DOCSLIB.ORG

Congenital Diaphragmatic Hernia

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Congenital Diaphragmatic Hernia Fetal Neck and Chest Lesions Extrinsic Compression Cervical teratoma Mediastinal teratoma Lymphangioma Goiter Thyroid cysts Thyroglosal duct cyst Branchial cleft cyst Neuroblastoma Hamartoma Hemangioma Lipoma Parotid tumor Cervical MMC Epignathus Fetal Neck Masses Cervical Teratomas •All 3 germ layers represented •Totipotential germ cells vs conjoined twin •40% contain thyroid tissue •Large bulky tumors •Airway compromise •Polyhydramnios in 40% •Benign lymph node metastases •Thyroid destruction •Parathyroid engulfment •Mandibular hypoplasia •Marginal mandibular nerve palsy •Laryngotracheomalacia Fetal Neck Masses •No matter the etiology •Very large masses can cause: •Neck hyperextension •Pulmonary hypoplasia •Secondary to carina pulled into neck •Lungs pulled into apex of hemithorax TheThe EXITEXIT ProcedureProcedure Airway Algorithm •Direct laryngoscopy •Flexible bronchoscopy •Rigid bronchoscopy •Tracheotomy with retrograde intubation •Tracheostomy •Mass resection + tracheostomy •TRI-EXIT Establishing an airway Laryngoscopy Bronchoscopy Tracheostomy Resection Congenital High Airway Obstruction Syndrome Prenatal Diagnosis Often misdiagnosed Bilateral CCAMs Bilateral extremely large echogenic lungs Compressed heart and mediastinum Non‐immune hydrops Complete airway obstruction MRI US CHAOS Intrinsic Airway Obstruction •Laryngeal atresia/stenosis •Tracheal atresia/stenosis •Laryngeal cyst Prenatal Natural History • 2/3 Hydrops and IUFD • 1/3 Resolution of hydrops •Laryngeal fistula •Tracheoesophageal fistula CHAOS • Diagnosed with bilateral CCAMs CHAOS • Dilated tracheobronchial tree • Massively enlarged lungs • Inverted and stretched diaphragms • Non-immune hydrops • Massive Ascites • Complete airway obstruction CHAOS CHAOS Fetal Lamb Model of CHAOS •Fetal tracheal occlusion at mid‐gestation •Non‐immune hydrops •Massive ascites •Massively enlarged lungs •Inverted stretched diaphragms •Dilated tracheobronchial tree •Release of tracheal occlusion •Resolution of hydrops •Normal at delivery CHAOS CHAOS Profound capillary leak syndrome Severe RDS Hepatic synthetic defect Diaphragmatic paralysis Stretch injury Hyper‐compliant lungs Ascites‐behave like cirrhotic Gut disfunction Prune belly Intact larynx Subglottic trecheal atresia Tracheal Reconstruction for CHAOS Laryngo-Tracheoplasty - Definition of natural history - Shunts - Open fetal surgery CCAM - Steroids Fetal MRI • CPAM Volume Ratio (CVR) - 3 dimensions volume of lesion /Head circumference Saggital - CVR= .52 x L x W x H HC - >1.6 high risk hydrops Transverse • Fastest rate of growth 20- 25 weeks • Plateau at 26 weeks Range 24-28 weeks • No growth once plateau reached • CAM volume lagged behind CVR • Low risk CPAM - Excellent survival (98%) • High risk CPAM n=42 − Lower survival(71.4%) • Excellent survival in non-hydropic high risk CPAM treated with steroid (100%) 8 weeks • Hydropic CPAM treated with steroids post steroid - Survival 45% compared to 0% in historical controls - 88.9% survival with resolution of hydrops - Persistent of hydrops predicts mortality Fetal Pericardial Teratoma • Rare benign congenital tumor • 40 prenatally diagnosed cases • 2:1 female to male ratio • Often associated pericardial