged mandible; abnormalities; Craniofacial nerve paralyses Description (metaphyseal (metaphyseal dysplasia) dysplasia) Name belly syndrome Prune 200 Anaesth. Paediatr with management in a child airway prune-belly syndrome. Complicated et al. E, Ustun D, Karakaya Baris S, Pseudohypoparathyroidism Pseudoxanthoma elasticum Pyle disease Henderson AM, Vallis CJ, Sumner E. Anaesthesia in the prune-belly syndrome. A review of 36 cases. Anaesthesia. 1987;42(1):54–6 Anaesthesia. A review of 36 cases. in the prune-bellyAnaesthesia syndrome. E. Sumner CJ, Vallis AM, Henderson Appendix A: Anesthesia Implications of Syndromes… 595 Table continues on the following page. continues on the following Table oliosis surgery: a retrospective oliosis surgery: a retrospective s and clinical evidence. Spine. Spine. s and clinical evidence. Anesthetize for investigation of of and decompression for investigation Anesthetize and steroids be receiving may Patient ICP. increased drugs by metabolized Avoid hypothermia. controlled and continue ventilation Control the liver. measures hypothermia and all supportive Administer anti-seizure medications on the morning anti-seizure Administer pulmonaryAssess ECG. Preoperative of surgery. risk of Severe respiratoryfunction. complications. SSEPs for spinal surgery present Often for scoliosis. if be contraindicated may but MEPs can be monitored apnea monitoring history Postoperatively, of seizures. or Insensitive supportor ventilatory needed. respiratory to be sensitive May pain. to hypersensitive seizures. control to Benzodiazepines drugs. depressant prevent syndrome; Cautions as for long QT tachycardia Anesthesia implications n alternating withn alternating apneic spells ement of scoliosis in Rett syndrome patients based on expert consensu syndrome ement of in Rett scoliosis Disabling neurologic disorder affecting only females. affecting only females. disorder Disabling neurologic seizures, autism, delay, developmental Underweight, instability, vasomotor abnormal pain sensation, scoliosis, irregular marked syndrome), arrhythmiascardiac (long QT hyperventilatio respiration: and fatty degeneration of metabolic encephalopathy Severe increased hyperaminoacidemia; viscera (especially liver): serum transaminases. blood ammonia, time, prothrombin is ingestion ofASA (aspirin) during cofactor Suspected biopsy. diagnosis liver reliable is by Most illness. prodromal been have There ICP is usually fatal. increased If untreated, very few cases in the last 15 years Description 2009;34(17):E607–17 of undergoing sc management and outcome syndrome patients with Rett Perioperative et al. Galtrey E, R, Krishnan I, Karmaniolou 2015;29(4):492–8 Anesth. J review. Name Rett syndrome 1996;6(2):155–8 Anaesth. Paediatr syndrome. for Rett Anaesthesia RW. Walker OR, Dearlove for manag Guidelines et al. Carter P, A, Bergman Downs J, Reye syndrome 2007;9(3):195–204 Drugs. a review of Paediatr syndrome: the evidence. Aspirin and Reye K. Schror, 2008;26(7):741–6 Funct. Cell Biochem syndromes. and Reye’s-like Reye’s D. Torre Beltramo T, A, Pugliese 596 Appendix A: Anesthesia Implications of Syndromes… cartcult airway : use opioids with caution, use opioids with: caution, and at induction. Diazepam and at induction. 2 maintain volume uid losses to -receptor antagonist. Parental Parental antagonist. -receptor 2 diffi cult airway; have Possible Possible diffi with Premedication fasting. prolonged Avoid and H midazolam available available may require postoperative ventilation. Risk of ventilation. postoperative require may postoperatively aspiration, ranitidine for crisis, an autonomic controls often manage be useful to and clonidine may gastric acidity, Epidural and spinal hypertension. postoperative but has anesthesia was thought be contraindicated to in a few cases with been used uneventfully increased stability and superiorcardiovascular analgesia lubricate and cover Caution with eyes, reported. presence may help. Atropine can be given. Require IV Require can be given. Atropine help. may presence fl replace hydration; blood loss is CVP ifstatus (monitor extensive titrate anesthetic agents: to Sensitive anticipated). can use barbiturates, effect; inhalational agents to Respiratory and relaxants. opioids, etomidate, propofol, CO to unresponsive center Anesthesia implications ciency of dopamine- -hydroxylase: autonomic dysfunction and decreased and decreased dysfunction autonomic -hydroxylase: cult airway management in a baby with Axenfeld-Rieger syndrome. Paediatr Anaesth. 1998;8(5):444 Anaesth. Paediatr syndrome. Axenfeld-Rieger with management in a baby cult airway See collagen diseases See collagen and malformations ofHypodontia of anterior chamber eye. abnormalities other developmental including have May maxillary hypoplasia Recessive disorder of disorder ganglia autonomic and sensory Recessive Defi Jews. Ashkenazi found in neurons β and orthostatic hypertension, paroxysmal sensation, absent lacrimation, Emotional lability, hypotension. poor sucking and swallowing. abnormal sweating, lung disease. aspiration pneumonia and chronic Recurrent heart rate sweating, profuse crisis (vomiting, Dysautonomic to instability) in response and hemodynamic can occur stress Description

Name Rheumatoid arthritis Rieger syndrome Diffi Shingu K. H, Matsumoto T, Asai Riley-Day syndrome 2006;16(6):611–20 Anaesth. Paediatr management ofAnesthesia familial dysautonomia. et al. JT, Kim I, Kreynin Ngai J, 2008;18:1136–7 Anesth. Pediatr (familial dysautonomia). syndrome Spinal anesthesia in Riley-Day Ahmed M. MM, Watve Ahmed N, (familial dysautonomia) (familial dysautonomia) Appendix A: Anesthesia Implications of Syndromes… 597 cult cult cartcult airway at diffi have cult; Table continues on the following page. continues on the following Table t J Anaesth. 2011;21:309–12 Anaesth. t J Acta Anesth Belg. 2014;65:179–82 Belg. Anesth Acta

Caution cartcult airway at hand. diffi cult airway; have airway cartairway at hand intubation; have diffi have intubation; be a may Secretions with respiratory depressants. of ventilatory Beware postoperative problem. or apnea depression Preoperatively assess cardiac (echocardiogram), renal renal (echocardiogram), assess cardiac Preoperatively and hepatic (if Crigler-Najjar (BUN/creatinine), evaluate is present, If Crigler-Najjar status. suspected) Caution with airway status. coagulation but usually not diffi recommended and (echocardiogram) assess cardiac Preoperatively diffi Anticipate pulmonary status. X-ray) (chest Anticipate Anticipate diffi Anesthesia implications hand. No other special considerations other special considerations No hand. sm See Pierre Robin syndrome syndrome Robin See Pierre and spinal hypoplasia), facial (midface Limb-shortening, genitalia. and hypoplastic disease, renal CHD, deformities, (Both disease. liver with be associated Crigler-Najjar May ofdiseases a result consanguinity) syndrome and Lange-Nielsen See Jervell delay, developmental toes, and great thumb Broad CHD (usually pulmonary have May microcephaly. aspiration repeated infections, chest frequent stenosis), Estimated lung disease. pneumonia and chronic leading to frequency: 1 of every developmental 500 institutionalized persons delayed dwarfi See Silver-Russell See gangliosidosis GM2 of closure cranial premature III, Acrocephalosyndactyly withsutures syndactyly Hypoplastic of the hands and feet. be present. malformed ears may hypertelorism, maxilla, in TWIST1 gene Mutation usually normal. Intelligence Description (fetal Name Pierre syndrome Robin Robinow syndrome syndrome) face 2003;13(8):725–7 Anaesth. Paediatr syndrome. implications ofAnaesthetic Robinow et al. A, Schuerholz Rieder J, Lirk P, Romano-Ward syndrome Rubinstein-Taybi syndrome 2004;14(7):610–11 Anaesth. Paediatr syndrome. management ofAnesthetic with a child Rubinstein-Taybi S. Cakmakkaya Altintas F, Russell-Silver syndrome 2008;18(1):81 Anaesth. Paediatr syndrome. with in a child Anesthesia Saethre-Chotzen JF. Mayhew Easely D, review. anesthetic management and literature syndrome: Child with Saethre-Chotzen Ramachandran R. S, Arora N, Patel A, Sharma Agarwal S, Ahmad YH, Talpesh M, et al. Anesthetic management of children with Rubinstein-Taybi syndrome--case reports. Mid Eas Mid reports. syndrome--case management ofAnesthetic with Rubinstein-Taybi children et al. M, Talpesh YH, Ahmad Agarwal S, Sandhoff disease Saethre-Chotzen Saethre-Chotzen syndrome 598 Appendix A: Anesthesia Implications of Syndromes… c cult airway airway cult cartcult airway diffi have cult; 2006;16(11):1189–92 diffi have cult intubation; available. Fiberoptic intubation has been Fiberoptic available. contraindicated; Succinylcholine recommended. nondepolarizing to possible abnormal response (See also myotonia inhalation agents. avoid relaxants, and paramyotonia) See mucopolysaccharidoses. No other specifi No See mucopolysaccharidoses. Diffi anesthesia problems described anesthesia problems Administer anti-seizure medications on the morning anti-seizure Administer assess respiratory status Preoperatively of surgery. Airway calcium. electrolytes and ionized X-ray), (chest be diffi and intubation may Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively for apnea. Monitor See mucopolysaccharidoses. with care Position dose. reduce Caution with opioids, Anesthesia implications cart at hand. Use short-acting drugs. Monitor Monitor short-acting drugs. Use cart at hand. respiratory status postoperative lippo Syndrome Type A. Pediatr Anesth. 2016;26(5):531–8 Anesth. Pediatr A. Type lippo Syndrome sm, developmental developmental sm, lippo syndrome: overall review. Pediatr Int. 2015;57(3):331–8 Int. Pediatr review. overall lippo syndrome: of Arab descent children ned to sm, microstomia, micrognathia, cleft palate, cleft palate, micrognathia, microstomia, sm, ciency. Sleep apnea may occur occur Sleep apnea may ciency. CNS malfunction in childhood progresses to developmental developmental to progresses CNS malfunction in childhood Emotional disturbance and agitation. and dementia. delay or major bone problems, cardiac hepatosplenomegaly, No problems hypocalcemia, hypoparathyroidism, Congenital dwarfi seizures, hyperphosphatemia, pulmonary Recurrent features. and dysmorphic delay, Confi infections. clouding, Corneal or almost normal intellect. Normal especially of aortic joint stiffness, hands and feet; hernias; insuffi Dwarfi bowing of long bones, limiting joint movement, myotonia (some normal intellect Usually thermoregulatory disorder. %) degree of in 25 delay developmental Description

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lippo syndrome lippo Name Sanfi Sanfi et al. M, Llarena Aldamiz-Echevarria L, Andrade F, Sanjad-Sakati syndrome Anaesth. Paediatr with management for the child Anesthesia Sanjad-Sakati syndrome. et al. L, Kachko D, Wasersprung Platis CM, Scheie syndrome 1980;35:85–91 Thorax. syndrome. Sleep apnea in Scheie’s Bradbury et al. RA, S, Cooper WH, Perks Schwartz-Jampel syndrome 1994;49(7):600–2 Anaesthesia. syndrome. with in a child Anaesthesia Schwartz-Jampel AP. Rubin S, Ray (mucopolysaccharidosis (mucopolysaccharidosis type III) with Sanfi in children and outcomes care Anesthetic et al. CB, Whitely Beebe DS, Cingi EC, (SSS) type(mucopolysaccharidosis formerly classifi IS, V) type Appendix A: Anesthesia Implications of Syndromes… 599 cult cartcult airway at Table continues on the following page. continues on the following Table cart cult airway available naesth. 2008;18(7):676–7 naesth. cult to enter. Prevent hypothermia Prevent enter. cult to cult mask ventilation, intubation, and intubation, cult mask ventilation, J Clin Anesth. 2002;14(6):474–7 Anesth. J Clin brosis—hypoxia. Veins may be invisible, be invisible, may Veins brosis—hypoxia. Prepare for diffi Prepare diffi have access; venous Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively ICP increased have May Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively Check history of therapy and other drug steroid Scarring possible diffi of and mouth, face history. airway and intubation; have diffi have and intubation; airway Diffuse poor compliance. Chest restriction, hand. pulmonary fi diffi impalpable, Anesthesia implications ions and anesthetic considerations. channel blockers blockers channel ++ in pulmonale (rare or cor brosis stemic sclerosis: clinical manifestat sclerosis: stemic roderma in childhood. Rheum Dis Clin N Am. 2008;34(1):239–55 Am. Rheum Dis Clin N in childhood. roderma sm, microcephaly (“birdlike” facies), prominent prominent facies), (“birdlike” microcephaly sm, Autosomal recessive disorder, developmental delay, delay, developmental disorder, recessive Autosomal dwarfi nose pointed micrognathia, maxilla, Diffuse cutaneous stiffening. May have hemifacial atrophy. hemifacial atrophy. have May Diffuse cutaneous stiffening. and constrictions. Plastic surgery for contracture required fi cardiac have May cause of Esophageal common death). children—but organ less multiple have Children GERD. dilation leads to arthritis than adults—more and myositis. involvement Therapy may rarely be present. phenomena may Raynaud Ca methotrexate, include steroids, hydrocephalus, the nose; to Linear nevi the forehead from and/or coarctation have may delay; developmental ofhypoplasia aorta Description Seckel Seckel syndrome Name Scleroderma scle and localized sclerosis Systemic F. Zulian neviSebaceous syndrome, linear 2006;16:359–60 Anesth. Pediatr syndrome. for Seckel Anesthesia et al. Dayioglu H, T, Hosten Y, Gurkan 2007;17:1121 Anesth. Pediatr with syndrome. in a child Anesthesia Seckel J. Mayhew Demiralp G, A Paediatr management ofAnesthetic pacemaker. and implanted a patient withsyndrome Seckel et al. Z, Ozkose AT, Dogan Y, Unal Roberts JG, Sabar R, Gianoli JA, et al. Progressive sy Progressive et al. Gianoli JA, Sabar R, JG, Roberts 600 Appendix A: Anesthesia Implications of Syndromes… Anesth Analg. 2002;95:50–2 Analg. Anesth J Anaesth. 2014;31:336–8 J Anaesth. and intubation; cult mask ventilation cult airway cart at hand. Monitor blood Monitor cartcult airway at hand. uids and phenylephrine; vasopressin may be the may vasopressin uids and phenylephrine; with important; hypotension treat uid replacement Caution with potent inhalation anesthetics; accurate accurate Caution with inhalation anesthetics; potent fl IV fl muscle Use refractory treat hypotension. best agent to withrelaxants caution of Problem page 367. See pheochromocytoma, evaluate MEN 2B, For endocrinemultiple disorders. of before for the presence hypotonia muscular relaxants muscle considering Possible Possible diffi diffi have Caution with hypothermia. to Prone level. glucose block monitor relaxants; Anesthesia implications rst 100 years. Am J Surg Pathol. 2005;29(2):254–74 Pathol. J Surg Am rst 100 years. See lipoatrophy with diabetes See lipoatrophy diffuse degenerationOrthostatic of hypotension; central lability of nervous systems; and autonomic pulse and blood possibly because of baroreceptor pressure defective hypersensitivity to sweating; decreased response; and angiotensin catecholamines familial medullary thyroid MEN 2A, forms: Three more Pheochromocytoma and MEN 2B. carcinoma, % of in 75 in MEN2A and MEN2B (bilateral common adenoma parathyroid medullary carcinoma, thyroid cases), MEN endocrine multiple neoplasia. in MEN 2A), (common and with neuromas 2B also presents mucocutaneous hypotonia muscular Short stature, skeletal asymmetry, micrognathia. Low birth Low micrognathia. asymmetry, skeletal Short stature, endocrine abnormalities, Café au lait spots, weight. hypogonadism Description sm (multiple (multiple endocrine type adenomatosis 2 (MEN 2)) Name Seip syndrome Shy-Drager syndrome 1984;39(12):1229–31 Anaesthesia. syndrome. for Shy-Drager Anaesthesia JC. Sugden RC, Hutchinson vasopressin. to responsive hypotension intraoperative unexplained and severe syndrome Shy-Drager J. Lee DeSouza G, R, Vallejo Silver-Russell dwarfi 1994;78(6):1197–9 Analg. Anesth anesthetic implications. syndrome: Russell-Silver et al. R, Ward Goldin EZ, Dinner M, Eur syndrome. for orphan management ofAnaesthesia disease: an infant with Silver-Russell Driessen JJ. E, Verwijs RH, Passier Sipple syndrome endocrine the fi multiple neoplasia: Familial Carney JA. Appendix A: Anesthesia Implications of Syndromes… 601 nadir 2 cartcult airway at Table continues on the following page. continues on the following Table rome. Anesthesiology. Anesthesiology. rome. nea: houston, we have a problem! a problem! have we houston, nea: for sleep apnea strategy during 10 Use sterile technique. Anticipate airway and airway Anticipate sterile technique. Use the diffi have intubation problems; Muscle with relaxants muscle caution. Use hand. rigidity with inhalational anesthetics has been with is not associated but this syndrome described, withMH and has not been associated rhabdomyolysis. TIVA Consider Review sleep study ifReview sleep study severity done with to of attention nocturnal desaturation (if nocturnal SaO <85 % = opioid sensitivity may be increased). Assess opioid sensitivity be increased). may = % <85 risk Small of desaturation with preoperative airway. of Beware obstruction airway acute duringsedation. allow airway, induction ofIntubate anesthesia. assess opioid sensitivity. to spontaneous respiration only one-half of require one-third to usual dose ofMay weight. ideal body normalized to opioids (morphine), the clock as around such opioid sparing approach Use patient Awaken and ibuprofen. acetaminophen unit transfer postanesthesia care before to completely closely for apnea postoperatively. Monitor (PACU). (See Chap. Anesthesia implications anesthesia) is blunted. is blunted. 2 somnolence, loud snoring, restless sleep, insomnia, insomnia, sleep, restless loud snoring, somnolence, and aggressive. be hyperactive may Children fatigue. infant death syndrome), trauma, infections, or neoplasms, or neoplasms, infections, trauma, infant death syndrome), (Ondine and primary hypoventilation alveolar central without occurs evidence Apnea of respiratorycurse). activitymuscle hypertrophy, Pierre Robin syndrome, or any other or any syndrome, Pierre Robin hypertrophy, occurs Apnea obstruction. airway causing chronic condition because of increased by obstruction and is accompanied CO to Response respiratory activity. muscle Ventilatory depression with increased depression opioids is markedly Ventilatory decreased for analgesia markedly and opioid requirements Disorders of breathing during sleep, including the following ofDisorders during breathing sleep, Central CNS immaturity sleep apnea due to (sudden 1. adenotonsillar obesity, Obstructive sleep apnea due to 2. cleft Microcephaly, of synthesis. error Inborn cholesterol anomalies genital and skeletal delay, developmental palate, hypotonia; hypoplasia, thymic (including micrognathia), infection susceptibility to increased have may 3. Mixed forms. Medical history include daytime may Medical forms. Mixed 3. Description Name Sleep apnea syndromes 2005;23(3):535–49 Am. Clinics N Anesth in children. Obstructive sleep apnea syndrome et al. T, Trimarchi LJ, Brooks Bandla P, 1:100–8 2009;19 Suppl Anesth. Pediatr in children. breathing A disquisition on sleep-disordered J. Lerman with sleep ap a focus on obstructive in children injury Death or neurologic tonsillectomy after Domino KB. KL, Posner CJ, Coté 2014;118(6):1276–83 Analg. Anesth Smith-Lemli-Opitz syndrome management of and airway Anesthesia pediatric patients with synd et al. Smith-Lemli-Opitz L, Schwartz J, Veihmeyer Quezado ZM, 2002;97(4):1015–9 602 Appendix A: Anesthesia Implications of Syndromes… cult but is ideal cartcult airway at diffi have cult; ux. Avoid succinylcholine. Caution succinylcholine. Avoid ux. 2011;24(1 Suppl 2):21–3 2011;24(1 Suppl Intubation may be diffi may Intubation hand. Risk ofhand. GI refl Monitor effect. prolonged have with relaxants—may and post-op ventilation Evaluate blood glucose. caution with good analgesia; Provide support. provide be diffi analgesia may Regional opioids. Assess cardiac status (echocardiogram and ECG); SBE and ECG); status (echocardiogram cardiac Assess Intubation with Care asepsis. if indicated. prophylaxis with Care padding and be easy. to reported Hyperthermia during anesthesia is positioning head. and temperature monitor (not MH); reported analgesia may Regional as indicated. cooling institute in some cases be indicated Anesthesia implications Degeneration of leads to anterior spinal neurons Bulbar weakness. muscle generalized progressive and sensation normal Intellect be present. may dysfunction Macrocephaly, dilated cerebral ventricles but normal cerebral dilated Macrocephaly, growth Accelerated Hypotonia. delay. Developmental ICP. and GU Cardiac hernias. to Prone during childhood. response immune Reduced abnormalities in a few patients. Description (cerebral (cerebral Adhami EJ, Cancio-Babu CV. Anaesthesia in a child with Sotos syndrome. Paediatr Anaesth. 2003;13(9):835–40 Anaesth. Paediatr with syndrome. in a child Anaesthesia Sotos Cancio-Babu CV. EJ, Adhami Pharm Regional. J Immunol Int with syndrome. anesthesia in a child Regional Sotos et al. A, Vergari A, Messina A, Chierichini atrophy Spinal muscle 804–16 2013;23: Anesth. Pediatr atrophy. and spinal muscle Anesthesia G. Islander Name Sotos syndrome gigantism) Appendix A: Anesthesia Implications of Syndromes… 603 brillation may may brillation cult (because of Table continues on the following page. continues on the following Table cartcult airway at hand ocedure: a retrospective study. study. a retrospective ocedure: and pulmonary function. uid status, report of Oral a patient with Stickler syndrome. the diffi have cult; occur. IV infusion essential but avoid cutdowns if cutdowns IV infusion essential but avoid occur. is possible (possibility ofKetamine infection). the best anesthetic agent probably skin lesions) but essential; cover ECG pads with cover skin lesions) but essential; surgical lubricant beneath the patient. and place 35–37 °C; approximately to the operating room Warm closely; Monitor danger of hypothermia. severe serious arrhythmias fi and ventricular Preoperatively assess cardiac status (echocardiogram status (echocardiogram assess cardiac Preoperatively fl and ECG), sterile technique Use therapy. steroid Check for recent intubation and avoid Oral lesions: isolation). (reverse insertion gentle pharyngeal of esophageal stethoscope, on gauze Vaseline mask with soft face Use suctioning. tracheal tube secure If intubation is required, skin. do not padded; tape that is well with tracheostomy is diffi Monitoring tape. use adhesive status (echocardiogram. assess cardiac Preoperatively patients. Robin Pierre similar to problems Anesthesia be very and intubation may maintenance Airway diffi Anesthesia implications blebs are present, pneumothorax present, blebs are culties in anesthetic management of patients with micrognathia: Urticarial lesions; erosions of the mouth, eyes, genitalia. genitalia. of eyes, erosions lesions; the mouth, Urticarial agents (drugs, hypersensitivity exogenous to Possible If pleural etc.). infections, common. and malnutrition are Dehydration occur. may is similar care Medical pericarditis. myocarditis, have May that ofto with a burn children injury; be on some may therapy high-dose steroid with hypoplasia, dominant disorder midface Autosomal appearance. “moon-face” and cleft palate, micrognathia, and hearing loss. degeneration, retinal myopia, Progressive also are prolapse Spinal abnormalities and mitral valve common Description