effusion • Rapid growth between 20-30 wks • 50% develop hydrops • Polyhydramnios common • Fatal in setting of hydrops/poly • Fetal Therapy • Pericardiocentesis • Fetal surgery Fetal Pericardial Teratoma Open Pericardium to expose Teratoma Fetal Pericardial Teratoma Resection/repair complete Improved Survival and Decreased Need for ECMO in CDH CFCC Liver 33 56% Patch 33 67% Pre‐CDH Team CDH Team CDH Team CHCO & FCC CDH Team Longest Methods LHR Right Lung Area/Head Circumference UCSF AP Method Tracing Methods Observed to Expected LHR Survival with Conventional Postnatal Therapy DePrest et al, Seminars Fetal and Neonatal Medicine 2008 Congenital Diaphragmatic Hernia Percent Predicted Lung Volume (PPLV) Total Lung Volume Total Thoracic Vol‐Mediastinal Vol = PPLV Mahieu‐Caputo et al BJOG 108: 863‐868, 2001 Paek et al Radiology 220: 63‐67, 2001 Gorincour et al Ultrasound Obstet Gynecol 26: 738‐744 Barnewolt et al J Pediatr Surg 42: 193‐197, 2007 TotalTotal LungLung VolumeVolume • By 34 wk fetal lung growth should be complete. • Fetal MRI to estimate total lung volume • Goal to identify, counsel, and develop a treatment plan for high risk CDH Mahieu‐Caputo et al. BJOG 2001; 108:863 Neff et al. AJR 2007;189:1307 patient Rypens et al. Radiology 2001; 236 AssessmentAssessment ofof PostnatalPostnatal PulmonaryPulmonary HypertensionHypertension inin CDHCDH • All prognostic indicators focus on lung - Indirect indicators - LHR, PPLV, TLV, Liver position - All measure lung size or volume • Indicators of pulmonary hypoplasia • No prenatal measurement has tried to predict severity of postnatal pulmonary hypertension Prenatal Pulmonary Hypertension Index== PPHIPPHI Vermis LPA RPA PPHI = (LPA d / Vermis l) x 10 ModifiedModified McGoonMcGoon IndexIndex == MGIMGI LPA Ao RPA MGI = (RPA d + LPA d) / Ao d CDH Prognostic Profile • Congenital heart disease Criteria for EXIT-to-ECMO • Karyotype (and tracheal occlusion) • Liver position Liver up • O/E LHR LHR< 1.0 - <25th% 20% survival • LHR PPLV <15 - >1.0 100% survival TLV <18 - < 1.0 50% survival Normal karyotype • PPLV early and late - < 15% high risk - > 22% low risk - Late more reliable • TLV late - > 25cc favorable - < 18cc unfavorable • Modified Magoon Index? - <0.8 severe pulm htn - >1.0 mild pulm htn CDH-Composite Prognostic Index (CDH-CDI) CDHCDH--CompositeComposite PrognosticPrognostic IndexIndex (CDH(CDH--CDI)CDI) CDHCDH TRACHEAL OCCLUSION • FETO Task Group • Leuven, Barcelona, London • FETO at 26-28 wks • Prenatal reversal of tracheal occlusion • Outcomes in 210 cases - Survival 49% • Conventional therapy - Survival 20% • NA Consortium: TOTAL • CHOP, Cincinnati, Denver • Maryland and Texas Deprest et al J Pediatr Surg 2011 • Toronto Jani et al Ultrasound Obstet Gynecol 2009 EXITEXIT--toto--ECMOECMO forfor HighHigh RiskRisk CDHCDH • Rationale: - Minimize hemodynamic instability - Minimize barotrauma - Eliminates resuscitation - Time for pulm vascular remodeling - Smooth transition to postnatal life - Early repair to allow remodeling • Maternal risks - Deep general anesthesia - Cesarean section for subsequent pregnancy - Risk of uterine atony Bouchard S, Johnson MP, Flake AW, Howell LJ, Myers LB, Adzick NS, Crombleholme TM: The EXIT Procedure: Experience and Outcome in 31 Cases. J Pediatr Surg 37: 428‐426, 2002 Met EXIT‐to‐ECMO Criteria Excluded