Surg Oral Med Oral Pathol Oral Radiol Endodont. 2006;102(6):e33–6 Oral Radiol Endodont. Oral Pathol Oral Med Surg management of adhesion) pr 19 infants undergoing glossopexy (tongue-lip Perioperative et al. M, Takeuchi K, Tachibana M, Fujii 2015;25(8):829–33 Anaesth. Paediatr (erythema multiforme) Name Stevens-Johnson syndrome 1984;39(5):443–55 Anaesthesia. skin disease. and severe Anaesthesia AJ. Shribman GB, Smith 1989;26(10):1038–40 Pediatr. Indian syndrome. Johnson in Stevens Anesthesia et al. Dhar P, R, Chawla R, Madan Stickler syndrome Diffi et al. AM, Tuzuner O, Ozkaynak Z, Kucukyavuz 604 Appendix A: Anesthesia Implications of Syndromes… c anesthetic c :254–6 cause fade on TOF urane may Use caution with muscle relaxants; monitor effects monitor caution with relaxants; muscle Use (Sevofl carefully. but have succinylcholine to be resistant May monitor). non-depolarizing to muscle normal response Effect of Opioids neostigminerelaxants. is normal. be appropriate. may Propofol rigidity. increase apnea for perioperative Monitor with Care port wine have stains treated. Often instrumentation of larynx in case of undiagnosed hypertension or raised prevent to Care angioma. duringintraocular intubation or extubation. pressure with laser therapy; treated repeat LMA well Often other specifi No for this procedure. suited problems Anesthesia implications cation, convulsions, convulsions, cation, culty swallowing may occur. occur. culty swallowing may exing the head and legs toward the trunk. the trunk. the head and legs toward exing Rare, genetic syndrome. Severe muscle rigidity muscle Severe appears at genetic syndrome. Rare, Exaggerated startle birth years. and persists for several be spasms may Life-threatening is present. response fl by terminated and diffi vomiting, Choking, activity muscle continuous EMG shows diseases See collagen trigeminal angioma nerve distribution, over Cavernous mesodermal Developmental capillary usually unilateral. Intracranial calcifi Glaucoma. defect. laryngeal and tracheal Possible delay. developmental involvement See Williams syndrome Description

(juvenile (juvenile Name Stiff syndrome baby “startle (hyperekplexia, disease”) 2008;36(2) Care. Intens Anaesth implications ofAnaesthetic disease’. hyperekplexia—‘startle Sharma P. Ramachandran R, Garg R, Still disease Sturge-Weber syndrome 1994;41(2):133–6 Anaesth. Can J syndrome. and the Sturge-Weber Anaesthesia et al. R, Madan V, Gulaya Batra RK, 1999;16(5):339–41 Anaesthesiol. J Eur syndrome. for Sturge-Weber Anaesthesia et al. Basar H, Cakan T, A, Ceyhan aortic Supravalvar stenosis with idiopathic infantile hypercalcemia Murphy C, Shorten G. Train of four fade in a child with stiff baby syndrome. Paediatr Anaesth. 2000;10(5):567–9 Anaesth. of Paediatr with four fade in a child stiff syndrome. baby Train Shorten G. C, Murphy arthritis)rheumatoid Appendix A: Anesthesia Implications of Syndromes… 605 cult Table continues on the following page. continues on the following Table cartcult airway at hand. Preoperatively assess cardiac/coronary status assess cardiac/coronary Preoperatively counts. and platelet Hb, and ECG), (echocardiogram and muscle caution with depressants cardiac Use and avoid ischemia for cardiac Monitor relaxants. and hypertension as per adult with tachycardia heartischemic disease status (echocardiogram assess cardiac Preoperatively affect heart may and Hemosiderosis and ECG). Facial be severe. may Anemia hepatic function. deformity: of cause diffi overgrowth maxilla may diffi have intubation; for the anemic patient (see considerations Anesthesia for aseptic techniques to Special attention page 191). Heterozygous had a splenectomy. who have children form (thalassemia minor) poses no special anesthesia problems Anesthesia implications Low-plasma high-density lipoproteins; accumulation of accumulation high-density Low-plasma lipoproteins; and lymph spleen, in large orange esters tonsils, cholesterol Peripheral and thrombocytopenia. Anemia nodes. coronary premature and abnormal EMG; neuropathy arteriesdisease (lipid deposits found in coronary of a reports of (No heart ischemic disease in 6-year-old). children) See gangliosidosis GM2 congenita See myotonia syndrome See Osler-Rendu-Weber but is most race affect any disease that may Hereditary rate Slow Asians. and Southeast in Mediterranean common of of Hb synthesis and high present. percentage HbF are transfusion leading to repeated require Hb levels Low be used MRI may dysfunction. and cardiac hemosiderosis chelation require May assess severityto of overload. iron reduce may splenectomy or total Partial birth. therapy from the need for transfusions Description (Cooley (Cooley

Name Tangier disease 1996;83(2):427–9 Analg. Anesth disease. Tangier SW. Mentis Tay-Sachs disease Thomsen disease Telangiectasia, hemorrhagic Thalassemia major anemia) 2009;27:321–36 Anesthesiology Clinics. and hemoglobinopathies. Anesthesia Firth PG. (analphalipoproteinemia) (analphalipoproteinemia) 606 Appendix A: Anesthesia Implications of Syndromes… c ’s syndrome undergoing dental treatment syndrome ’s rst year (35–40 % mortality from (35–40 rst year anesthesia regimen is indicated, except that pimozide that pimozide except anesthesia regimen is indicated, (see interval syndrome QT cause prolonged may page 571) Establish rapport with patient and family. Continue Continue Establish rapport with patient and family. specifi No preoperatively. Sedate medications. Preoperatively assess cardiac function if assess cardiac CHD present Preoperatively transfusion Platelet for surgery(echocardiogram). or elective Avoid with hematologist. consult bleeding; surgery in the fi intracranial hemorrhage) Anesthesia implications to progresses cit disorder ciency: the incidence of associated medical and musculoskeletal conditions. J Hand Surg Am. Am. Surg J Hand ciency: conditions. of the incidence medical and musculoskeletal associated spasmodic repetitious movements that may become become that may movements spasmodic repetitious coprolalia exhibit also may Patient jerks. muscle powerful High (repetitions). and echolalia speech) (profane with haloperidol, ofincidence Treated migraine headaches. or pimozide clonidine, See Glanzmann disease deletion. a 1.q21.1 chromosomal Thought be due to to infection, stress, by precipitated thrombocytopenia Episodic adulthood. normal by to increase Platelets etc. surgery, % of cases (especially tetralogy of and Fallot CHD in 30 with also associated tracheoesophagealASD); and renal anomalies See Wiskott-Aldrich syndrome disease See Moschcowitz with disorder onset in neuropsychiatric Complex Attention-defi childhood. Description (TAR (TAR Name Thromboasthenia Thrombocytopenia with absent radius syndrome) Radial longitudinal defi PR. Manske L, Wall Goldfarb CA, Thrombocytopenia with and repeated eczema infections Thrombotic thrombocytopenic purpura Tourette syndrome 1986;65(2):200–2 Analg. Anesth anesthetic implications. syndrome: Tourette Lockhart CH. JE Jr, Morrison sedation and general anesthesia for a patient with Gilles de la Tourette Intravenous et al. H, Fukayama T, Takagi F, Yoshikawa 2006;31(7):1176–82 2002;46:1279–80 Scand. Anesthsiol Acta Appendix A: Anesthesia Implications of Syndromes… 607 cult or GlideScope beroptic or GlideScope beroptic enet A. enet A. Table continues on the following page. continues on the following Table be adjuncts must cult airway e. Can J Anesth. 1988;35:80–85 Anesth. Can J e. es. Pediatr Anesth. 2012;22:752–8 Anesth. Pediatr es. straction osteogenesis. Paediatr Paediatr straction osteogenesis. be adjuncts must cult airway the diffi have cult intubation; airway cart at hand. Diffi cartairway at hand. immediately available, and a fi available, immediately intubation should be anticipated Extremely Extremely diffi Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively a Having not be possible. may ventilation Mask surgeon capable of establishing a surgical is airway Diffi reasonable. and a fi available immediately airway topical intubation should be anticipated; analgesia with lidocaine will awake nebulized facilitate insertion of anesthesia induce an LMA and is used to or for intubation in the neonate and as a conduit before should be fully awake The child infant. tracheotomy require Some children extubation. Anesthesia implications ngers manage cult to yly syndrome is caused by recurrent mutation of MYH8. Am J Med G J Med Am of mutation recurrent MYH8. is caused by yly syndrome deformityexion of the fi us pseudocamptodactyly (Dutch-Kentucky or Hecht Beals) syndrom or Hecht us pseudocamptodactyly (Dutch-Kentucky llins syndrome: a review ofllins syndrome: management in 240 pediatric airway cas when wrist is extended. Short and foot deformities stature when wrist is extended. for surgery present mandible to May occur. may Micrognathia and slanting jawbone, aplastic zygoma, aplastic zygoma, and slanting jawbone, Micrognathia microtia. of coloboma eyelids, atresia, choanal microstomia, anomalies. and cardiac cleft palate have often Patients diffi more progressively becomes Airway are but often normal intelligence, have Most with age. hearing impaired mouth opening Decreased dominant condition. Autosomal of process the mandible and/or enlarged coronoid due to abnormal ligaments plus fl Description

Toydemir RM, Chen H, Proud VK, et al. Trismus-pseudocamptodact et al. VK, Proud Chen H, RM, Toydemir 2006;140(22):2387–93 Frawley G, Espenell A, Howe P, et al. Anesthetic implications ofAnesthetic with infants with treated mandibular di mandibular hypoplasia et al. P, Howe A, Espenell G, Frawley 2013;23(4):342–8 Anaesth. Name Treacher-Collins syndrome 2004;14:53–9 Anesth. in patients with Pediatr The airway craniofacial abnormalities. CN. Nargozian Co for Treacher Anesthesia A. Carlyle D, Zoanetti J, Hosking Trismus- pseudocamptodactyly syndrome) (Dutch-Kentucky implications for the trismAnaesthetic D. Blackstock H, Vaghadia (mandibulofacial dysostosis) (mandibulofacial dysostosis) 608 Appendix A: Anesthesia Implications of Syndromes… complex—follow-up of 20 children. of 20 children. complex—follow-up particularly tract in obstruction, ow Administer anti-seizure medications in the morning anti-seizure Administer assess cardiac Preoperatively of surgery. status; /creatinine) (BUN and renal (echocardiogram) spontaneously but may resolve to tumors tend cardiac cause right outfl the kidney, by with Care drugs excreted infants. especially radiocontrast MRI material during repeat Possible q6 months. assessments that generally occur arrhythmia of cardiac and rupture lung cysts. management depends on preoperative Anesthetic and limitations ofexamination organ functions normothermia and normocarbia Maintain to found. levels Check anticonvulsant seizures. prevent postoperatively Anesthesia implications hildren with tuberous sclerosis with sclerosis hildren tuberous cation, cation, spectrum of clinical manifestations in c seizures. May have tumors in the brain, heart, lungs, and lungs, heart, tumors in the brain, have May seizures. Cardiac occur. may failure and renal pyelonephritis kidneys; % of 90 in up to occur benign but may are rhabdomyomas (as the same in size remain to These tumors tend neonates. spontaneously less important)the heart or resolve grows Neurocutaneous condition with hamartoma condition growth in Neurocutaneous adenoma of sebaceous disease: skin, Multisystem body. intracranial calcifi delay, developmental epilepsy, Description Brain Dev. 2014;36(4):306–14 Brain Dev. Name Trisomies 13 Trisomy 18[E] Trisomy 21 Trisomy (TSC) sclerosis Tuberous syndrome See Edwards syndrome See Patau See Down syndrome 2000;10:121–8 Anesth. Pediatr management of with phakomatoses. congenital children Perioperative Diaz JH. Wide et al. MK, Bernhard A, Merkenschlager RR, Mettin Appendix A: Anesthesia Implications of Syndromes… 609 cult airway airway cult Table continues on the following page. continues on the following Table 128–30 t and what we should do in t and what we 2012;26:442–4 the diffi have cult; cartcult airway at hand. the diffi have cult; Examine neonates with any of withExamine neonates for other any these features assess cardiac Preoperatively lesions. congenital status. (BUN/creatinine) and renal (echocardiogram) of considerations by management dictated Anesthesia individual lesions cart at hand. Administer anti-seizure medications in anti-seizure Administer cart at hand. the morning of surgery Preoperatively assess cardiac (echocardiogram) and (echocardiogram) assess cardiac Preoperatively be may Intubation status. (BUN/creatinine) renal diffi drying). Gentle anticholinergics (excessive Avoid mucosal laryngoscopy prevent and intubation to and Airway be thickened. may cords Vocal trauma. be diffi intubation may Maintain normal blood pressures. Ensure thyroid thyroid Ensure normal blood pressures. Maintain with Care renal date. therapy is up to replacement drugs excreted Anesthesia implications cardiac C vertebral anomalies vertebral tracheoesophageal V T renal anomalies ( renal n in Turner syndrome: what we know, what we are unclear abou are what we know, what we syndrome: n in Turner R anal atresia, anal atresia, A cation and epilepsy may develop. Highly develop. cation and epilepsy may limb defects also in VACTERL) VACTERL) limb defects also in L cult airway to VACTERL anomaly: An anesthetic challenge. Anaesth Pain & Intens Care. 2013;17(2):192–4 Care. & Intens Pain Anaesth anesthetic challenge. An anomaly: VACTERL to cult airway esophageal atresia, esophageal atresia, E stula, stula, disease and fi (congenital scoliosis), scoliosis), (congenital XO females. Short stature, infantile genitalia, webbed neck; neck; webbed infantile genitalia, Short stature, females. XO dissecting CHD coarctation, possible micrognathia. aneurysm in of Hypothyroidism aorta or PS. (teenagers) % of cases than 50 anomalies in more Renal some cases. Be alert possibility to of Beckwith syndrome deposits or aphonia (eosinophilic hyaline-like Hoarseness be may in larynx and pharynx) Tongue and skin eruption. and friable. dry, membranes thickened, Mucous thickened. Intracranial calcifi parents withassociated consanguineous A nonrandom association of defects: Description