for N=19 Abnormal Posture N=2 Excluded for Comfort Care EXIT‐to‐ECMO Non‐EXIT N=1 N=8 N=8 Cesarean Delivery Vaginal Delivery N=7 N=1 Cesarean Cesarean with Delivery Alone ECMO Standby N=5 N=2 ECMO ECMO* ECMO ECMO 00% (8/8) 60% (3/5)50% (1/2) 100% (1/1) Survival Survival Survival 0% 20% (1/5) 50% (1/2) EXIT‐to‐ECMO Non‐ EXIT Delivery Survival Survival 50% (4/8) * 2 died at birth 25% (2/8) Conclusions • Essential role of prenatal imaging in neck and chest lesions • Combination of ultrasound, MRI and echocardiography • Serial assessment • Prognostic assessment • Treatment options •Open fetal surgery •Fetoscopic treatment? •Interstitial laser treatment •EXIT strategy •EXIT‐to‐Airway •EXIT‐to‐Resection Fetal Care Center of Cincinnati •Maternal-Fetal Medicine •Pediatric Surgery •Neonatology − Mounira Habli MD •Timothy Crombleholme,MD − James Greenberg, MD − Tanya Cahill, MD −William Polzin, MD •Foong-Yen Lim,MD −Kim Brady, MD − Kurt Schibler, MD −Jim VanHook, MD •Sundeep Keswani MD − Beth Haberman, MD −Ronald Jaekle,MD •Anesthesiology − Paul Kingma, MD −David Lewis, MD –Ann Boat,MD − Amy Nathan, MD •Nursing Director •Fetal Echocardiography –Mohammad Mahmoud,MD −Erik Michelfelder, MD − Kelli Young –Bonnie Hugus −James |Cnota,MD •Coordinators −William Gottliebson, MD •Radiology − Gina Sharp, RN −Allison Divanovic,MD •Connie Bitters − Deb Voet, RN −Haleh Hadarian, MD •Linda Martin − Jenni Mason − Steve Imhoff •Urology/Nephrology –Beth Kline-Fath, MD −Pramod Reddy,MD − Judy Hostiuck −Robert Defore,MD –Maria Calvo, MD •Genetics −Elizabeth Jackson,MD –Eva Rubio, MD − Rob Hopkin,MD •ENT –Leann Linam, MD − Howard Saul,MD − Christine Spaeth, CGC −Ravi Ellarhu,MD –Kyuran Choe, MD −Paul Willging,MD − Diana Smith, CGC •Operating Room Nurses •Neurosurgery •Social Services −Karin Bierbrauer,MD •Missy Ritter − Erin Hartman, MSW −Francesco Mangano,MD •Stacy Ruth •Administrative •Cardiac Surgery •Tracy Heidrich − Rachel Jones − Emmie Beyer −Pirooz Eghtesady,MD •Latressa Ratner −Peter Manning,MD − Cheryl Snell •Curtis Johnson •Level III L&D Nurses • Good Samaritan Hospita •Nurse Midwife – Karen McGirr Colorado Fetal Care Center •Maternal-Fetal Medicine • Fetal and Pediatric Surgery •Neonatology - Henry Galan, MD •Timothy M Crombleholme MD - Regina Reynolds, MD - Shane Reeves, MD •Stigg Somme, MD - Peter Hulac, MD - John Hobbins MD •Anesthesiology - Jason Gien, MD - Joyce Sung, MD –Geoffrey Galinkin, MD - John Kinsella, MD •Fetal Echocardiography –Debnath Chaterjee, MD - Therese Grover MD - Adel Younozsai, MD –Joy Hawkins, MD •Coordinators - Lisa Howley, MD – Carrie Dean, MD - Kelli Young, RN - Pei Ni Jone MD –Christine Wood, MD - Jann Hodge, RN - Karrie Villavicencio,MD •Radiology - Carrie Rafferty, RN •Genetics •Urology/Nephrology • Lisa Waters, RDMS - David Manchester, MD - Duncan Wilcox, MD – Mariana Meyers, MD - Naomi Meks, MD - Jeffrey Campbell, MD – Kim Dannull, MD - Vijay Velmulakonda, MD – Laura Brown, MD •Administrative •ENT – David Mersky, MD - Peggy Kelley MD • Obstetric Nursing - Kelli Young, RN, CNP - Jeremy Prager, MD •Jennifer Livingston, MD - Mary Beth Martin, RN, MBA •Neurosurgery •Jena - Gina Dooley - Michael Handler, MD •Tiffany - Corbett Wilkinson, MD •Operating Room •Cardiac Surgery • Erin Hughey,RN - James Jaggers, MD • Nicole Stuart, RN - David Campbell, MD • Heidi - Max Mitchell, MD .