(gonadal clinical practice? Int J Adolesc Med Health. 2014;26(4):469–88 Health. Med Adolesc J Int clinical practice? (VACTERL association) (VACTERL 2011;16(6):56 Dis. Orphanet J Rare Association. VACTERL/VATER Solomon BD. Name Turner syndrome dysgenesis) 2008;29(7):219–27 Rev. Ped syndrome. Turner ML. Loscalzo 2005;17(2): Anesth. J Clin approach. a systematic syndrome: management ofAnesthetic Turner MA. Acquadro N, Sunder GA, Mashour hernia in infancy Umbilical Urbach-Wiethe disease 1989;63(5):609–11 Anaesth. J Br disease. Urbach-Wiethe Lipoid proteinosis: et al. D, Jonathan MT, Simpson JE, Kelly association VATER ofAssociation diffi K. Gurpreet S, Supriya D, Jain Ornek D, Aydin GB, Kahveci K, et al. Anesthetic management of a child with both Marfan syndrome and Turner syndrome. J Anesth. J syndrome. management ofAnesthetic and Turner withsyndrome a child both Marfan et al. K, Kahveci GB, Aydin Ornek D, dilatation and dissectio Aortic and Zacharin M. Cheung M, SC, Wong (cutaneous mucosal hyalinosis) 610 Appendix A: Anesthesia Implications of Syndromes… the have cult; ose of opioid or opioid-

cult airway cart at hand. May present for present May cartcult airway at hand. ciency: a literature review. Pediatr Anesth. Anesth. Pediatr ciency: review. a literature Preoperatively assess cardiac status (echocardiogram). status (echocardiogram). assess cardiac Preoperatively be diffi and intubation may Airway diffi occur Obstructive sleep apnea may pharyngoplasty. pharyngoplasty;after one-half to use one-third the normal weight normalized d sparing strategy with and ibuprofen. acetaminophen monitoring for apnea is recommended Postoperative Preoperatively assess cardiac (echocardiogram) and (echocardiogram) assess cardiac Preoperatively glucose fasting and administer Minimal status. liver is contraindicated Propofol infusions perioperatively. succinylcholine. Avoid vehicle). emulsion (due to Low-dose medications. depressant myocardial Avoid and regional analgesia, opioids, agents, volatile safe considered NSAIDS are Anesthesia implications culties due culties with very long-chain acyl-coenzyme A dehydrogenase defi A dehydrogenase with very acyl-coenzyme long-chain ssures, and retruded mandible. Associated Associated and retruded mandible. ssures, A phenotypic variant of the 22q deletion syndrome, diffi Speech DiGeorge syndrome. from separate learning disability velopharyngeal (mild), to anomalies, large nose facies: and characteristic VSD), CHD (especially narrow vertically long face, with nasal bridge, broad palpebral fi especially risk Long-term disorder for psychiatric OSA. schizophrenia. Disorder ofDisorder for fatty potential acid metabolism leads to and cardiomyopathy, failure, liver hypoglycemia, rhabdomyolysis Description ciency 2014;24:1115–19 Name Velocardiofacial syndrome 2009;2(2):149–67 Disabil. Intellect Res Health J Ment Syndrome. Velo-Cardio-Facial et al. E, Michaelovsky A, Frisch Gothelf D, Very long-chain acylcoenzyme A defi dehydrogenase agents in patients Anesthetic Stewart C. C, Redshaw Kirschner RE and Baylis AL. Surgical considerations in 22Q11.2 deletion syndrome. Clin Plast Surg. 2014;41(2):271–82 Clin Plast Surg. in 22Q11.2 deletion syndrome. Surgical considerations AL. RE and Baylis Kirschner Appendix A: Anesthesia Implications of Syndromes… 611 Table continues on the following page. continues on the following Table h. 1996;43:467–70 h. Paediatr Anaesth. 2006;16:680–3 Anaesth. Paediatr Preoperatively evaluate renal (BUN/creatinine) and (BUN/creatinine) renal evaluate Preoperatively infusion IV glucose Continuous hepatic status. lactated and perioperatively; avoid preoperatively blood sugar and acid-base Monitor solution. Ringer’s of Report Caution with propofol. balance. in a patient with pancreatitis this postoperative disease) similar Forbes III (Cori disease; Type disease. disease) VI (Hers but milder than type Type to I. but milder than typesimilar to I and (BUN/creatinine) assess renal Preoperatively for pheochromocytoma Investigate hepatic status. crises (urinary Hypertensive vanillylmandelic acid). management for Preoperative occur. may prior surgery. to days for several pheochromocytoma most is now tumor excision to approach Laparoscopic common Anesthesia implications a patient with glycogen storage disease type 1b. Can J Anaest Can J disease type a patient with storage glycogen 1b. cytoma: von Hippel-Lindau disease. J Neurosurg Anesthesiol. 2004;16:26–8 Anesthesiol. J Neurosurg disease. Hippel-Lindau von cytoma: Developmental delay, marked hepatomegaly, renal renal hepatomegaly, marked delay, Developmental and leucopenia, lactic acidosis, stomatitis, hyperplasia, bleeding diathesis (usually nose bleeds secondary to causes hypoglycemia Fasting aggregation). platelet impaired disturbances; biochemical Severe and convulsions. epinephrine to and glucagon. is unresponsive hypoglycemia syndrome). (see Fanconi syndrome Fanconi also have May hepatic tumors and pancreatitis Associated hemangioblastomas; angiomas and cerebellar Retinal pulmonary, have may in some; pheochromocytoma Paroxysmal cysts. renal adrenal, hepatic, pancreatic, tumor or cerebellar hypertension due to pheochromocytoma Description

(glycogen storage disease) storage (glycogen 2006;29(5):321–6 Res. Blood Press Kidney in the diagnosis and treatment of advances pheochromocytoma. Recent J Jr. Widimsky Name disease type Gierke von I management of Anaesthetic et al. S, Meretyk Y, Golub Shenkman Z, von Hippel-Lindau syndrome pheochromo Unsuspected G. Korula U, Gurunathan Bustamante SE, Appachi E. Acute pancreatitis after anesthesia with propofol in a child with glycogen storage disease type with storage anesthesia with in a child after glycogen propofol pancreatitis IA. Acute E. Appachi SE, Bustamante 612 Appendix A: Anesthesia Implications of Syndromes… (3):353–8 125:547–51 cartcult airway at with surgeon and hematologist ir of looking at craniosynostosis: hand. Caution with neck; may need in-line Caution with may neck; hand. blocking drugs; neuromuscular Titrate stabilization. effects of be may non-depolarizing relaxants muscle with care drugs If involved, kidneys are prolonged. kidneys material including contrast by excreted Preoperatively assess pulmonary (chest X-ray), renal renal assess pulmonary X-ray), (chest Preoperatively function and cardiac (BUN/creatinine), be could Intubation as indicated. (echocardiogram) diffi tumor; have by complicated Preoperatively Preoperatively consult Monitor optimal management strategy. develop to VIII VIII and bleeding time maintain factor factor % activity at >50 Anesthesia implications ow bromatosis. Paediatr Anaesth. 2002;12:374 Anaesth. Paediatr bromatosis. brinolytics help may vanillylmandelic acid). Often vanillylmandelic acid). management of a child with von Willebrand disease undergoing surgical repa management ofWillebrand with a child von esthetic implications of von Recklinghausen’s neurofi esthetic implications of Recklinghausen’s von brosing alveolitis “honeycomb (cystic) lung” predisposing to predisposing (cystic) lung” “honeycomb alveolitis brosing Café au lait spots (>5): tumors in all partsCafé au lait spots (>5): of the CNS and may peripheral with Tumors tumors associated nerve trunks. in the larynxoccur or trachea and right outfl ventricular have May be unstable. neck may %; tract; kyphoscoliosis in 50 fi artery Renal (hypertension) dysplasia pulmonary problems. % (all these patients in 1 Pheochromocytoma common. should be investigated—urinary a q6-month MRI surveillance require Prolonged bleeding time (decreased von Willebrand factor and factor Willebrand von bleeding time (decreased Prolonged VIII activity defective leading to factor decreased associated History of and capillary abnormality. adhesiveness) platelet Several etc.). bruising epistaxis, and bleeding (menorrhagia, types therapy. of to the response and determine the disease exist therapy for type is effective 1 (DDAVP) acetate IV desmopressin (Consult others. exacerbate and some type but may 2 patients, VIII concentrates. factor 2 and 3 require hematologist.) Types transfusions by of be controlled fresh-frozen Bleeding may Antifi plasma and/or cryoprecipitate. Description

M, Derham J, et al. Current management of von Willebrand disease and von Willebrand syndrome. Curr Opin Anaesthesiol. 2014;27 Anaesthesiol. Opin Curr syndrome. Willebrand disease and von Willebrand management of Current von et al. Derham J, M, eld DR, Bontempo FA, et al. Adenotonsillectomy in children with von Willebrand disease. Arch Otolaryng Head & Neck Surg. 1999; Surg. & Neck Otolaryng Head Arch disease. Willebrand with von in children Adenotonsillectomy et al. FA, Bontempo eld DR, bromatosis) bromatosis) (pseudohemophilia) Name disease Recklinghausen von Ana Martin M. de la Matta Delgado JM, von Willebrand disease Allen GC, Armfi (neurofi Maquoi I, Bonhomme V, Born JD, et al. Perioperative Perioperative et al. Born JD, V, Bonhomme I, Maquoi unusual targets. Anesth Analg. 2009;109(3):720–4 Analg. Anesth unusual targets. Mazzeffi ME, Stone Appendix A: Anesthesia Implications of Syndromes… 613 cult Table continues on the following page. continues on the following Table anemia. Johns Hopkins Med J. J. Med Hopkins Johns anemia. be less in older culties may cult ):443–52 Preoperatively assess cardiac (echocardiogram) and (echocardiogram) assess cardiac Preoperatively or cold, heat, trauma fat by to Prevent status. renal use cardiac blood volume; Maintain pressure. the kidneys by drugsdepressant and drugs excreted with caution Preoperatively assess severity ofPreoperatively pulmonary system is Succinylcholine ofand extent weakness. muscle Use hyperkalemia). (due to contraindicated or respiratory drugsrelaxants depressant with and support be required, may Ventilatory caution. be diffi may weaning Anesthesia implications Caution with airway and intubation; have diffi Caution with have and intubation; airway Diffi cartairway at hand. children ofrst year ed by consumption coagulopathy and microangiopathic hemolytic and microangiopathic coagulopathy consumption ed by cult intubation cult ciency. Pericardium: leads to restrictive pericarditis. pericarditis. restrictive leads to Pericardium: ciency. Skeletal overgrowth leading to craniofacial and digital leading to overgrowth Skeletal and short neck, micrognathia, Relative abnormalities. anterior larynx diffi lead to including the following: of situation, fat in any Necrosis adrenal or chronic cause acute tissue—may Retroperitoneal insuffi causes convulsions Meninges: with scoliosis onset in fi Onset in infancy; severe life. >95 % require gastrostomy feeding by 24 months of feeding by gastrostomy % require >95 life. to procedure is another common Fundoplication age. Most respiratory Chronic problems. aspiration. prevent pubertypatients die before Description

1970;126:344–9 Name Weaver syndrome 2005;15:893–6 Anaesth. Paediatr syndrome. Weaver in The upper airway D. Rohan MW, Crawford Weber-Christian disease nonsuppurative (chronic panniculitis) disease complicat Weber-Christian M. Coleman Lindo S, JL, Spivak Werdnig-Hoffman disease 2012;11(5 Neurol. Lancet and challenges. controversies atrophy: Childhood spinal muscular ST. Iannaccone Bertini E, E, Mercuri (infantile muscular atrophy, atrophy, (infantile muscular SMA type 1) 2007;86(5):349–55 Rehabil. Med J Phys Am disease. of considerations survival long-term Medical of JR. Bach Werdnig-Hoffmann 614 Appendix A: Anesthesia Implications of Syndromes… c Werner syndromes. Curr Aging Aging Curr syndromes. Werner es of pediatric patients with primary 6