Load more

Recommended publications
  • Lung Pathology: Embryologic Abnormalities
    Chapter2C Lung Pathology: Embryologic Abnormalities Content and Objectives Pulmonary Sequestration 2C-3 Chest X-ray Findings in Arteriovenous Malformation of the Great Vein of Galen 2C-7 Situs Inversus Totalis 2C-10 Congenital Cystic Adenomatoid Malformation of the Lung 2C-14 VATER Association 2C-20 Extralobar Sequestration with Congenital Diaphragmatic Hernia: A Complicated Case Study 2C-24 Congenital Chylothorax: A Case Study 2C-37 Continuing Nursing Education Test CNE-1 Objectives: 1. Explain how the diagnosis of pulmonary sequestration is made. 2. Discuss the types of imaging studies used to diagnose AVM of the great vein of Galen. 3. Describe how imaging studies are used to treat AVM. 4. Explain how situs inversus totalis is diagnosed. 5. Discuss the differential diagnosis of congenital cystic adenomatoid malformation. (continued) Neonatal Radiology Basics Lung Pathology: Embryologic Abnormalities 2C-1 6. Describe the diagnosis work-up for VATER association. 7. Explain the three classifications of pulmonary sequestration. 8. Discuss the diagnostic procedures for congenital chylothorax. 2C-2 Lung Pathology: Embryologic Abnormalities Neonatal Radiology Basics Chapter2C Lung Pathology: Embryologic Abnormalities EDITOR Carol Trotter, PhD, RN, NNP-BC Pulmonary Sequestration pulmonary sequestrations is cited as the 1902 theory of Eppinger and Schauenstein.4 The two postulated an accessory he clinician frequently cares for infants who present foregut tracheobronchia budding distal to the normal buds, Twith respiratory distress and/or abnormal chest x-ray with caudal migration giving rise to the sequestered tissue. The findings of undetermined etiology. One of the essential com- type of sequestration, intralobar or extralobar, would depend ponents in the process of patient evaluation is consideration on the timing of the accessory foregut budding (Figure 2C-1).
    [Show full text]
  • 427 © Springer Nature Switzerland AG 2020 R. H. Cleveland, E. Y. Lee
    Index A Arterial hypertensive vasculopathy ABCA3 deficiency, 158 imaging features, 194 pathological features, 194 Aberrant coronary artery, 22 Aspergillosis, 413 Acinar dysplasia, 147–150 Aspergillus, 190, 369, 414 Acquired bronchobiliary fistula (aBBF), 79, 80 Aspergillus fumigatus, 110, 358, 359 Acquired immunodeficiency, 191, 192 Aspergillus-related lung disease, 413 Aspiration pneumonia, 136 Acrocyanosis, 1 Aspiration syndromes, 248 Actinomycosis, 109, 110 causes, 172 Acute cellular rejection (ACR), 192, 368 imaging features, 172 Acute eosinophilic pneumonia, 172 pathological features, 172 Acute infectious disease Associated with pulmonary arterial hypertension (APAH), 257 imaging features, 167 Asthma pathological features, 167 ABPA, 343 Acute interstitial pneumonia (AIP), 146, 173, 176 airway biopsy, 339 imaging features, 176 airway edema, 337 pathological features, 176 airway hyperresponsiveness, 337 symptoms, 176 airway inflammation, 337 Acute Langerhans cell histiocytosis (LCH), 185 allergy testing, 339 Acute pulmonary embolism, 386, 387 anxiety and depression, 344 complications, 388 API, 339 mortality rates, 388 biodiversity hypothesis, 337 pre-operative management, 387 bronchial challenge tests, 338 technique, 387, 388 bronchoconstriction, 337 treatment, 387 bronchoscopy, 339 Acute rejection, 192 clinical manifestations, 337 Adenoid cystic carcinoma, 306, 307 clinical presentation, 338 Adenotonsillar hypertrophy, 211 comorbid conditions, 342, 343 Air bronchograms, 104 definition, 337 Air leak syndromes, 53, 55 diagnosis, 338–340,
    [Show full text]
  • Surfactant Abnormalities in ALTE and SIDS Arch Dis Child: First Published As 10.1136/Adc.71.6.501 on 1 December 1994