2517–24 Preoperatively assess cardiac status (echocardiogram status (echocardiogram assess cardiac Preoperatively and ischemia and ECG) for evidence of myocardial manage If is present, diabetes dysfunction. as for patients with conducted Anesthesia accordingly. and tachycardia arterycoronary avoid disease; hypertension Preoperatively assess for renal and pituitary assess for renal Preoperatively of tumor. presence carcinoid dysfunction: with consult endocrinologist for specifi Preoperative at glucose Administer management issues. glucose perioperative and monitor rates maintenance tumors (page 536) See also carcinoid values. Anesthesia implications y organ systems usually y organ systems : molecular alterations leading to Hutchinson-Gilford progeria and progeria Hutchinson-Gilford leading to molecular alterations : docrine neoplasia syndromes in children. Horm Res. 2007;68(Suppl 5):105– 2007;68(Suppl Res. Horm in children. docrine neoplasia syndromes multiple endocrinemultiple neoplasia type the surgical outcom 1 diagnosis improves Hyperparathyroidism, anterior tumors of pituitary, anterior tumors of pituitary, Hyperparathyroidism, insulinomas of (hypoglycemia), the pancreas common. and gastric are ulcers hyperparathyroidism, failure Renal occur. tumors of may tree Carcinoid bronchial These patients require is possible. kidney stones due to MRI evaluations offrequent man in delay developmental %, in 50 aging; diabetes Premature cardiac bone lesions, osteomyelitis-like early cataracts, %, 50 and failure infarction, on a yearly basis on a yearly Description (multiple (multiple Sci. 2008;1(3):202–12 Sci. Name Wermer syndrome Endocrine surgical aspects of B. en multiple Harrison, Werner syndrome 2007;101:13–26 Res. Circ aging syndromes. of disease in accelerated cardiovascular Mechanisms EG. Nabel FS, Collins Capell BC, endocrine type adenomatosis 1 (MEN1)) aged children Prematurely D. Araujo-Vilar Dominguez-Gerpe L, hyperparathyroidism. J Pediatr Surgery. 2014;49(4):546–50 Surgery. J Pediatr hyperparathyroidism. Peranteau WH, Palladino AA, Bhatti TR, et al. The surgical management of insulinomas in children. J Pediatr Surg. 2013;48(12): Surg. J Pediatr The surgical management of et al. insulinomas in children. TR, Bhatti AA, Palladino WH, Peranteau Preoperative et al. TA, Rich LF, Morris AMA, Romero Appendix A: Anesthesia Implications of Syndromes… 615 Table continues on the following page. continues on the following Table issencephaly. Pediatr Anaesth. Anaesth. Pediatr issencephaly. Administer anti-seizure medications in the morning anti-seizure Administer glucose- then avoid If diet, on ketogenic of surgery. administer Ringer’s; solutions and lactated containing One report describes BIS very normal low saline. patient with paradoxical “awake” in an values noted may BIS monitor duringchanges anesthesia induction. Parents anesthesia depth. monitor to be unreliable is when their child be helpful in determining may “awake” Anesthesia implications cits, cits, Infantile spasms, hypsarrhythmia on EEG, and hypsarrhythmia on EEG, spasms, Infantile defi neurologic Seizures, impairment. psychomotor lissencephaly, and severe developmental delay. Long-term Long-term delay. developmental and severe lissencephaly, Some patients required. treatment with anticonvulsants diet with treated ketogenic successfully Description Name West syndrome and l syndrome West with children BIS values in two preanesthetic low Extremely et al. A, Machotta TG, de Leeuw AJ, Valkenburg 2008;18:446–8 2013;36(1):2–8 J. Biomed Dietary therapies HS. for epilepsy. Wang EH, Kossoff 2013;27(3):175–84 CNS Drugs. of epileptic encephalopathies. Treatment JH. Cross A, McTague 616 Appendix A: Anesthesia Implications of Syndromes… cult; have have cult; ne institution’s experience. Anaesth Anaesth experience. ne institution’s report and review of literature. Ann Ann report and review of literature. of High incidence cartcult airway cardiac at hand. Preoperatively assess cardiac (echocardiogram, ECG, ECG, (echocardiogram, assess cardiac Preoperatively renal arteryand coronary anatomy/obstruction), Check and calcium. function (BUN/creatinine), perioperatively. calcium levels Monitor history. steroid be diffi and intubation may ventilation Mask diffi A careful duringarrest induction of anesthesia. inhalation induction withsmooth very slow modest but an doses of be used, inhalation agent may induction with an opioid-based intravenous Avoid and preferred. anesthetic is recommended and drugs depressants causing tachycardia. cardiac avoid loading is essential to volume Generous may echocardiography Transesophageal hypotension. This cohort has a high aid anesthetic management. arrest cardiac in the perioperative representation website: registry!Also check cautious! Be extremely www.williams-syndrome.org Anesthesia implications some with n facies, cult to resuscitate. Sudden Sudden resuscitate. cult to haemodynamic complications in Williams syndrome patients: a review patients: of syndrome o Williams in complications haemodynamic Cardiac anomalies: usually supravalvular aortic stenosis, aortic usually supravalvular stenosis, anomalies: Cardiac peripheral pulmonary artery and obstruction, elfi (IQ 40–80), delay developmental micrognathia, “party personality.” Fixed cardiac output and cardiac Fixed “party personality.” micrognathia, Very and angina. dyspnea leading to ischemia myocardial high anesthetic risk and diffi calcium diet, low require Hypercalcemia, occur. death may Hypothyroidism surgery. corrective and cardiac steroids, be present abnormalities may and renal Description n (elfi Name Williams syndrome 2005;15:243–7 Anaesth. Pediatr syndrome. of care Williams the patient with Perioperative JD. Tobias P, Russo J, Medley a case syndrome: death under anesthesia in pediatric cardiac Williams patient with Sudden et al. S, Goyal JD, Tobias P, Gupta Cardiac Anesth. 2010;13:44–8 Anesth. Cardiac Anaesthesia-related et al. DA, Costi CJ, Fahy Olsen M, facies syndrome) facies syndrome) Intens Care. 2014;42:619–24 Care. Intens Appendix A: Anesthesia Implications of Syndromes… 617 Table continues on the following page. continues on the following Table Anaesth. 2002;12:665–73 Anaesth. w of the literature. Pediatr Pulmonol. Pulmonol. Pediatr w of the literature. Preoperatively assess renal (BUN/creatinine) and (BUN/creatinine) assess renal Preoperatively that do not relaxants muscle Use hepatic status. function depend upon hepatic or renal responses. unpredictable (cisatracurium) avoid to kidneys by withCare drugs excreted Antibiotic prophylaxis may be indicated be indicated may prophylaxis Antibiotic of blood and platelets Transfusions preoperatively. be must All blood products be required. may Use graft-versus-host reaction. irradiated prevent to isolation) (reverse sterile technique Preoperatively assess cardiac (echocardiogram) and (echocardiogram) assess cardiac Preoperatively a history of have May pulmonary status. X-ray) (chest Monitor therapy requiring supplementation . steroid for apnea postoperatively Anesthesia implications ciency. Curr Opin Hematol. 2013;20(6):501–8 Opin Hematol. Curr ciency. deposits, especially in the deposits, with brosis cystic interstitial major abdominal surgery in children with liver disease. Paediatr disease. major abdominal surgery with liver in children brosis brosis brosis. Pathogenesis unknown; possibly due to possibly due to unknown; Pathogenesis brosis. toxicity or barotrauma toxicity 2 Decreased ceruloplasmin; copper ceruloplasmin; Decreased lung chronic (<1500 g birthPrematurity severe weight); generalized fi disease leading to of production Decreased disease. recessive This is an x-linked herpes simplex severe hypersusceptibility to microplatelets; asthma, eczema, mechanism), immune infections (disordered of 10 years die before age, Most and lymphoid malignancy. generalized herpes from or opportunistic infection. many results with transplant cell mixed has been attempted Stem liver and CNS motor nuclei. Renal tubular acidosis; hepatic tubular acidosis; Renal nuclei. and CNS motor liver fi due to failure right aspiration, infection, chest repeated emphysema, try to prevent be given to may Steroids failure. ventricular pulmonary fi O Description Kaler SG. Inborn errors of copper metabolism. Handb Clin Neurol. 2013;113:1745–54 Clin Neurol. Handb of errors Inborn metabolism. copper SG. Kaler 2008;43(10):1004–12 Name Wilson disease (hepatolenticular degeneration) of The choice inhalation anaesthetic for EM. Ashley DW, Green Wilson-Mikity syndrome 2004;13:174–180 Surg. Sem Pediatr implications of respiratory problems. common Perioperative AF. Stasic diagnostic updated criteria based on nine cases and a revie syndrome: Wilson-Mikity et al. A, Petrocheilou Seear M, A, Hoepker Wiskott-Aldrich syndrome 2008;15:30–6 Opin Hemat. Curr syndrome. Wiskott-Aldrich HD. Ochs CH, Miao LD, Notarangelo transplantation cell for primary Stem defi immune P. Veys Booth C, AJ, Worth 618 Manual of Pediatric Anesthesia 24(6):982–3 Treatment is entirely supportive, but if anesthesia is supportive, is entirely Treatment assessments of then appropriate cardiac, required, are and hematologic systems hepatic, pulmonary, advised Preoperatively review cardiac status with review cardiac cardiologist; Preoperatively review and ECG if echocardiogram with associated During or pancuronium. atropine Avoid CHD. be anesthetic may propofol studies, electrophysiologic of little effect on abnormal having conduction choice pathways Anesthesia implications undergoing radiofrequency urane-based anesthetics in children cation. Resembles Niemann-Pick disease, disease, Niemann-Pick Resembles cation. eath-holding spells in a child with Worster-Drought syndrome. J Anesth. 2010; Anesth. J syndrome. Worster-Drought witheath-holding spells in a child Death ltration (of including myocardium.) all tissues, A form of palsy with onset epilepsy cerebral childhood appropriately monitor apnea is possible; Postoperative Failure to thrive because of thrive visceral to xanthomatous changes: Failure calcifi adrenal and foam cell hypersplenism, with hepatosplenomegaly, infi with enzyme replacement 6 months of Trials usually by age. some promise ongoing and show are syndrome See cerebrohepatorenal Anomalous conduction path between atria and ventricles. atria path between and ventricles. conduction Anomalous QRS with interval; prolonged phasic ShortECG: P-R supraventricular paroxysmal to Prone %. variation in 40 Infants, defects. other cardiac have May (SVT). tachycardia be May SVT. to very prone are especially preterm, Paroxysmal arrhythmias. to Prone withassociated CHD. SVT on induction of treat anesthesia has been reported; blockers or calcium channel beta blockers with adenosine, verapamil) (e.g., Description

(familial Worster-Drought Worster-Drought syndrome catheter ablation for supraventricular tachycardia. Anesthesiology. 2002;96(6):1386–94 Anesthesiology. tachycardia. ablation for supraventricular catheter Name Wolff-Parkinson-White Comparison of effects of and isofl et al. electrophysiologic propofol JM, Hall RJ, Kanter Erb TO, Wolman disease xanthomatosis) br cyanotic Postoperative DK. Baidya A, Chhabra syndrome Zellweger (WPW) syndrome APPENDIX B Cardiopulmonary Resuscitation, Including Neonatal Resuscitation

Cardiopulmonary resuscitation (CPR) is concerned with the restoration of pul- monary and cardiovascular function and the prevention of neurologic damage. Initially, it consists of artifi cial ventilation and artifi cial circulation by whatever means are immediately available. This is termed basic life support . Its object is to prevent clinical death from progressing to biologic death before other remedial measures (i.e., advanced life support ) can be instituted. As in adults, heroic resuscitative efforts may not be indicated in children with lethal terminal disease. This is a decision that should be made in advance and clearly documented and communicated. The overall success rate for pediatric CPR is worse than for adults, especially if success is defi ned as long-term survival without neurologic defi cit. A possible reason is that the majority of cardiac arrests in children result from hypoxemia. In such children, it must be assumed that by the time the heart has suffered hypoxia enough to stop it, the brain has also suffered hypoxia enough to severely damage it. This being so, every effort must be directed at detecting and treating any respiratory compromise before it leads to serious hypoxemia.

PREVENTION OF CARDIAC ARREST

Awareness of precipitating factors is essential in preventing cardiac arrest in children. Common causes include: 1. Failure of ventilation: (a) Due to central depression, airway obstruction, or primary pulmonary disorders (b) Secondary to regurgitation and pulmonary aspiration (c) Secondary to neurologic and neuromuscular disorders (i.e., residual neuromuscular block) 2. Hypovolemia.

© Springer International Publishing Switzerland 2016 619 J. Lerman et al., Manual of Pediatric Anesthesia, DOI 10.1007/978-3-319-30684-1 620 Appendix B: Cardiopulmonary Resuscitation…

3. Toxicity (drugs, poisons, toxins). 4. Primary cardiac disorders. (These account for only a small percentage of car- diac arrests on the general wards of a pediatric hospital.) Prevention requires: 1. Recognition of potential causes 2. Constant surveillance 3. Early recognition of respiratory failure

Special Hazards for Children

Anesthesiologists should constantly be aware of factors that may be insignifi cant in the adult but may rapidly be life-threatening in infants and children; 1. The upper airway may become obstructed by: (a) Laryngospasm (common); due to small amounts of mucus or blood or inadequate or unwisely planned anesthesia (see Chap. 4 ) (b) Hypertrophied adenoidal tissue and/or enlarged tonsils, which may completely block the airway (c) The relatively large tongue, associated with: • Muscle fl accidity in the anesthetized patient • Inadvertent displacement or compression of submental soft tissue and tongue by the anesthesiologist’s fi ngers • Inadequate neck extension • Inadequate elevation of the mandible • Premature removal of an artifi cial airway (d) Regurgitated stomach contents—a common occurrence because of the frequency of feedings Remember : Infants have large tongues and may be primarily nose breathers. If the nasal airway is inadequate, an oral airway should be inserted without delay. 2. Ventilation may be compromised if the stomach becomes infl ated, usually a result of: (a) Excessive infl ation pressures (b) Partial airway obstruction (c) Crying and swallowing air After protecting the airway with a tracheal tube, pass a No. 10 or 12 suction catheter into the stomach and aspirate the contents to reduce the possibility of aspiration. 3. Blood volumes are small—signifi cant hypovolemia may develop rapidly. Appendix B: Cardiopulmonary Resuscitation… 621

Routine Precautions

Preoperative 1. Be prepared to give atropine to all young children who are scheduled for laryngoscopy/tracheal intubation or to receive cholinergic drugs (i.e., halo- thane, succinylcholine) or have surgery that may elicit vagal refl exes that may result in bradycardia and asystole. Vagal refl exes are brisk and may lead to cardiac arrest. The greater the delay before giving atropine, the greater the time until it takes effect. ( N.B. atropine does not correct hypoxemia!)

2. Always give 100 % O2 before intubating the trachea in children. (Desaturation occurs much more rapidly in children, particularly infants, than in adoles- cents). Intubate the trachea as quickly and smoothly as possible. 3. Select the tracheal tube size carefully, secure it fi rmly, and check its position;

confi rm EtCO2 and listen to both sides of the chest. Position and support the tracheal tube so that it cannot kink. (These procedures are critical in children.)

Perioperative 1. Carefully maintain a patent airway and adequate ventilation. 2. Monitor the following constantly: (a) Heart and lung functions by stethoscope, ECG, and NIBP

(b) SaO 2 and PetCO2 (do not disable alarms) (c) Body temperature (d) Blood loss 3. Meticulously measure all gases, vapors, and drugs; always read the drug label. 4. Measure fl uid losses accurately and replace as indicated. (Even a small loss is signifi cant in a small child.) Ensure that there is a reliable, generous sized IV route before allowing surgery to start. 5. Remember that rapid infusion of blood products may cause hyperkalemia or hypocalcemia (especially when given into a central vein in small infants). 6. Prevent unintentional pressure on the chest and abdominal wall from dress- ings, hands, and surgical assistants leaning on the drapes. 7. If problems become apparent, advise other members of the team (especially the surgeon) immediately.

Postoperative Note: Cardiac arrest in the postanesthesia care unit (PACU) is as common as in the operating room: 1. For all infants and all seriously ill children: do not extubate the trachea unless and until the child is reacting vigorously. 622 Appendix B: Cardiopulmonary Resuscitation…

2. Whenever possible, all children should be transported to the PACU in the lateral position, with the upper leg fl exed at the hip and knee and the neck moderately extended (the “tonsil” or “recovery” position). Administer oxy-

gen and monitor respiration. SaO2 is an unreliable monitor of ventilation in the presence of oxygen. 3. In the PACU:

(a) Immediately monitor SaO2 , NIBP, and ECG. (b) Provide a full report to the PACU nursing staff regarding underlying medical problems, surgical problems, medication doses and time of administration, and anticipated possible PACU problems. (c) Ensure that the child remains safely positioned with a clear airway:

• Order humidifi ed O 2 by mask until the child is responding well. • Document stable vital signs at handoff. (d) Do not leave until you are assured vital signs are stable and have handed over care of your child to a nurse. (e) Before discharge from the PACU, ensure that the danger of drug-induced respiratory depression has passed and that the child is fully conscious. (f) Some neonates and infants need to be stimulated frequently to maintain ventilation. (g) All former preterm infants of less than 60 weeks conceptual age and those with a history of chronic respiratory disease should be monitored on an apnea alarm for at least 24 h or 12 h apnea-free (see Chap. 2) . (h) Children with obstructive sleep apnea (OSA) require extended observa- tion and monitoring and may be sensitive to the respiratory depressant effects of opioids (see Chap. 10 ) .

Treatment of Arrhythmias

Arrhythmias that cause hemodynamic compromise or those that might prog- ress to cardiac arrest must be promptly treated. The advice of a pediatric cardi- ologist should be obtained whenever this is possible.

Supraventricular Tachycardia (SVT) (a) SVT (heart rates >220 bpm in infants or greater than 180 bpm in children suggest SVT) may be diffi cult to differentiate from sinus tachycardia, but the history and the heart rate usually clarify the diagnosis. (b) Early consultation with a pediatric cardiologist is recommended for hemodynamically stable VT. Vagal maneuvers ( Valsalva maneuver or ice applied to face) are recommended as initial therapy. Adenosine may be administered and if this is unsuccessful amiodarone may be indicated Appendix B: Cardiopulmonary Resuscitation… 623

(see Appendix A for dosing). If circulatory instability is present, immediate synchronized electrical cardioversion (0.5–1 J/kg) is recommended.

Ventricular Tachycardia (VT) and Ventricular Fibrillation (VF) (a) For VT with hypotension but with a palpable pulse, immediate synchro- nized cardioversion is recommended. Children with less hemodynamic compromise should be assessed for the cause of the VT and may be sedated before cardioversion. (b) For pulseless VT and VF, chest compressions followed by very prompt defi - brillation is recommended. If this is unsuccessful or VT recurs, amiodarone 5 mg/kg may be considered.

Non-shockable Rhythm (Asystole and Pulseless Electrical Activity [PEA]) Asystole and PEA are the most common ECG fi ndings in cardiac arrest in infants and children. PEA is a cluster of slow, wide QRS complexes in the absence of palpable pulses. CPR should be continued; defi brillation is not indicated. Underlying causes should be sought.

CARDIOPULMONARY RESUSCITATION

The guidelines for cardiopulmonary resuscitation and neonatal resuscitation are based on peer reviewed scientifi c publications that are critically analyzed to derive a consensus. This appendix incorporates the recommendations from their most recent International Liaison Committee on Resuscitation (ILCOR) deliberations (2010).

Basic Life Support

N.B. For basic life support in hospitals, use a bag and mask as soon as possible to prevent risk of infection to hospital personnel. Make sure that such equip- ment is immediately available and functioning in all patient-care areas. In rare instances, it may be necessary to resort to mouth-to-mouth resuscitation. Do not leave the child. Call for help and equipment (including a defi brillator). Begin with the ABCs: 1. Airway: check patency and apply jaw thrust. 2. Breathing (four ventilations) preferably with bag and mask. 3. Cardiac activity: check with stethoscope—palpate brachial artery in an infant and femoral or carotid artery in a child. 624 Appendix B: Cardiopulmonary Resuscitation…

When called to resuscitate a child, assess the situation immediately according to the following priorities: 1. Check ventilation: (a) If there are respiratory efforts: • Position the child in the lateral position (recovery position) to provide a clear airway and decrease the risk of aspiration should vomiting occur.

• Give O2 by mask as soon as it becomes available. (b) If respiratory efforts are present, but evidence of airway obstruction is pres- ent (breath sounds absent, intercostal retraction, fl aring of lateral chest margins, cyanosis): • Pull the tongue or mandible forward (by pressing behind the man- dibular condyles) and remove any foreign matter from the pharynx, keeping the mouth slightly open. • Extend the neck if necessary (caution if neck injury—but remember that ventilation is the fi rst priority).

• Give O2 by mask. • Check for improved chest movement and breath sounds. 2. If there is no respiratory effort or ventilation appears inadequate: (a) Begin positive pressure ventilation at once. (b) Ventilate directly, mouth to mouth if necessary, until resuscitation equip- ment is placed in your hand. (The small infant face necessitates applica- tion of your mouth to the infant’s mouth and nose.) An infant’s tidal volume is small (8–10 mL/kg); therefore, only puffs are necessary. As soon as possible, begin ventilation using a bag and mask with oxy- gen. Assure adequate expansion of the chest with each breath. 3. If cardiac activity is undetectable by auscultation or by femoral, carotid, or brachial artery palpation (or if the heart rate is <60 bpm despite ventilation and oxygenation and there are signs of poor perfusion [pallor, cyanosis]): (a) Start external cardiac compression at once (“push hard, push fast”): • The site of compression in an infant is one fi nger-breadth below the intermammary line; in a child, it is over the lower sternum, one fi n- ger-breadth above the xiphisternum. In an infant, two fi ngers may be used. In a child, the heel of the hand should be used. • Depth of compression: one-third to one-half of the anteroposterior diameter of the chest; between compressions, release completely to allow the chest wall to fully recoil. • Rate of compression: 100/min for infants, children, and adolescents. Appendix B: Cardiopulmonary Resuscitation… 625

• Rate of ventilation (basic life support—unintubated airway): – Single-person technique: 2 ventilations for 30 compressions. – Two-person technique: 2 ventilations to 15 compressions. – For intubated airways, a rate of 100 cardiac compressions per min-

ute and 8–10 ventilations with 100 % O 2 per minute should be ini- tiated immediately. Prevent hyperventilation. – Do not interrupt the compressions for ventilation. – Apply cardiac compressions to infants by encircling the chest with your hands (Fig. B.1 ). This method results in a larger cardiac out- put than anterior sternal compression alone.