    Archives ofDisease in Childhood 1994; 71: 501-505 501 Surfactant abnormalities in ALTE and SIDS Arch Dis Child: first published as 10.1136/adc.71.6.501 on 1 December 1994. Downloaded from I B Masters, J Vance, B A Hills Abstract mechanical stability of the distal airspaces.2 Abnormalities in the relative concentra- Reduced concentrations of surfactant, in tions ofthe components ofsurfactant have particular disaturated phosphatidylcholine been implicated in prolonged expiratory (DPPC) have been described in infants apnoea (PEA) and sudden infant death with PEA and SIDS.3-9 Southall et al have syndrome (SIDS). Controversy has, implicated low concentrations of DPPC and however, surrounded these findings, as postulated lung mechanic, neurosensory, and they may be secondary to terminal pulmonary vascular mechanisms for these life events. In this study the physical events.5 6 properties of surfactant were measured in Previously we have shown that both an children with recurrent apparent life infant and young child with prolonged threatening events (ALTEs), PEA, and expiratory apnoea had significant quantitative SIDS. Bronchial lavage samples were and qualitative abnormalities in their obtained from 21 children with recurrent surfactant.'I These findings were similar to the ALTEs, two SIDS victims, and 26 control low concentrations of DPPC found in other patients. Lipid components were immedi- studies.2-5 The reliability of such findings, ately elutriated from these samples however, is questionable in both those and with liquid chloroform. The physical our own observations, as the ability to extract properties of the extracted surfactant surfactant with lung washings in a standardised were studied on a Langmuir trough in fashion from the living subject's lung is which the area (A) of the monolayer was difficult.
    [Show full text]
  • Adult Outcome of Congenital Lower Respiratory Tract Malformations M S Zach, E Eber
    500 Arch Dis Child: first published as 10.1136/adc.87.6.500 on 1 December 2002. Downloaded from PAEDIATRIC ORIGINS OF ADULT LUNG DISEASES Series editors: P Sly, S Stick Adult outcome of congenital lower respiratory tract malformations M S Zach, E Eber ............................................................................................................................. Arch Dis Child 2002;87:500–505 ongenital malformations of the lower respiratory tract relevant studies have shown absence of the normal peristaltic are usually diagnosed and managed in the newborn wave, atonia, and pooling of oesophageal contents.89 Cperiod, in infancy, or in childhood. To what extent The clinical course in the first years after repair of TOF is should the adult pulmonologist be experienced in this often characterised by a high incidence of chronic respiratory predominantly paediatric field? symptoms.910 The most typical of these is a brassy, seal-like There are three ways in which an adult physician may be cough that stems from the residual tracheomalacia. While this confronted with this spectrum of disorders. The most frequent “TOF cough” is both impressive and harmless per se, recurrent type of encounter will be a former paediatric patient, now bronchitis and pneumonitis are also frequently observed.711In reaching adulthood, with the history of a surgically treated rare cases, however, tracheomalacia can be severe enough to respiratory malformation; in some of these patients the early cause life threatening apnoeic spells.712 These respiratory loss of lung tissue raises questions of residual damage and symptoms tend to decrease in both frequency and severity compensatory growth. Secondly, there is an increasing with age, and most patients have few or no respiratory number of children in whom paediatric pulmonologists treat complaints by the time they reach adulthood.13 14 respiratory malformations expectantly; these patients eventu- The entire spectrum of residual respiratory morbidity after ally become adults with their malformation still in place.
    [Show full text]
  • (12) Patent Application Publication (10) Pub. No.: US 2010/0210567 A1 Bevec (43) Pub
    US 2010O2.10567A1 (19) United States (12) Patent Application Publication (10) Pub. No.: US 2010/0210567 A1 Bevec (43) Pub. Date: Aug. 19, 2010 (54) USE OF ATUFTSINASATHERAPEUTIC Publication Classification AGENT (51) Int. Cl. A638/07 (2006.01) (76) Inventor: Dorian Bevec, Germering (DE) C07K 5/103 (2006.01) A6IP35/00 (2006.01) Correspondence Address: A6IPL/I6 (2006.01) WINSTEAD PC A6IP3L/20 (2006.01) i. 2O1 US (52) U.S. Cl. ........................................... 514/18: 530/330 9 (US) (57) ABSTRACT (21) Appl. No.: 12/677,311 The present invention is directed to the use of the peptide compound Thr-Lys-Pro-Arg-OH as a therapeutic agent for (22) PCT Filed: Sep. 9, 2008 the prophylaxis and/or treatment of cancer, autoimmune dis eases, fibrotic diseases, inflammatory diseases, neurodegen (86). PCT No.: PCT/EP2008/007470 erative diseases, infectious diseases, lung diseases, heart and vascular diseases and metabolic diseases. Moreover the S371 (c)(1), present invention relates to pharmaceutical compositions (2), (4) Date: Mar. 10, 2010 preferably inform of a lyophilisate or liquid buffersolution or artificial mother milk formulation or mother milk substitute (30) Foreign Application Priority Data containing the peptide Thr-Lys-Pro-Arg-OH optionally together with at least one pharmaceutically acceptable car Sep. 11, 2007 (EP) .................................. O7017754.8 rier, cryoprotectant, lyoprotectant, excipient and/or diluent. US 2010/0210567 A1 Aug. 19, 2010 USE OF ATUFTSNASATHERAPEUTIC ment of Hepatitis BVirus infection, diseases caused by Hepa AGENT titis B Virus infection, acute hepatitis, chronic hepatitis, full minant liver failure, liver cirrhosis, cancer associated with Hepatitis B Virus infection. 0001. The present invention is directed to the use of the Cancer, Tumors, Proliferative Diseases, Malignancies and peptide compound Thr-Lys-Pro-Arg-OH (Tuftsin) as a thera their Metastases peutic agent for the prophylaxis and/or treatment of cancer, 0008.
    [Show full text]
  • Fetal Lung Hypoplasia: Biochemical and Structural Variations and Their Possible Significance
    Arch Dis Child: first published as 10.1136/adc.56.8.606 on 1 August 1981. Downloaded from Archives of Disease in Childhood, 1981, 56, 606-615 Fetal lung hypoplasia: biochemical and structural variations and their possible significance J S WIGGLESWORTH, R DESAI, AND P GUERRINI Department ofPaediatrics and Neonatal Medicine, Institute of Child Health, Hammersmith Hospital, London SUMMARY Quantitative biochemical criteria for lung growth and maturation were compared with the histological appearances in hypoplastic lungs from 20 fetuses and newborn infants. Cases associated with oligohydramnios showed a characteristic series of changes with narrow airways, retardation of epithelial and interstitial growth, delay in development of blood-air barriers, and low concentrations of phospholipid phosphorus, lecithin phosphorus, total palmitate, and lecithin palmitate. The growth and maturation arrest appeared to affect the peripheral part of the acinus. Examples of other types oflung hypoplasia showed different features. Hypoplastic lungs from infants with normal or increased amniotic fluid were of mature structure with phospholipid concentrations similar to those of infants with normally developed lungs at term. The hypoplastic left lung in 2 cases of congenital diaphragmatic hernia had an immature structure with low phospholipid con- centrations, whereas the right lung was structurally and biochemically more mature. It is suggested that fetal lung growth may be impaired by any influence which reduces thoracic volume but that maturation arrest is due specifically to loss ofthe ability to retain lung liquid. copyright. It is now recognised that hypoplasia of the fetal arrest." Other studies on infants with renal agenesis lungs is the immediate cause of neonatal death in a or dysplasia have found the lungs to be structurally number of malformation syndromes.
    [Show full text]
  • Respiratory Distress in the Newborn
    Respiratory Distress in the Newborn Suzanne Reuter, MD,* Chuanpit Moser, MD,† Michelle Baack, MD*‡ *Department of Neonatal-Perinatal Medicine, Sanford School of Medicine–University of South Dakota, Sanford Children’s Specialty Clinic, Sioux Falls, SD. †Department of Pediatric Pulmonology, Sanford School of Medicine–University of South Dakota, Sanford Children’s Specialty Clinic, Sioux Falls, SD. ‡Sanford Children’s Health Research Center, Sioux Falls, SD. Educational Gap Respiratory distress is common, affecting up to 7% of all term newborns, (1) and is increasingly common in even modest prematurity. Preventive and therapeutic measures for some of the most common underlying causes are well studied and when implemented can reduce the burden of disease. (2)(3)(4)(5)(6)(7)(8) Failure to readily recognize symptoms and treat the underlying cause of respiratory distress in the newborn can lead to short- and long-term complications, including chronic lung disease, respiratory failure, and even death. Objectives After completing this article, the reader should be able to: 1. Use a physiologic approach to understand and differentially diagnose the most common causes of respiratory distress in the newborn infant. 2. Distinguish pulmonary disease from airway, cardiovascular, and other systemic causes of respiratory distress in the newborn. 3. Appreciate the risks associated with late preterm (34–36 weeks’ gestation) and early term (37–38 weeks’ gestation) deliveries, especially AUTHOR DISCLOSURES Drs Reuter, Moser, by caesarean section. and Baack have disclosed no financial 4. Recognize clinical symptoms and radiographic patterns that reflect relationships relevant to this article. This commentary does not contain information transient tachypnea of the newborn (TTN), neonatal pneumonia, about unapproved/investigative commercial respiratory distress syndrome (RDS), and meconium aspiration products or devices.