Fig. B.1 Two-handed method of external cardiac compression . Note how both hands encir- cle the chest and how both thumbs are used for cardiac compression. (From Todres D, Rogers MC: Method of external cardiac massage in the newborn infant. J Pediatr 86:781, 1975, with permission)

Defi brillation

Early defi brillation is recommended for children who have ventricular fi brilla- tion or pulseless ventricular tachycardia. Defi brillation is more successful after effective chest compression in adults. These are more likely when there has been a sudden unexpected arrest: 1. For infants and children weighing less than 20 kg, use pediatric defi brillator plates (diameter of 4.5 cm for infants and 8 cm for children). 2. Set the machine to deliver shocks appropriate to the child’s size (to maximize the chance of success and minimize the danger of electrically induced myo- cardial damage); 2 J (W s)/kg should be the initial setting. 3. Give one shock and then immediately resume CPR for 2 minutes and assess response. If unsuccessful, the dose should be doubled to 4J. 4. Many AEDs have the potential to detect pediatric “shockable” rhythms and can be adjusted to deliver appropriate energy to each shock. Ensure that AEDs installed in a pediatric care environment conform to these requirements. 626 Appendix B: Cardiopulmonary Resuscitation…

5. Children who are digitalized should be treated with the reduced power set- tings initially; then the power setting is gradually increased. Normal doses of countershock may cause irreversible cardiac arrest in the presence of bound digitalis in the heart muscle.

ADVANCED LIFE SUPPORT

The foregoing provides only basic interim resuscitation. Most children also require:

1. Ventilation with O 2 as soon as it is available. Ventilation and oxygenation are the fi rst line of therapy for the acidosis that accompanies cardiac arrest. 2. Establish an airway by rapidly intubating the trachea; a cuffed tube may be used when appropriate to protect the airway. (The laryngeal mask airway may

be useful in some instances.) Exhaled CO2 detection is recommended for early confi rmation of successful intubation and to ensure adequate pulmonary circulation with chest compressions. Do not delay ventilation during lengthy attempts at intubation: ventilation is essential, intubation is optional. 3 . D e fi nitive ECG diagnosis of cardiac activity and defi brillation if indicated (see previous discussion). 4. Establish an intravenous or intraosseous route for drug administration. Intratracheal drug administration is no longer recommended. 5. Supportive drugs, primarily epinephrine (see later discussion). 6. Further pharmacologic and medical treatment, including fl uid replacement, as indicated. 7. Consideration of the possibility of lung injury by aspirated acidic gastric contents. 8. Early assessment of neurologic function—plan early and continue treatment to minimize and prevent further hypoxic-ischemic brain damage.

DRUG THERAPY

Although subsequent drug therapy is necessarily individualized, a standard ini- tial protocol is advantageous: 1. Epinephrine: To be maximally effective, it must be given intravenously (pref- erably into a central vein) or, if this is not possible, by the intraosseous route: (a) Initial and subsequent doses: 10 μg/kg (0.1 mL/kg of a 1:10,000 solu- tion). Continue CPR for 2 minutes and assess response. (b) High-dose (100 μg/kg) epinephrine is no longer recommended (except possibly in the treatment of β-blocker overdose). Appendix B: Cardiopulmonary Resuscitation… 627

2. Vasopressin has been successful in some cases of prolonged cardiac arrest but has not resulted in increased survival to a neurologically intact hospital discharge. 3. Dopamine infusion may be required for continued hypotension and poor tissue perfusion; 5–20 μg/kg/min may be titrated to achieve the desired effect. 4. Sodium bicarbonate administration is no longer recommended as a routine; but it might be considered in prolonged cardiac arrest for documented con- tinuing severe metabolic acidosis despite adequate ventilation, oxygenation, and chest compressions. It may also be useful in the treatment of hyperkale- mia, hypermagnesemia, or tricyclic antidepressant overdose. N.B. Administration of excessive doses of sodium bicarbonate produces hyperosmolarity, hypernatremia, hypokalemia, decreased ionized calcium, impaired cardiac action, and possibly severe alkalosis after recovery. 5. Calcium is no longer recommended except as the defi nitive treatment for hyperkalemic-induced arrhythmias or arrest and citrate-induced hypocalcemia. 6. Glucose: Documented hypoglycemia should be treated by glucose infusions. Otherwise, avoid any glucose administration because hyperglycemia (glucose >200 mg/dL) may compromise neurologic outcome after a hypoxic event.

Route of Administration of Drugs

Inject epinephrine into a central line, if one is available; otherwise, use a peripheral intravenous or intraosseous line. The tracheal route is only used as a last resort. Intracardiac injections should not be made. Damage to the heart and coro- nary arteries and/or a pneumothorax may result.

Fluid Replacement

1. Insert a large-bore intravenous cannula as soon as possible: (a) To provide a route for drug therapy. (b) For rapid replacement of fl uid. (In cardiac arrest, hypoxic capillaries leak rapidly, diminishing the circulating blood volume.) 2. Replace losses initially with crystalloids and later with colloids (plasma or blood) as indicated. N.B.: (a) Even a child previously in congestive heart failure needs infusions total- ing at least 10 % of the expected blood volume (EBV; equal to approxi- mately 1 % of body weight). 628 Appendix B: Cardiopulmonary Resuscitation…

(b) With recovery, the extravasated fl uid returns slowly to the vascular com- partment, giving time for assessment of fl uid volume and a decision as to whether diuretic therapy is necessary. 3. Avoid the use of dextrose-containing solutions. They may cause hyperglyce- mia, which may compromise cerebral survival. If hypoglycemia is suspected, it should be confi rmed by blood glucose determination and treated accordingly.

Post-resuscitation Care

Emphasis is placed on this as being critical to a favorable outcome: 1. Hyperventilation may be harmful and should be avoided: maintain normocarbia. 2. Induced hypothermia (32–34 °C) for 12–24 h may be considered for children who remain comatose after CPR. 3. Prevention of hyperthermia is essential. 4. Hemodynamic support using vasoactive drugs (e.g., dopamine) should be used when necessary to improve the circulatory status. 5. Target blood glucose levels to achieve normal levels. 6. Restrict fl uid replacement: avoid large infusions of crystalloid solutions once cardiovascular stability is ensured. 7. Treat seizure activity with phenobarbital and/or phenytoin (Dilantin). 8. Obtain an early neurologic consultation. 9. Maintain cerebral perfusion pressure. References Berg MD, Nadkarni VM, Berg RA. Cardiopulmonary resuscitation in children. Curr Opin Crit Care. 2008;14(3):254–60. Berg MD, Schexnayder SM, Chameides L, et al. Part 13: Pediatric basic life support: 2010 American Heart Association guidelines for cardiopulmonary resuscitation and emer- gency cardiovascular care. Circulation. 2010;122:S862–75. Dingeman RS, Mitchell EA, Meyer EC, et al. Parent presence during complex invasive procedures and cardiopulmonary resuscitation: a systematic review of the literature. Pediatrics. 2007;120(4):842–54. Shaffner DH, Heitmiller ES, Deshpande JK. Pediatric perioperative life support. Anesth Analg. 2013;117:960–79. Sharman M, Meert KL. What is the right dose of epinephrine? Pediatr Crit Care Med. 2005;6(5):592–4. Appendix B: Cardiopulmonary Resuscitation… 629

NEONATAL RESUSCITATION

The anesthesiologist is frequently called upon to assist at or manage the care of the neonate immediately after birth. Neonatal resuscitation must be based on a detailed knowledge of the normal physiologic changes that occur during transition to extrauterine life (see Chap. 2 ) plus a recognition of the pathologic processes in the mother or the fetus that may affect the infant at this time. Most infants require little help. Those born at term, with clear amniotic fl uid, who are crying or breathing and have good tone should be dried and kept warm. No further interventions are necessary. Others, however, require rapid intervention if serious sequelae are to be pre- vented. Preexisting maternal or fetal disease and/or events during labor may affect the neonate’s status after delivery. Frequently, infants at risk may be recog- nized before birth, and preparations can then be made for their immediate resuscitation on delivery. However, some infants who have demonstrated no antenatal signs of distress may need urgent intervention after birth.

Immediate Assessment of the Neonate

A rapid assessment must be made to determine the extent of stepwise treatment that is required. This determination is made on the basis of respirations, heart rate, and color. The steps in resuscitation are: 1. Clearing the airway, positioning, stimulating 2. Ventilation 3. Chest compressions 4. Medications or volume expansion

Procedures for Neonatal Resuscitation

These are the latest recommendations (ILCOR 2005) with some background information: 1. Assess respirations. If the infant is breathing but cyanotic, give oxygen; if cyanosis persists, then ventilate. If the infant has apnea or HR less than 100, then ventilate. 2. Assess heart rate. If heart rate is less than 60 bpm, continue to ventilate and initiate chest compressions. Reassess heart rate; if heart rate remains less than 60 bpm, despite effective ventilation and compressions, then: 630 Appendix B: Cardiopulmonary Resuscitation…

Administer epinephrine (10–30 μg/kg IV). Augment blood volume (10 mL/kg isotonic crystalloid).

Some Notes

If bag-mask ventilation is unsuccessful in achieving good ventilation, intubate the trachea immediately. (In some instances, a laryngeal mask airway may be useful as an alternative.)

Initial Resuscitation: Oxygen vs. Room Air

Whether to use supplementary oxygen or air to ventilate the lungs in the neonate is controversial. The use of oxygen has been associated with potentially adverse pulmo- nary and cerebrovascular effects and may result in tissue damage from oxygen-free radicals. Oxygen use during neonatal resuscitation has been linked to the develop- ment of childhood cancer. The results of animal studies are confl icting. However, human studies suggest that the results of resuscitation with room air are equal to or better than those when oxygen is used. Hence current recommendations are: 1. If respiratory efforts are absent or inadequate, priority should be given to lung infl ation/ventilation using room air. 2. If the heart rate remains low after ventilation is established, priority should be given to support cardiac output with chest compressions. 3. Supplementary oxygen should be considered for infants with continuing

central cyanosis. Monitor SpO2 to prevent hypoxia and hyperoxia. 4. Excessive oxygen may cause damage and should be avoided, especially in the preterm. 5. Avoid overventilation and hypocapnia.

Ventilation Strategies Initial breaths of the neonate establish the functional residual capacity (FRC). The peak pressures and infl ation time required to initiate ventilation and establish the FRC in the apneic neonate have not been determined. Pressures varying from 30 to

60 cm H 2O and ventilation rates of 30–60 per minute have been used successfully in reported series. Preterm lungs may be damaged by overinfl ation. Continuous posi- tive airway pressure (CPAP) may help stabilize and improve lung function in sick neonates and may be useful in preterm infants, decreasing the need for intubation. Current recommendations are: 1. Establishing effective ventilation is the primary objective. 2. If bradycardia is present, an increase in heart rate is the primary index of adequate ventilation. Appendix B: Cardiopulmonary Resuscitation… 631

3. Chest wall movement should be assessed if heart rate does not increase. 4. If airway pressure is being monitored, an initial infl ation pressure of 20 cm

H2 O may be effective but some full-term infants may require 40 cm H 2O . 5. For preterm infants, prevent excessive distention of the lungs as evidenced by

chest wall movement. Initial infl ation pressures of 20–25 cm H 2O are usually adequate. Self-infl ating bags, fl ow-infl ating bags, or T-piece systems can be used. The LMA is not recommended as a primary airway device but may be useful if intu- bation is unsuccessful or “not feasible.” No recommendations are made regard- ing the use of CPAP in resuscitation.

Chest Compressions Chest compressions are indicated for a heart rate less than 60 bpm despite ade- quate ventilation with supplementary oxygen for 30 s. 1. Compressions should be delivered at a rate of 100 per minute over the lower third of the sternum, preferably using the chest encircling/thumbs compress- ing technique. 2. Ventilation to compression ratio should be 1:3, coordinated to prevent com- pression during an inspiratory phase of ventilation. 3. Check the heart rate every 30 s (stethoscope). 4. Compressions should continue until the spontaneous heart rate equals 60 bpm.

Medications These are rarely indicated in neonatal resuscitation, and there is a lack of data regarding the value of drugs in improving outcomes. High doses of epinephrine may reduce survival rates and increase neurologic damage. Naloxone, if given to an infant of opioid addicted mother, may cause seizures: 1. Epinephrine may be indicated if the heart rate remains less than 60 bpm despite adequate ventilation and cardiac compressions. The IV route is rec- ommended (0.01–0.03 mg/kg); high doses are not recommended. The intra- tracheal route is not recommended. 2. Naloxone is not recommended for initial neonatal resuscitation. Depressed ventilation should be treated with bag and mask. 3. Sodium bicarbonate is not recommended.

Volume Expansion Volume expansion is indicated for infants when blood loss is suspected, if the infant appears pale with a weak pulse and has not responded fully to other measures: 632 Appendix B: Cardiopulmonary Resuscitation…

4. Isotonic crystalloid solution, 10 mL/kg, is recommended and may need to be repeated. 5. Caution: In preterm infants, large volumes rapidly infused have been associ- ated with intraventricular hemorrhage.

Meconium Intrapartum suctioning has not been demonstrated to reduce the incidence of meconium aspiration syndrome and is no longer recommended. Tracheal suc- tioning should be performed on meconium-stained depressed infants before stimulation. Meconium stained vigorous infants do not require suctioning.

Post-resuscitation Care

1. Temperature control; hyperthermia is bad and increases the risk of mortality and morbidity. Selective head cooling may decrease the incidence of cerebral morbidity following encephalopathy. 2. Check blood glucose level and treat hypoglycemia.

The Preterm Infant

Some special considerations are necessary for the very small infant: 1. Special care must be taken to prevent heat losses; immediately dry and place the infant on a warm mattress under a heating lamp. Use humidifi ed oxygen.

2. Infants weighing greater than 1000 g should be given O 2 , suctioned, and stimulated. 3. Infants weighing less than 1000 g are very likely to require early intubation and ventilation. Be prepared to intervene rapidly unless the infant obviously is in satisfactory condition. 4. Any preterm infant displaying respiratory diffi culty should be intubated to provide for optimal ventilation and oxygenation. References Finan E, Aylward D, Aziz K. Neonatal resuscitation guidelines update: a case-based review. Paediatr Child Health. 2011;16(5):289–94. Kattwinkel J, Perlman JM, Aziz K, et al. Part 15: Neonatal Resuscitation: 2010 American heart association guidelines for cardiopulmonary resuscitation and emergency car- diovascular care. Circulation. 2010;122:S909–19. O’Donnell CP, Gibson AT, David PG. Pinching, electrocution, ravens’ beaks, and positive pressure ventilation: a brief history of neonatal resuscitation. Arch Dis Child Fetal Neonatal Ed. 2006;91:F369–73. APPENDIX C Drug Doses

PREOPERATIVE PERIOD

N.B. Avoid giving drugs intramuscularly (IM) if possible. IM injections are painful and children do not like them. If IM drugs are necessary and more than one has to be given, combine them in the same syringe whenever possible.

Drugs for Premedication

Anticholinergics Atropine: IV 0.02 mg/kg at induction (maximum dose, 0.6 mg); IM 0.02 mg/kg 30–60 min preoperatively (maximum, 0.6 mg). PO same dose, 60–90 min preoperatively. Glycopyrrolate: 0.01 mg/kg IV or IM.

Sedatives Midazolam (Versed): 0.5–0.75 mg/kg PO, 0.2 mg/kg intranasally, 1.0 mg/kg PR, 0.1 mg/kg IM, or 0.05–0.1 mg/kg IV (in a monitored area). Clonidine: 4 μg/kg oral, or 1–2 μg/kg intranasal. Dexmedetomidine: 2.5 μg/kg oral or 1 μg/kg intranasal. (N.B. No data on neuro- toxicity for intranasal injection.) Lorazepam (Ativan): for adolescents, 1–2 mg PO. Midazolam/ketamine mixture: 0.3–0.5 mg/kg midazolam combined with 2–6 mg/kg ketamine plus 0.02 mg/kg atropine PO (This combination may result in considerable sedation—use in a monitored setting).

Antacids: H 2 -Histamine blocking agents Cimetidine: 10 mg/kg PO, or 30 mg/kg PR, or 5 mg/kg IV. Ranitidine: 2–5 mg/kg PO, or 1.5 mg/kg IV or IM. Sodium citrate: 0.4 mL/kg PO.

© Springer International Publishing Switzerland 2016 633 J. Lerman et al., Manual of Pediatric Anesthesia, DOI 10.1007/978-3-319-30684-1 634 Appendix C: Drug Doses

Drugs to Speed Gastric Emptying

Metoclopramide: 0.15 mg/kg IV. ( Note : Atropine blocks the effect of metoclo- pramide and should be withheld until induction of anesthesia.)

Topical Local Anesthetics

Eutectic mixture of local anesthetics ( EMLA): prilocaine (2.5 %) and lidocaine (2.5 %). Apply to skin 60–90 min before procedure. Cover with occlusive dressing. Caution: metabolism of prilocaine may result in methemoglobin- emia in neonates—limit application to 1 g EMLA cream over 10 cm2 skin. Amethocaine gel (Ametop ): Tetracaine (4 %). Apply to skin 45 min before proce- dure. Cover with occlusive dressing. Caution: not recommended for infants <1 month. Ela- Max ( 4 % Lidocaine ): Apply to skin 30 min before procedure. Cover with occlusive dressing. S- Caine Patch : Eutectic mixture of 70 mg lidocaine and 70 mg tetracaine in each patch. Apply 20 min before procedure. Cover with occlusive dressing.

INTRAOPERATIVE PERIOD

Induction Agents

Thiopental sodium ( Pentothal): neonates (younger than 1 month), up to 3–4 mg/ kg; infants (1 month–1 year), up to 7–8 mg/kg; children, up to 5–6 mg/kg. Etomidate : 0.25–0.3 mg/kg. Methohexital: up to 2 mg/kg IV or 15–25 mg/kg of a 1 % or 20–30 mg/kg of a 10 % solution PR. Propofol ( Diprivan ): infants 1.5–2 mg/kg, children 2.5–3.5 mg/kg. Ketamine : 2 mg/kg IV or 4–8 mg/kg IM (plus atropine 0.02 mg/kg IM/IV).