    [Show full text]
  • Pulmonary Hypoplasia: Lung Weight and Radial Alveolar Count As Criteria of Diagnosis
    Arch Dis Child: first published as 10.1136/adc.54.8.614 on 1 August 1979. Downloaded from Archives of Disease in Childhood, 1979, 54, 614-618 Pulmonary hypoplasia: lung weight and radial alveolar count as criteria of diagnosis S. S. ASKENAZI AND M. PERLMAN Hadassah Hospital, Jerusalem SUmmARY A working definition of pulmonary hypoplasia (PH) was established by retrospective assessment of lung growth both in recognised and hypothetical PH-associated conditions. Lung weight: body weight ratios (LW:BW) were calculated, and morphometry was determined by the radial alveolar count (RAC) (Emery and Mithal, 1960). Both parameters were reduced compared with those of normal controls in diaphragmatic hernia, anencephalus, anuric renal anomalies, chondrodystrophies, and osteogenesis inperfecta. Comparison of LW:BW ratio and RAC indicated that the RAC was the more reliable criterion of PH, LW:BW ratio of .0-012 (67% of mean nor- mal ratio) and/or RAC of < 4.1 (75 % of mean normal count) are suggested as diagnostic criteria of PH. Evidence ofPH was incidentally discovered in a number ofclinically unsuspected cases and retro- spectively clarified the clinical and radiological findings. Routine assessment of lung growth should be an essential part of the neonatal necropsy. Pulmonary hypoplasia (PH) is a poorly defined establish. There has been only one study of both copyright. condition considered to be almost invariably LW:BW ratio and morphometry in a substantial secondary to other anomalies and is usually diag- number of pathological cases (Reale and Esterly, nosed in association with them; primary or isolated 1973). PH has not been reported (Reale and Esterly, 1973).
    [Show full text]
  • Non-Invasive Diagnosis of Tracheobronchomalacia Using a Modified Ventilation Radioisotope Lung Scan a Gour, M J Peters, I Gordon, a J Petros
    1122 CASE REPORT Arch Dis Child: first published as 10.1136/adc.88.12.1122 on 11 December 2003. Downloaded from Non-invasive diagnosis of tracheobronchomalacia using a modified ventilation radioisotope lung scan A Gour, M J Peters, I Gordon, A J Petros ............................................................................................................................... Arch Dis Child 2003;88:1122–1123 incremental levels of peak end expiratory pressures (PEEP) The use of radionuclide ventilation lung scan to characterise was performed to determine the opening pressures of any the physiological effects of tracheobronchomalacia is a novel malacic segments detected. The TBG confirmed the presence application of this non-invasive technique. In the reported of TBM with opening pressures at the trachea of 10 cm H2O case the right upper lobe was found to be not ventilated and for both bronchi of 10–15 cm H2O (fig 2). below a pressure of 20 cm H2O despite evidence from a A diagnostic isotope ventilation lung scan using krypton dynamic tracheobronchogram of the right upper lobe (Kr81m) scan was performed to assess the degree of left bronchus opening at the lower pressure of 15 cm H2O. pulmonary hypoplasia. This revealed no isotope present on the ventilation scan in the area of the right upper lobe. Only when a PEEP of 15 cm H2O was applied did the right upper lobe region become poorly ventilated. Isotope intensity was racheobronchomalacia (TBM) is an important cause of only equal to the left upper lobe area when a PEEP of morbidity resulting in prolonged stay on the paediatric 20 cm H2O was applied. This localised segment of severe Tintensive care unit (PICU).