Drugs for Intubation

Succinylcholine : infants, 2 mg/kg IV; older children, 1 mg/kg IV or 4–5 mg/kg IM. Rocuronium : 0.3–1.2 mg/kg IV. ( N.B. Large doses of rocuronium in infants have a rapid onset but may result in prolonged blockade). Vecuronium : 0.1 mg/kg IV. Appendix C: Drug Doses 635

( N.B.: Do not inject rocuronium or vecuronium immediately after thiopental; thiopental precipitates and may occlude IV). Cis -atracurium: 0.1–0.2 mg/kg IV. Pancuronium : 0.1 mg/kg IV. Topical lidocaine for laryngeal spray: maximum dose 4 mg/kg.

Maintenance

Fentanyl : bolus doses 1–2 μg/kg IV prn. IV infusion for major surgery: loading dose 5 μg/kg, infuse at 2–4 μg/kg/h. Hydromorphone : 0.01–0.02 mg/kg IV. Morphine: 10–100 μg/kg IV or intravenous infusion (for children older than 5 years of age); loading dose 100 μg/kg over 5 min, infusion at 40–60 μg/ kg/h. Remifentanil : Loading dose—0.5 to 2 μg/kg IV. IV Infusion—0.05–0.3 μg/kg/min. Acetaminophen: 30–40 mg/kg single-dose PR followed by 20 mg/kg q6h or 10–15 mg/kg PO q4–6 h (maximum daily dose 90–100 mg/kg); 7.5 mg/kg q6h IV for neonates and infants <10 kg or 10 mg/kg q6h IV for children >2 years (maximum daily dose 75 mg/kg)

Neuromuscular Blocking Drugs

1. Usual route of administration IV; IM succinylcholine only in emergency situation. 2. Give initial and repeat doses preferably as indicated by nerve stimulator, especially in infants (whose response to these drugs is extremely variable). 3. Remember that potent inhalational anesthetics (especially sevofl urane and isofl urane) reduce the dose requirement of non-depolarizing drugs. 4. Infusion rates are given as a guide only and should be modifi ed as indicated by neuromuscular blockade monitoring. Cis -atracurium: initial dose 0.1 mg/kg IV, repeat dose 0.03 mg/kg. Infusion: loading dose 0.1 mg/kg, infusion at 2–3 μg/kg/min. Pancuronium : initial dose 0.06–0.1 mg/kg IV; repeat doses should not exceed one sixth of the initial dose. Rocuronium : initial dose 0.3–1.2 mg/kg IV/IM, incremental doses 0.15 mg/kg. Infusion: rate: 10–12 μg/kg/min. Vecuronium : loading dose 0.1 mg/kg, incremental doses 0.02 mg/kg. Infusion rate 0.1 mg/kg/h. 636 Appendix C: Drug Doses

Antagonism of Neuromuscular Blockade

Atropine 0.02 mg/kg or glycopyrrolate 0.01 mg/kg mixed with neostigmine 0.05 mg/kg-administer IV slowly; use a nerve stimulator to monitor effect; OR Atropine 0.02 mg/kg, followed by edrophonium 1 mg/kg IV.

POSTOPERATIVE PERIOD

Analgesics

Acetaminophen ( Tylenol ): 10–20 mg/kg q4–6 h PO or 30–40 mg/kg dose PR followed by 20 mg/kg q6h (maximum daily dose 90–100 mg/kg); 10 mg/ kg q6h IV (children >2 years) (maximum daily dose <75 mg/ kg ) Acetaminophen with codeine elixir: each 5 mL contains 120 mg acetamino- phen + 12 mg codeine [ N.B . The FDA issued a box warning regarding the use of codeine in children with obstructive sleep apnea after tonsillectomy because of deaths in ultra- rapid metabolizers. Codeine is no longer recommended for use in any child] Volume of elixir to administer should be equivalent to 1 mg/ kg codeine PO q4 h Codeine (useful for minor surgery): 1–1.5 mg/kg IM. (Note : Codeine must not be given intravenously). [ N.B . Codeine is no longer recommended for use in children] Diclofenac : 1 mg/kg PO or PR. Hydromorphone ( Dilaudid ): Bolus dose: 0.01–0.02 mg/kg IV q3 to 4 h. Continuous infusion rates: 3–5 μg/kg/h. Caudal analgesia: 10 μg/kg. Ibuprofen: 10 mg/ kg PO. Ketorolac: 0.5 – 1.0 mg/ kg IV (maximum 15 mg for children <50 kg and 30 mg for >50 kg). Morphine : children, 0.05–0.1 mg/kg; infants, 0.05 mg/kg IM or IV. Morphine infusion: children, 10–30 μg/kg/h. To prepare a solution mix: [0.5 × the child’s weight (kg)] mg morphine in 50 mL saline. The solution then contains 10 μg/kg/mL. Infuse at 1–3 mL/h for postoperative analgesia (equivalent to 10–30 μg/kg/h). For infants, give 5–15 μg/kg/h (i.e., 0.5–1.5 mL/h). Caudal or epidural morphine: 30 μg/kg single dose. Spinal morphine (preservative free): 10 μg/kg single shot. Nalbuphine : 0.1–0.2 mg/kg IV (Max 5 mg/kg) IV Infusion 0.1–0.2 mg/kg/h. Oxycodone : 0.1–0.2 mg/kg PO q4h. Tramadol: 1.0–2.0 mg/kg q6h PO. (>4 years) Appendix C: Drug Doses 637

Opioid Antagonist

Naloxone ( Narcan): 0.5–2 μg/kg IV or IM. This drug should be titrated slowly until undesired opioid effects are reversed. Rapid administration of an exces- sive dose results in loss of analgesia, pain, and extreme restlessness. The same dose of naloxone (μg IV) that resulted in desired effect should then be given IM to prevent recrudescence.

Prophylaxis Against PONV

First-line medications Dexamethasone : 0.0625–0.15 mg/kg IV (maximum 8 mg). Ondansetron : 0.05–0.15 mg/kg IV. Metoclopramide : 0.15 mg/kg IV. Second-line medications Dimenhydrinate ( Gravol, Dramamine ): 1 mg/kg IV or 2 mg/kg PR.

Ancillary Drugs

Single IV antibiotic doses are listed below (maximum daily dose is shown in parentheses). The smaller dose should be given to neonates less than 1 week of age (limited neonatal liver and renal function). Antibiotics should be infused over several minutes only (i.e., never give antibiotics as IV boluses!) to minimize the possibility of adverse reactions. Some must be given over a greater period (e.g., vancomycin). Regimens for antibiotic prophylaxis against subacute bacte- rial endocarditis are listed on page 447. Ampicillin2 : 25–100 mg/kg (300 mg/kg). Cefazolin : 20–40 mg/kg (100 mg/kg). Cefoxitin : 20–40 mg/kg (160 mg/kg). Cefuroxime : 20–50 mg/kg (240 mg/kg). Clindamycin : 5–10 mg/kg (30 mg/kg). Cloxacillin : 12–25 mg/kg (100 mg/kg). Erythromycin : 2.5–5 mg/kg (20 mg/kg). Gentamicin : 2.0 mg/kg (7.5 mg/kg). Benzyl penicillin (see Footnote 2): 30,000–50,000 IU/kg (250,000 IU/kg). Vancomycin (see Footnote 2): 10 mg/kg (60 mg/kg) (must be given over a period of at least 1 h).

2 Use caution in children with renal failure. 638 Appendix C: Drug Doses

Adrenocorticosteroids Dexamethasone ( Decadron ): 0.2–0.5 mg/kg IV (maximum, 10 mg). Methylprednisolone ( Solu- Medrol ): 5–25 mg/kg IV slowly over 10 min. Hydrocortisone sodium succinate ( Solu- Cortef ): 1–5 mg/kg IV over 8–10 min.

Cardiovascular Drugs (titrate infusions to effect) (see below for infusion setup) Adenosine: 100 μg/kg as a rapid IV bolus. Repeat up to maximum dose 0.3 mg/ kg or 12 mg. Amiodarone : loading dose 5 mg/kg (over 30–60 min) IV. Amrinone: loading dose 0.75 mg/kg IV; infusion 3–5 mg/kg/min in neonates and 5–10 mg/kg/min in children. Calcium chloride : 5–15 mg/kg IV. Calcium gluconate : 10–30 mg/kg IV. Dopamine: 5–20 μg/kg/min infusion. Dobutamine: 5–20 μg/kg/min infusion. Epinephrine: 0.1–1 μg/kg/min infusion. Esmolol: 100–500 μg/kg IV, 50–100 μg/kg/min infusion. Hydralazine : 0.1–0.2 mg/kg IM or IV. Isoproterenol ( Isuprel): 0.025–0.1 μg/kg/min infusion. Lidocaine : 1–2 mg/kg IV. Milrinone: 50–100 μg/kg loading dose over 15–30 min (reduce loading dose if hypotension occurs) followed by 0.5–1.0 μg/kg/min infusion. Norepinephrine ( Levophed): 0.1–1 μg/kg/min infusion. Nitroglycerin: 1–10 μg/kg/min infusion. Phenoxybenzamine : loading dose 0.25 mg/kg × 4 over 2–4 h; maintenance, 0.25 mg/kg q6h. Phentolamine : 0.2 mg/kg IV. Phenylephrine: 0.1–1 μg/kg/min infusion. Procainamide : 5–15 mg/kg IV. Propranolol : 0.01–0.1 mg/kg IV over 10 min.

Prostaglandin E1 : 0.05–0.1 μg/kg/min (starting dose). (Maintenance infusion rate, e.g., after PDA open, may be between 0.005 and 0.4 μg/kg/min.) Sodium nitroprusside: 0.5–10 μg/kg/min infusion. Verapamil: 0.1–0.3 mg/kg IV. (Do not give to infants younger than 1 year of age).

Diuretics Ethacrynic acid : 0.5–1 mg/kg IV. Furosemide ( Lasix ): 1 mg/kg IV. Mannitol: 0.5–1.0 g/kg IV (administer over several minutes to prevent transient hypotension). Appendix C: Drug Doses 639

Anticonvulsants Diphenylhydantoin ( Dilantin): loading dose 15–20 mg/kg IV slowly; maintenance, 2.5–5 mg/kg bid IV or PO. Phenobarbital : loading dose 10 mg/kg IV; maintenance, 1.5–2.5 mg/kg bid IV.

Bronchodilators Salbutamol ( Albuterol ): loading dose 5–6 μg/kg IV; infusion 0.1–1.0 μg/kg/min; inhaled aerosol 100 μg dose q6 h (delivery of aerosol through pediatric tra- cheal tubes is only 3–10 %; to deliver a more effective dose, activate the canis- ter (during inspiration); (1) into a spacer, (2) through a catheter inserted part way down the tube, or (3) after inserting it into the barrel of a 60 mL syringe

that is connected to the CO2 port and reinserting the plunger into the barrel). Aminophylline: loading dose 5 mg/kg over 30 min IV; infusion 1 mg/kg/h (if no recent doses). Monitor blood levels (therapeutic range, 10–12 μg/mL).

Local Anesthetics Recommended safe maximum doses Lidocaine plain : 5 mg/kg Lidocaine with epinephrine : 7 mg/kg N.B. The maximum recommended dose of epinephrine to be infi ltrated during halothane anesthesia is 10 μg/kg. Bupivacaine : 2.5 mg/kg Ropivacaine: 2.5 mg/kg

DRUGS INFUSIONS FOR INFANTS AND CHILDREN

These formulas are designed to permit medications to be infused with limited fl uid volumes. (N.B. Weight = the child’s weight in kilograms). Dopamine or dobutamine : Weight × 6 mg of drug in 100 mL; then 1 mL/h = 1 μg/kg/min In the case of neonates and infants <10 kg use: (Weight × 30) mg of drug in 100 mL; then 1 mL/h = 5 μg/kg/min Epinephrine : (Weight × 0.6) mg of drug in 100 mL; then 1 mL/h = 0.1 μg/kg/min Sodium nitroprusside or nitroglycerin : (Weight × 6) mg of drug in 100 mL; then 1 mL/h = 1 μg/kg/min 640 Appendix C: Drug Doses

Isoproterenol : (Weight × 0.15) mg of drug in 100 mL; then 1 mL/h = 0.025 μg/kg/min Prostaglandin : (Weight × 60) μg of drug in 20 mL; then 1 mL/h = 0.05 μg/kg/min

DRUGS TO REDUCE BLEEDING

Desmopressin : May improve platelet function and reduce bleeding in some platelet diseases. Dose: 0.3 μg/kg by slow infusion over 20 min after weaning from cardiopulmonary bypass. Monitor cardiovascular parameters carefully during infusion. ε- Aminocaproic acid (Amicar ): Used to treat fi brinolytic states. May reduce post- operative bleeding, especially in cyanotic children. Should be administered before sternotomy. Loading dose: 40–100 mg/kg (maximum 5 g) diluted and infused slowly over 20–60 min followed by continuous infusion during sur- gery at 10–30 mg/kg/h. Tranexamic acid: a synthetic drug that forms a reversible complex with both plasminogen and plasmin by combining at lysine binding sites inhibits fi bri- nolysis and reduces bleeding. Loading dose 100 mg/kg IV followed by infu- sion during surgery at 10 mg/kg/h. Index

A intravenous therapy (see Intravenous Acetaminophen , 71 , 218 therapy ) Acute abdomen, 372 , 373 laryngeal mask airway (see Laryngeal Acute infectious croup. See mask airway (LMA) ) Laryngotracheobronchitis laryngoscopy , 94–95 , 287 Air-tissue interface , 19 laryngospasm , 91–92 , 212 Airway infections mask anesthesia, 90–91 bacterial tracheitis , 300 monitoring during anesthesia epiglottitis , 294 (see Monitoring, during perioperative management , anesthesia) 296–297 nasotracheal intubation, 97–98 postoperative management , 297 postintubation croup , 92 preoperative management , 295–297 postobstructive pulmonary edema, 92 epinephrine inhalations, 298 preterm infants laryngotracheobronchitis , 297–298 apnea , 133–134 nasotracheal intubation, 298–299 benzyl alcohol , 134 tracheotomy , 299–300 coagulation , 134 Airway management fl uid administration, 134 anesthetic circuits , 111 hypoglycemia and hyperglycemia , 134 anesthetic gases, humidifi cation, 113 infection , 133 blood replacement intraventricular hemorrhage , 133 alternatives to blood transfusion , oxygenation , 134 132–133 temperature control, 134 blood loss, assessment of, 130–131 rapid sequence induction, 98 blood transfusion, 131 tracheal extubation, 109–110 massive blood transfusion, 131 tracheal intubation, 95–97 perioperative management , 130 ventilation preoperative assessment, 128–130 manual , 111 body temperature mechanical , 112–113 body heat conservation , 121–122 Alveolar matrix stability , 15 hyperthermia during surgery , 122 Ambulatory surgery . monitoring of , 121 See Outpatient surgery cannulation (see Cannulation ) Anesthesia chart, 121 cricoid pressure , 99–100 Anesthesia management , 332 diffi cult tracheal intubation (see Anesthetic circuits , 111 Diffi cult tracheal intubation ) Anesthetic gases, humidifi cation of, 113

© Springer International Publishing Switzerland 2016 641 J. Lerman et al., Manual of Pediatric Anesthesia, DOI 10.1007/978-3-319-30684-1 642 Index

Aneurysm of vein of Galen Beckwith–Wiedemann syndrome, 351 anesthetic management, 249–250 Benzyl alcohol , 134 special anesthesia problems, 249 Bezold–Jarisch refl ex, 332 Anterior mediastinal masses (AMM), Biliary atresia 362–364 anesthesia , 352 , 353 Antibiotic prophylaxis , 305 , 308 , 309 jaundice , 352 Anticholinergic drug, 104 Blalock-Taussig procedure, 422 Anticholinesterase therapy , 365 Blood cardioplegia, 396 Antidiuretic hormone (ADH), 127 Blood conservation, 132 Antihypertensive polypharmacy, 473 Blood loss assessment, 130–131 Aortic coarctation (CoA) Blood pressure (BP) , 27 , 115 cardiotonic drugs, 420 Blood replacement classifi cation, 418 alternatives to blood transfusion , coarctation, aorta, 419 132–133 prostaglandin , 420 blood loss, assessment of , Aortic stenosis, 430 130–131 Apnea , 17 , 133–134 blood transfusion, 131 Appendicitis , 372 massive blood transfusion, 131 Arnold-Chiari malformation perioperative management , 130 anesthesia management , 245 preoperative assessment, 128–130 associated conditions , 244 Blood transfusion, 131–133 surgical procedure, 245 Body heat conservation , 121–122 Arterial catheter , 115 Body temperature Arterial lines, 118 body heat conservation , 121–122 Asthma hyperthermia during surgery , 122 anesthetic considerations, 169–171 monitoring of , 121 perioperative anesthesia Bradycardia , 87 management , 173 Brain death determination, 374 , 375 postperative anesthesia Bronchial injury , 497 management , 171 Bronchoscopy , 291–292 preoperative anesthesia Bupivacaine management , 170 disadvantage , 143 Atelectasis , 411 peripheral and epidural Atrial septal defect (ASD), 425–426 blocks , 143 Atrioventricular (AV) canal, 428 toxicity , 144 Atropine , 87 Burn injury Atypical plasma cholinesterases, 190 airway management and fl uid Autotransfusion , 490 resuscitation, 500 Awake ear surgery , 285 anesthesia management Awake intubation, 10 , 97 perioperative , 502–504 postoperative , 504 preoperative , 501–502 B anesthesia problems , 500–501 Bacterial tracheitis, 300 debridement and grafting , 500 Balloon atrial septostomy (BAS), 429 fasciotomy/escharotomy , 500 Becker muscular dystrophy , 186 systemic effects, 498–500 Index 643

C blood volume, 27 Cannulation bradycardia , 24 external jugular vein, 119–120 circulatory changes at birth , 24 femoral artery, 117–118 fetal circulation, 22 internal jugular vein , 118–119 hypoxemia , 28 radial artery, 116–117 myocardium , 24 venous , 124 oxygen transport , 28 , 29 Cardiac catheterization, 439–440 pulmonary circulation, 26 Cardiologic procedures SNP infusion, 412 analgesics , 394 systolic and diastolic pressures , 24 anemia , 395 transitional circulation, 24 anesthetic drugs , 395 treatment , 411 anesthetic management vasodilating effect , 412 fentanyl , 400 ventricular dysfunction , 412 hematocrit , 401 Cardioversion , 442 , 443 induction methods , 400 Celecoxib , 220 inotropic drugs, 399 Central venous pressure (CVP) , 116 , muscle relaxant , 401 486 myocardial function , 401 Cerebral arteriography, 516 nasopharyngeal and rectal/bladder Cerebral blood fl ow (CBF), 10 , 11 , thermometer , 401 228–230 tracheal intubation, 400 Cerebral metabolic rate for oxygen

blood supplies, 398 , 399 (CMRO 2) , 229 CPB , 396 Cerebral palsy (CP) diastole and diastolic pressure , 394 anesthesia management , 189–190 heparin , 395 postoperative care, 190 inhalational agents, 395 special anesthesia problems, 188–189 myocardial function , 394 Cervical spine injury , 485 , 493 PAH , 394 C F. See Cystic fi brosis (CF) pathophysiologic implications , Chalazion excision surgery, 264 392–394 2-chloroprocaine , 143 premedication , 398 3-chloroprocaine , 143 preoperative assessment and Choanal atresia preparation , 396–398 CHARGE association, 272 PVR/SVR , 395 perioperative anesthesia management , temperature control, 395 272–273 vasoconstriction , 395 postoperative anesthesia management, ventilatory efforts , 395 273 Cardiology, interventional techniques , preoperative anesthesia management , 440–442 272 Cardiomyopathy , 435 primary problem, 272 Cardiopulmonary resuscitation , 623 surgical procedures, 272 Cardiorespiratory syndrome , 277 Circle absorber system , 111 Cardiovascular system Circumcision , 383 anesthesia , 411 Cis-atracurium , 69 644 Index

Cleft lip and cleft palate (CLP) propofol (see Propofol (2, alveolar bone grafting, 317 6-diisopropylphenol)) anesthesia management , 314–316 thiopental , 57 fractured mandible metabolism and elimination anesthesia management , 318–319 of drugs , 41 special anesthesia problems, neuroleptics , 59–60 318–319 neuromuscular blocking drugs , 65 surgical procedures, 318 nonsteroidal anti-infl ammatory drugs interdental wiring removal , 319 acetaminophen , 71–72 palatal splints, 316 diclofenac , 72 pharyngoplasty , 317 ibuprofen , 72 special anesthesia problems, 312 ketorolac , 72 surgical management , 312–316 tramadol , 73 Clinical pharmocology opioid antagonists, 65 alpha-2 agonist drugs , 58 , 59 opioid medications antagonism, neuromuscular blockade, alfentanil , 65 70 , 71 codeine , 61 atracurium , 69 fentanyl , 62 benzodiazepine antagonists, 58 hydromorphone , 64 cis-atracurium , 69 methadone , 64 distribution of administered drugs , morphine , 60 40–41 remifentanil , 63 inhaled agents, 44 , 49 sufentanil , 64 CBF , 43 prolonged duration relaxants , 69–70 depressed ventilation, 42 rocuronium , 68 desfl urane, 47 routes of administration halothane (see Halothane ) intralingual , 39 helium , 52 intramuscular , 39 hypoxic pulmonary intranasal , 40 vasoconstriction , 43 intraosseous , 40 increased alveolar ventilation , 41 intratracheal , 40 isofl urane, 48 intravenous , 39 MAC , 42 oral route, 40 malignant hyperthermia, 43 rectal , 40 myocardial depression , 42 succinylcholine nitric oxide, 51 intraocular pressure , 67 nitrous oxide, 44 IV and IM, 66 NMBDs , 43 metabloization , 66 oxygen , 51 myoglobinemia and sevofl urane (see Sevofl urane) myoglobinuria, 66 intravenous agents vecuronium , 68 etomidate , 57 Clonidine , 58 , 89 , 145 ketamine , 55 , 56 Closed limb fractures , 453 , 454 methohexital , 57 Coagulation , 134 midazolam , 52 , 53 Codeine , 61 , 220 Index 645

Compartment syndrome , 452 Craniopharyngioma , 248–249 Computed tomography (CT), 509 Craniosynostosis Congenital cysts , 288 anesthetic management, 241–242 Congenital diaphragmatic hernia associated conditions , 241 abdominal procedure , 338 surgical procedure, 241 aggressive invasive monitoring, 339 Craniotomy for tumors, 245–248 anesthesia problems , 338 Cricoid pressure , 99–100 diagnosis , 337 Cyanosis , 391 incidence , 337 Cystic fi brosis (CF) perioperative , 339–340 perioperative anesthesia management , preoperative management , 338 173 radiographic appearance, 337 postoperative anesthesia management, Congenital heart disease (CHD), 175 , 173–174 308–310 preoperative anesthesia management, 173 antibiotic routine, patients, 447 special anesthesia problems, 172 diagnosis , 389 , 390 Cystic hygroma, 326 in neonate, 389 Cystoscopy , 469 incidence , 389 , 390 myocardial ischemia, 391 obstructive lesions , 391 D pulmonary function, 390 Dantrolene , 181 respiratory system , 390–391 1-deamino-8-D-arginine vasopressin systemic effects, 390 (DDAVP), 197 , 198 Congenital hypertrophic pyloric stenosis Deep hypothermic circulatory arrest anesthetic considerations, 347–349 (DHCA) gastric outlet obstruction , 347 acid-base balance, 410 pyloromyotomy , 347 alpha stat approach, 410 Congenital laryngotracheoesophageal anesthesia management , 409 cleft circulatory arrest , 410 tracheostomy and gastrostomy, 346 postoperative metabolic alkalosis , 410 type 1 and high tracheal lesions , Dental procedures 346–347 anesthetic principle, 305–306 type 3 and 4 clefts, 347 congenital heart disease, 308–310 types , 346 deep sedation, 308 Congenital lobar emphysema perioperative anesthesia management , abnormal distention , 335 306–307 anesthesia management, 335–337 postoperative anesthesia management, anesthesia problems , 335 307–308 older children, anesthesia preoperative anesthesia management , management , 337 306 surgical procedure, 335 Desfl urane, 47 Congenital webs , 288 Dexamethasone , 110 Congestive heart failure, 473 Dexmedetomidine , 59 , 89 Contrast media, 517 Diabetes mellitus Controlled hypotensive anesthesia , 235 insulin pump, 201 Corticosteroids , 110 ketoacidosis , 201–202 646 Index

Diabetes mellitus ( cont. ) Electroretinography (ERG), 268 postoperative anesthesia management, Emergence delirium , 213 200 Emery–Dreifuss muscular dystrophy , 186 preoperative anesthesia management , Encephalocele 199–200 anesthesia management , 243–244 Diclofenac , 72 , 219 associated conditions , 243 Diffi cult tracheal intubation special anesthesia problems, 243 extubation , 110 surgical procedures, 243 management of Endocarditis prophylaxis , 305 , 306 , 309 anesthetized vs . awake/sedated Endoscopy intubation, 104 anesthesia management , 286 diffi cult airway algorithm , 104 bronchoscopy , 291–292 failed intubation, 109 fl exible bronchoscopy, 293 fl exible bronchoscopy, 108 laryngoscopy (see Laryngoscopy ) GlideScope , 107 laser surgery, 290 laryngeal mask airway, 107 procedures , 286 light wand intubation, 108–109 rigid esophagoscopy, 293–294 retrograde intubation, 109 special anesthesia problems, 286 standard , 104–107 End-tidal carbon dioxide, 115 preoperative assessment, 103–104 Epidural , 223 DiGeorge syndrome, 422 Epiglottitis Digitalis therapy, 397 , 473 perioperative management , 296–297 Dilaudid , 64 postoperative management , 297 Dinamap , 115 preoperative management , Direct laryngoscopy , 94 , 104 295–297 Disseminated intravascular coagulation Epinephrine , 145 (DIC), 489 Epinephrine inhalations, 298 Diuretic therapy, 397 , 411 , 473 ERG . See Electroretinography (ERG) Down syndrome (DS), 87 , 116 Esophageal atresia (EA) . See associated conditions , 175–176 Tracheoesophageal fi stula (TEF) special anesthesia problems, 176 External jugular vein cannulation , Droperidol , 59 119–120 Duchenne muscular dystrophy Ex utero intrapartum treatment (EXIT) (DMD), 453 procedure, 328 anesthetic management, 186 Eye surgery pathophysiology , 186 anesthetic principles, 257–260 Ductal-dependent defects, 393 chalazion excision surgery, 264 ERG , 268 glaucoma surgery E perioperative anesthesia Electrical burns , 501 management , 263 Electrocardiogram , 115 postperative anesthesia Electrocorticography management , 263 anesthesia management , 250–251 preoperative anesthesia special anesthesia problems, 250–251 management , 263 Electrophysiologic studies, 442 Index 647

special anesthesia problems, 262 surgical procedures, 318 nasolacrimal duct surgery , 264 Functional endoscopic sinus surgery penetrating eye trauma (FESS), 274–275 perioperative anesthesia Functional residual capacity (FRC), 18 management , 265 postperative anesthesia management , 265 G preoperative anesthesia Ganglioneuroma , 357–359 management , 265 Gases, humidifi cation, 113 special anesthesia problems , 264–265 Gastroesophageal refl ux disease radiotherapy , 267 (GERD), 188 retinoblastoma , 266 Gastroschisis ROP , 265–266 abdominal defect , 349 strabismus correction anesthesia management , 350 , 351 associated conditions , 260 congenital defects , 349 perioperative anesthesia hypoglycemia , 350 management , 261–262 surgical procedure, 350 postoperative anesthesia Gastrostomy (PEG) , 520 management , 262 General and thoraco-abdominal surgery preoperative anesthesia anesthetic agents, 334 management , 261 bronchial blocker, 333 special anesthesia problems, carbon dioxide embolism, 332 260–261 cephalad movement, diaphragm and VEPs , 267–268 lungs , 332 heart rate, 332 in infants and children, 331–334 F OLV , 334 Failed intubation, 109 physiologic consideration , 333 Fasting, preoperative, 77 , 78 pyloromyotomy , 332 Fat mass (FM), 177 Univent tube, 334 Feeding orders, preoperative , 77 General endotracheal anesthesia , Femoral artery cannulation, 117–118 320–322 Femur fractures , 454 Glasgow coma scale, 490–493 FESS . See Functional endoscopic sinus Glaucoma surgery surgery (FESS) perioperative anesthesia management , Fiberoptic intubation awake, 461 263 Flexible bronchoscopy, 108 , 293 postperative anesthesia management , Fluid and electrolyte therapy, 412 263 Fluid management , 126–128 preoperative anesthesia management , Flumazenil , 58 263 Fontan procedure, 432 , 433 special anesthesia problems, 262 Forearm fractures , 454 GlideScope , 107 Fractured mandible Glomerular fi ltration rate (GFR), 33 , 41 anesthesia management , 318–319 Glycopyrrolate , 86 special anesthesia problems, 318–319 Gunshot wounds, 505 648 Index

H preoperative anesthesia management , Halothane 238 hypotension prevention , 50 surgical procedures"prochydro , 238 inexpensive , 49 Hydrocodone , 62 , 220 intercostal muscle activity, 49 Hydromorphone , 64 , 220 MAC values , 50 Hyoscine , 86 metabolization , 50 Hyperbilirubinemia , 32 myocardial contractility and heart rate Hyperglycemia , 30 , 134 depression, 49 Hypermagnesemia , 31 vs. sevofl urane, 49 Hyperthermia , 122 HeadFIX ® Immobilization Device, 267 Hyperventilation , 112 Head injury, 490 Hypocalcemia , 31 Heart–lung transplantation, 437–438 Hypoglycemia , 30 , 120 , 134 Heart transplantation, 205–206 , 435 , 436 Hypomagnesemia , 31 Heat and moisture exchanger (HME) , 113 Hyponatremia , 128 Hematologic disorders Hypoplastic left heart syndrome (HLHS) , anemia , 191–193 431 , 432 hemophilia (see Hemophilia ) Hypospadias repair , 469 , 470 SCD (see Sickle cell disease (SCD)) Hypotension , 10 Hemoglobin electrophoresis, 194 Hypoxia , 16 Hemophilia factor VIII defi ciency (Type A) perioperative anesthesia I management , 197 Ibuprofen , 72 , 219 postoperative anesthesia Ideal body weight (IBW) , 178 management , 197 Informed consent , 84–85 preoperative anesthesia Infusion pump, 122 management , 197 Inguinal hernia, 381 , 382 surgical management , 196 Inhaled agents, 41 factor IX defi ciency (Type B), 198 Inhaled anesthetics agents Hepatic and renal function , 392 CBF , 43 Hepatic blood fl o w, 4 1 depressed ventilation, 42 Hepatic portoenterostomy , 352 desfl urane, 47 Herbal preparations, 78 , 84 halothane (see Halothane ) Hering-Breuer infl ation refl e x, 1 6 helium , 52 High frequency oscillatory ventilation hypoxic pulmonary vasoconstriction, 43 (HFOV), 112 isofl urane, 48 Hip arthrograms , 453 MAC , 42

Histamine 2 -blocking drugs, 104 malignant hyperthermia, 43 Humidifi cation, anesthetic gases , 113 myocardial depression , 42 Hydrocephalus nitric oxide, 51 perioperative anesthesia management , nitrous oxide, 44 239 NMBDs , 43 postoperative anesthesia management, oxygen , 51 239 sevofl urane (see Sevofl urane) Index 649

Intensive care unit (ICU), 413 J Intermittent positive-pressure ventilation Jugular vein cannulation (IPPV), 395 external , 119–120 Internal jugular vein cannulation, internal , 118–119 118–119 Juvenile diabetes , 199 Interrupted aortic arch, 422 Interventional cardiology, 440 Intestinal malrotation, 352 K Intestinal obstruction, neonate , Kasai procedure, 352 353 , 354 Ketamine , 89 Intracerebral tumors , 245 Ketorolac , 73 , 219 Intracranial physiology Kyphoscoliosis , 455 , 456 anesthetic drug effects , 228–230 and pathophysiology , 227–228 L Intracranial pressure (ICP), 10 , 227–229 , Laboratory tests, preoperative , 86 231 , 232 , 234–235 , 248 , 254 Laparoscopic surgery, 331 Intracranial surgery principles , 227–230 Laryngeal mask airway (LMA) Intraocular pressure (IOP), 257 , diffi cult airway, 107 259 , 262 insertion methods, 100 Intraocular surgery . See Glaucoma surgery positioning , 100 Intraosseous infusions, 125 ProSeal LMA, 100 , 102 Intrathoracic procedures, 333 size selection and cuff volumes, 100 Intravenous therapy uses , 102 blood replacement , 235 Laryngeal papillomas, 289 controlled hypotensive techniques , Laryngomalacia , 288 235–236 Laryngoscopy , 94–95 , 287 general rules , 234 Laryngospasm , 91–93 , 212 ICP control, 234–235 Laryngotracheobronchitis , 297–298 intraoperative fl uid management , Laser surgery, 290 126–128 Latex allergy, 174–175 intraosseous infusions, 125 Levobupivacaine , 143 preoperative fl uid replacement Lidocaine , 142 dehydration , 125 Light wand intubation, 108–109 extracellular water and Liver transplantation, 206 , 376–380 sodium, 126 Local anesthetics overt/impending shock, amino amides, 141 treatment of , 126 amino esters , 141 potassium , 126 bupivacaine , 143 venipuncture , 123 caudal block venous cannulation, 124 awake single-shot caudal Intraventricular hemorrhage (IVH) , analgesia, 152 10 , 133 caudal morphine, 149 Intussusception , 372 concentrations and volumes , 151 Iron defi ciency anemia , 392 continuous caudal analgesia, Ischemic optic neuropathy , 460 152–153 650 Index

Local anesthetics ( cont. ) M landmarks , 149 , 150 Magnetic resonance imaging, 510 lower limb/perineal surgery , 149 Major craniofacial surgery . See Maxillary needle insertion, direction of , 150 osteotomies chloroprocaine , 143 Major otologic procedures clonidine , 145 perioperative anesthesia management , doses of , 142 284–285 epinephrine , 145 postoperative anesthesia management, head, neck and upper limb blocks 285 brachial plexus, 159–161 preoperative anesthesia management , greater occipital nerve block, 158 284 infraorbital nerve block , 158 Malignant diseases superfi cial cervical plexus block , 158 drug adverse effects, 203–204 supraorbital and supratrochlear special anesthesia problems, 202–203 nerve blocks , 158 Malignant hyperthermia (MH) ilioinguinal and iliohypogastric nerve anesthesia induction, 185 block , 155 clinical manifestations , 180 intercostal nerve blocks , 154–155 pathophysiology , 178 intravenous block, 164 preoperative investigation , 183–184 levobupivacaine , 143 preoperative preparation , 184–185 lidocaine , 142 susceptibility detection, 179–180 lower extremity blocks therapeutic regimen , 181–183 ankle block, 163 Mandibular distraction osteogenesis continuous femoral nerve block , 162 (MDO), 325 femoral nerve block, 161 Manual ventilation , 111 lateral femoral cutaneous nerve , 162 Mask anesthesia, 90–91 sciatic nerve block, 162–163 Masseter spasm , 185 lumbar and thoracic epidural blocks , Massive blood transfusion, 131 153–154 Maxillary osteotomies penile block, 156–158 anesthesia management , 323–325 pharmacokinetics , 141–142 principle , 322–325 ropivacaine , 143 special anesthesia problems, 323 rules for, 146 Maximal allowable blood loss (MABL) , 130 spinal anesthesia M D O. See Mandibular distraction awake spinal anesthesia, 147 osteogenesis (MDO) cerebrospinal fl uid, volume of , 147 Mean arterial blood pressure (MAP), 227 contraindications , 148 Mechanical ventilation, 112–113 lumbar puncture, 147 Meckel diverticulum , 372 , 373 perioperative management , 148–149 Meningocele. See Meningomyelocele preoperative management , 148 Meningomyelocele toxicity , 144 anesthesia management , 243–244 Local anesthetic systemic toxicity associated conditions , 243 (LAST), 144 special anesthesia management, 243 Lorazepam , 89 surgical procedures, 243 Lung surgery , 359–361 Methemoglobinemia , 142 Lung transplantation, 206 , 437 , 438 Metoclopramide , 104 Index 651

M H. See Malignant hyperthermia (MH) preoperative anesthesia management , Midazolam , 52 , 88–89 274 Minimum alveolar concentration special anesthesia problems, 273–274 (MAC), 42 surgical procedures, 273 Minor otologic procedures, 282 , 283 Nasotracheal intubation, 97–98 , 298–299 , Mitochondrial myopathy (MM) , 187 305–307 “Modifi ed” Blalock anastomosis , 424 Neck tumors Monitoring anesthesia management , 326–327 anesthesia chart, 121 special anesthesia problems, 326 blood glucose , 120 Neonatal necrotizing enterocolitis (NEC), of body temperature, 121 355 , 356 during anesthesia Neonate arterial catheter , 115 cardiovascular system blood pressure , 115 blood volume, 27 CVP , 116 bradycardia , 24 electrocardiogram , 115 circulatory changes at birth , 23 , 24 end-tidal carbon dioxide, 115 fetal circulation, 22 peripheral nerve stimulator , 115 hypoxemia , 28 pulse oximeter , 113 myocardium , 24 stethoscope , 114 oxygen transport , 28 , 29 thermistor probe , 115 pulmonary circulation, 26 urine output, 116 response to hypovolemia, 27 fl uid administration, 120 systolic and diastolic pressures , 24 Morphine , 60 , 220 transitional circulation, 24 Motor-evoked potentials (MEPs) , 230 CBF , 10 , 11 MRI. See Magnetic resonance imaging central nervous system , 9 , 10 Muscle paralysis , 338 composition and regulation, body Muscular dystrophy . See Duchenne fl uids muscular dystrophy (DMD) body water , 32 Myasthenia gravis, 364–367 fl uid losses, 34 Myelodysplasia , 242 maintenance requirements , 34 Myocardial hypertrophy, 393 renal function , 33 Myringotomy and tube (M&T) cranium and intracranial pressure , 10 placement, 282 , 283 CSF , 11 metabolism bilirubin homeostasis , 32 N calcium homeostasis, 31 Naloxone hydrochloride, 65 glucose homeostasis, 29–30 Nasal polyps, 273 magnesium homeostasis, 31 Nasogastric tube (NGT) , 13 nonobstructive hydrocephalus, 12 Nasolacrimal duct surgery , 264 obstructive hydrocephalus , 11 Nasopharyngeal tumors, 273 physiology of temperature perioperative anesthesia management , homeostasis, 35 , 36 274 respiratory system postoperative anesthesia management, anatomic difference, 13 , 14 274 apnea , 17 652 Index

Neonate (cont. ) Oculocardiac refl ex (OCR), 257 biochemical and refl ex Oculogastric refl ex, 260 mechanisms , 16 Omphalocele . See Gastroschisis changes with anesthesia, 13 , 21 , 22 Open-heart surgery Hering-Breuer infl ation antifi brinolytic agents, 403 refl e x, 1 6 calcium infusion , 406 hypoxia , 16 cerebral oximetry, 402 lung growth and development, 21 coagulation indices, 406 lung surfactants , 20 , 21 cold-induced stress, 404 lung volumes, 19 CPAP , 408 muscles of respiration , 17 CPB , 403 periodic breathing, preterm , 16 dopamine infusion , 406 physiology , 14–15 fentanyl , 404 pulmonary function fl uid loading , 402 testing , 21 furosemide , 405 respiratory mechanics, 18 hemodilution , 403 ventilation and perfusion heparin , 403 relationship, 20 hypertension , 404 ventilatory response, hypercapnia, 16 hypoxemia , 408 work of breathing, 20 inotropic agents , 405 R O P, 1 2 inotropic drugs/vasodilators, 409 Neuroanesthesia management low heart rate, 405 induction , 231–232 metabolic acidosis, 405 maintenance , 232 milrinone infusion , 407 monitoring , 232–233 muscle relaxants , 408 premedication , 231 myocardial contractility , 405 ventilation , 232 platelet dysfunction , 406 Neuroapoptosis , 230 pulmonary hypertensive crisis, 407 Neuroblastoma , 357–359 secundum-type ASD, 425 Non-cardiac surgery, infants and children , sternal closure, 407 444–448 TEE , 402 Non-depolarizing neuromuscular ultrafi ltration, 406 blocking drugs (NMBDs), 43 Operating room (OR), 6 Non-obstructive hydrocephalus , 12 Operations for epilepsy, 250–251 Normocapnia , 394 Ophthalmic medications side effects, 257 , 258 systemic effects, 257 , 258 O Ophthalmology . See Eye surgery Obesity , 177–178 Opioids Obstructive hydrocephalus , 11 infusions , 462 Obstructive lesions , 393 premedication , 90 Obstructive sleep apnea (OSA) Optimal external laryngeal manipulation intensive monitoring and (OELM), 94 postoperative care, 276–277 Orchidopexy , 383 polysomnography , 276 Organ transplantation , 374 , 375 postoperative sleep studies, 277 Index 653

heart , 205–206 P liver , 206 , 376 Packed red blood cells (PRBCs), 130 lung , 206 P A C U. See Post-anesthesia care unit Orthopedic surgery (PACU) acute normovolemic hemodilution, 459 Pain management anesthesia , 454 assessment , 216 caudal epidural analgesia, 453 postoperative (see Postoperative pain club feet , 453 management ) corrective surgery, 458–464 Palliative surgery, pulmonary blood fl ow heart rate and blood pressure, 451 anastomosis , 422–424 hemodynamic and metabolic congenital abnormalities , 424 responses, 452 distal PA pressure , 424 malignant hyperthermia, 451 fentanyl , 423 metabolic/espiratory acidosis, 452 heparin and fl uid therapy , 422 neuromuscular disease , 451 polycythemia , 422 optimal management, 452 pulmonary blood fl ow, 423 pulmonary management , 466 shunting procedures, 422 urinary retention, 453 Pancuronium , 69 OSA . See Obstructive sleep apnea (OSA) Paradoxical movement, 18 Otologic conditions Parental presence at induction awake ear surgery , 285 (PPIA), 4 major procedures Patient-controlled analgesia (PCA) perioperative anesthesia system , 221–222 management , 284–285 Patent ductus arteriosus (PDA) postoperative anesthesia anesthesia , 415 , 416 management , 285 anomalous innominate artery preoperative anesthesia suspension, 417–418 management , 284 older infants and children , 413 , 414 minor procedures premature baby , 413 perioperative anesthesia preterm infants , 415–417 management , 283 routine post-thoracotomy, 414 postoperative anesthesia treatment , 415 management , 283 vascular rings, 417–418 preoperative anesthesia Pediatric anesthesia management , 283 craniofacial surgery , 1 special anesthesia problems, 283 distraction strategies , 6 special anesthesia problems, 282 genitalia surgery, 1 Outpatient surgery induction , 4 , 5 advantages , 135 informed consent , 3 anesthesia techniques, 136–138 OR , 6 complications , 139 parental anxiety, 3 patient selection , 135 postoperative care, 7 postoperative care, 138–139 premedication , 5 , 6 preoperative preparation , 135 psychological preparation, 2 Oxycodone , 61 , 220 separation , 1 654 Index

Pediatric invasive neuroradiology mild analgesics, 218–220 anesthetic management, 254 morphine infusion rate , 221 special anesthesia problems, 253–254 PCA system , 221–222 Penetrating eye trauma regional analgesia, 223–224 perioperative anesthesia management , systemic analgesic drugs , 218 265 Potassium replacement, 126 postperative anesthesia management , Preductal (infantile type) , 419 265 Premedication preoperative anesthesia management , opioids , 90 265 outpatient surgery , 136 special anesthesia problems, 264–265 sedatives and tranquilizers Peripheral nerve stimulator , 115 clonidine , 89 Peritonsillar abscess (quincy), 281 dexmedetomidine , 89 Periventricular leukomalacia, 11 ketamine , 89 Pharyngoplasty lorazepam , 89 anesthesia management , 317 midazolam , 88–89 special anesthesia problems, 317 special considerations, 90 Pheochromocytoma , 367–369 topical local anesthetics , 86 Phrenic nerve injury , 411 vagal blocking drugs Plasmapheresis , 365 atropine , 87 Plastic surgery principle , 311 glycopyrrolate , 86 Pneumothorax , 338 hyoscine , 86 Polycythemia , 391 Preoperative preparation Polysomnography , 276 concurrent medications and anesthetic Polysplenia syndrome , 352 implications, 78 , 82–83 PONV . See Postoperative nausea and fasting , 77 , 78 vomiting (PONV) hemorrhage prophylaxis, 85 Positive end-expiratory pressure herbal preparations and anesthetic (PEEP), 395 implications, 78 , 84 Post-anesthesia care unit (PACU) informed consent , 84–85 anesthesiologist role, 211–212 laboratory tests , 86 emergence delirium, 213 medical history, review of , 78–81 general management, 211–212 outpatient surgery , 135 laryngospasm , 212–213 premedication (see Premedication ) PONV , 214 Preoperative urinalysis, 86 shivering and rigidity, 213 Pressure support ventilation (PSV) , 113 stay duration, 214–215 Preterm infants stridor , 213 apnea , 133–134 Postductal (adult type) , 420–422 benzyl alcohol , 134 Posterior fossa surgery, 247 coagulation , 134 Postintubation croup, 92 fl uid administration, 134 Postobstructive pulmonary edema, 92 hypoglycemia and hyperglycemia, 134 Postoperative nausea and vomiting infection , 133 (PONV), 214 intraventricular hemorrhage , 133 Postoperative pain management oxygenation , 134 Index 655

temperature control, 134 diuresis , 478 Prophylactic antibiotic therapy, 446 glomerular function, 480 Propofol (2,6-diisopropylphenol) hemodynamic stability, 479 airway refl exes, 54 hyperkalemia and acidosis, 479 application , 55 hypoglycemia , 480 asepsis , 53 immunosuppressive therapy, 478 pain , 54 pulmonary edema , 480 PRIS , 55 by pulse oximetry , 480 TIVA , 54 tubular damage , 480 Propofol infusion syndrome (PRIS) , 55 vascular anastomosis, 479 Pulmonary edema , 394 Respiratory system Pulmonary function, 455 anatomic difference, 13 , 14 Pulmonary hypertensive crisis, 393 apnea , 17 Pulmonary vascular obstructive disease in infants and children, 410 , 411 (PVOD), 427 lung growth and development, 21 Pulmonary vascular resistance (PVR), lung surfactants , 20 , 21 23, 339 lung volumes, 19 Pulseless electrical activity (PEA), 144 periodic breathing, preterm , 17 Pulse oximeter , 113 physiology , 14 Pyeloplasty , 470 pulmonary function testing , 21 Pyloric stenosis , 347 PVR , 411 ventialtion and perfusion relationship, 20 ventilatory response, hypercapnia, 16 Q Retinoblastoma , 266 Quinsy tonsil. See Peritonsillar abscess Retinopathy of prematurity (ROP) , 12 , 265–266 R Retrograde intubation, 109 Radial artery cannulation, 116–117 Rigid esophagoscopy, 293–294 Radiation therapy, 517 Rocuronium , 68 Rapid sequence induction (RSI), 98 , 485 Ropivacaine , 143 Recombinant human erythropoietin therapy , 471 S Reconstructive burn surgery Scalds , 498 , 500 anesthesia management , 320–322 S C D. See Sickle cell disease (SCD) special anesthesia problems, 320–322 Scoliosis surgery , 456–458 , 465–466 Regional analgesia . See Local anesthetics Sedatives , 88–89 Reimplantation of ureters, 470 Selective posterior rhizotomy , Remifentanil , 63 252–253 Remifentanil-based TIVA protocol, 465 S e v o fl urane Remote anesthesia, 507 CBF and oxygen consumption , 45 Renal failure , 471 CYP2E1 affi nity, 46 Renal transplantation degradation , 46 anesthetic management, 478 emergence delirium, 46 aseptic technique, 478 epileptiform EEG activity , 46 656 Index

Sevofl urane (cont. ) Supraglottic infections. See Epiglottitis fl uorinated methyl isopropyl Supraventricular tachycardia (SVT), 622 ether, 44 Surfactant therapy , 343 MAC , 45 Syndrome of inappropriate antidiuretic myocardial depression , 45 hormone secretion (SIADH) , single breath induction, 45 234 , 466 TIVA , 45 Syringomyelia , 244 ventilation , 45 Systemic vascular resistance (SVR), 23 Shikani optical stylet , 109 Sickle cell disease (SCD), 193 T hemoglobin electrophoresis , 194 Teratomas , 326 perioperative anesthesia management , Testicular torsion, 373 196 Tethered cord , 252 postoperative anesthesia management, Tetralogy of Fallot (TOF), 428 , 429 196 Thermistor probe , 115 , 121 preoperative anesthesia management , Thoracoabdominal injury 195 anesthesia special anesthesia problems, 194 maintenance , 495–496 Sickle cell test, 86 perioperative management , 495 Somatosensory- evoked potentials postoperative management, 496 (SSEPs), 230 preoperative management, 494–495 Spinal cord injury without radiographic problems , 494 abnormalities (SCIWORA) , chest wall and lungs, 497 485, 493 great vessels, 498 Spinal cord tumors , 252 heart and pericardium injury, 498 Spinal osteotomy, 465–466 hepatic injury, 496 Splenectomy , 367 immediate management, 494 Stethoscope , 114 initial assessment, 494 Strabismus correction laparoscopy , 493 associated conditions , 260 renal injury , 496 perioperative anesthesia management , ruptured diaphragm, 497 261–262 tracheal/bronchial injury, 497 postoperative anesthesia management, Thoracoscopy , 380 , 381 262 Thoracotomy , 128 preoperative anesthesia management , Thrombocytopenia , 134 261 Thymectomy , 365 special anesthesia problems, 260–261 Tongue tie , 381 Subendocardial ischemia, 394 Tonsillectomy and adenoidectomy (T&A) Subglottic edema, 92 anesthesia management , 278 Subglottic hemangioma, 288–289 cardiorespiratory syndrome , 277 Subglottic stenosis, 301 indications , 275–276 anesthesia management , 301–302 OSA anterior cricoid split procedure , 302 intensive monitoring and associated conditions , 301–302 postoperative care, 276–277 Succinylcholine , 66 , 259 Index 657

polysomnography , 276 burns and scalds (see Burn injury ) postoperative sleep studies, 277 cause of death , 483 peritonsillar abscess , 281 cervical spine injury, 493 postoperative anesthesia management, defi nitive care , 483 278–279 Glasgow coma scale and pediatric reoperation for bleeding , 279–281 modifi cations, 490–493 special anesthesia problems, 278 gunshot wounds, 505 Topical anesthetics , 86 head injury, 490 Total Anomalous pulmonary venous initial fl uid resuscitation, 487 drainage, 426 intravenous therapy, 486–487 Total intravenous anesthesia (TIVA), 54 , massive transfusion, 484 , 488–490 136 , 462 minor , 505 T-piece system , 111 primary survey , 483 Tracheal injury, 497 secondary survey, 483 Tracheal intubation, 95–97 thoracoabdominal injury Tracheal tubes , 90 , 91 (see Thoracoabdominal injury ) Tracheoesophageal fi stula (TEF) Tricuspid atresia, 433 bronchoscopy , 342 Truncus arteriosus, 434 complications , 345 Tympanic membrane probes , 121 H-type fi stula, 340 maternal polyhydramnios, 340 U nesthesia management Upper respiratory tract infection (URTI) management-staged repair, 345 complications , 167 primary repair, 342–345 management algorithm, 168 plain radiography, 340 Urologic surgery surgical management , 342 acid–base balance, 475 tracheal defects , 342 antibiotics , 473 tracheal stenosis, 340 blood pressure determinations, 474 Tracheotomy children, poor renal function , 471–477 perioperative anesthesia management , child’s renal function , 469 299–300 genitourinary , 470 postoperative anesthesia management, inhalational anesthetics , 476 300 muscle relaxants, 476 , 477 preoperative anesthesia management , opioid doses, 477 299 succinylcholine , 476 Trachlight , 108 tracheal intubation, 477 Tramadol , 73 , 221 with normal renal function, 469–470 Tranquilizers , 88–89 Transposition, great arteries, 429 , 430 Trauma V accidents , 483 VACTERL. See VATER airway, establishment, 484–486 Vagal blocking drugs anesthesiologist, problems , 483–484 atropine , 87 autotransfusion , 490 glycopyrrolate , 86 blood transfusion, indications, 487 hyoscine , 86 658 Index

Vascular anomalies, 245–248 Ventricular fi brillation (VF), 623 VATER , 341 , 609 Ventricular tachycardia (VT), 623 Venous air embolism (VAE), 236–237 Visual evoked potentials (VEPs) , Venous cannulation, 124 267–268 Ventilation Visual loss, 460 manual , 111 Visually assisted thoracic surgery mechanical , 112–113 (VATS), 333

Ventilator induced lung injury (VILI) , 112 Vitamin K1 , 8 5 Ventricular septal defect (VSD), 427 Vitamin K defi ciency bleeding (VKDB) , 85 anesthesia plan, 427 Volume targeted ventilation (VTV) , 112 complications , 427 Von Willebrand disease (VWD), 198 hybrid techniques , 427 V W D. See Von Willebrand disease (VWD) interrupted aortic arch, 422 physiologic effects, 427 type I (supracristal, 427 W type II (infracristal) , 427 Wilms tumors (Nephroblastoma), type III (AV canal type), 427 370 , 371 type IV (muscular) , 427 Wong-Baker FACES pain rating scale, 216