    [Show full text]
  • EUROCAT Syndrome Guide
    JRC - Central Registry european surveillance of congenital anomalies EUROCAT Syndrome Guide Definition and Coding of Syndromes Version July 2017 Revised in 2016 by Ingeborg Barisic, approved by the Coding & Classification Committee in 2017: Ester Garne, Diana Wellesley, David Tucker, Jorieke Bergman and Ingeborg Barisic Revised 2008 by Ingeborg Barisic, Helen Dolk and Ester Garne and discussed and approved by the Coding & Classification Committee 2008: Elisa Calzolari, Diana Wellesley, David Tucker, Ingeborg Barisic, Ester Garne The list of syndromes contained in the previous EUROCAT “Guide to the Coding of Eponyms and Syndromes” (Josephine Weatherall, 1979) was revised by Ingeborg Barisic, Helen Dolk, Ester Garne, Claude Stoll and Diana Wellesley at a meeting in London in November 2003. Approved by the members EUROCAT Coding & Classification Committee 2004: Ingeborg Barisic, Elisa Calzolari, Ester Garne, Annukka Ritvanen, Claude Stoll, Diana Wellesley 1 TABLE OF CONTENTS Introduction and Definitions 6 Coding Notes and Explanation of Guide 10 List of conditions to be coded in the syndrome field 13 List of conditions which should not be coded as syndromes 14 Syndromes – monogenic or unknown etiology Aarskog syndrome 18 Acrocephalopolysyndactyly (all types) 19 Alagille syndrome 20 Alport syndrome 21 Angelman syndrome 22 Aniridia-Wilms tumor syndrome, WAGR 23 Apert syndrome 24 Bardet-Biedl syndrome 25 Beckwith-Wiedemann syndrome (EMG syndrome) 26 Blepharophimosis-ptosis syndrome 28 Branchiootorenal syndrome (Melnick-Fraser syndrome) 29 CHARGE
    [Show full text]
  • Congenital Acinar Dysplasia: Report of a Case and Review of Literature
    9 Congenital Acinar Dysplasia: Report of a Case and Review of Literature Mary Langenstroer, MD 1 S.J. Carlan, MD 1 Na’im Fanaian, MD 2 Suzanna Attia, MD 3 1 Department of Obstetrics and Gynecology, Winnie Palmer Hospital, Address for correspondence S.J. Carlan, MD, 105 West Miller St., Orlando Regional Healthcare, Orlando, Florida Orlando, FL 32806 (e-mail: [email protected]). 2 Department of Pathology, Orlando Regional Healthcare, Orlando, Florida 3 Department of Pediatrics, Orlando Regional Healthcare, Orlando, Florida Am J Perinatol Rep 2013;3:9–12. Abstract Objective Describe a case of congenital acinar dysplasia and review the literature. Study Design Retrospective chart review and literature search. Results Congenital acinar dysplasia is a rare malformation of growth arrest of the lower respiratory tract resulting in critical respiratory insufficiency at birth. It is a form of pulmonary hypoplasia that is characterized by diffuse maldevelopment and derange- ment of the acinar and alveolar architecture of the lungs, resulting in the complete absence of gas exchanging units. The growth-arrested lung tissue resembles the Keywords pseudoglandular phase of 16 weeks’ gestation. The etiology is unknown. It is diagnosed ► pulmonary hypoplasia by exclusion of all other causes of pulmonary hypoplasia and a summation of clinical, ► respiratory imaging, and histopathologic findings. insufficiency Conclusion There is no cure and clinical treatment is supportive until death of the ► lung maldevelopment infant. We present a case of congenital
    [Show full text]
  • Perinatal/Neonatal Case Presentation
    Perinatal/Neonatal Case Presentation Primary Unilateral Pulmonary Hypoplasia: Neonate through Early Childhood F Case Report, Radiographic Diagnosis and Review of the Literature Matthew E. Abrams, MD (Figure 1) and a retrosternal opacity on lateral view (Figure 2). Veda L. Ackerman, MD Serial radiographs failed to show improvement in the appearance William A. Engle, MD of the right hemithorax. However, the patient’s respiratory status normalized. Computed topography (CT) scan of the chest (Figure 3) at the infant’s referring hospital was interpreted as complete collapse of the right upper lobe versus a right upper lobe Unilateral pulmonary hypoplasia is a rare cause of respiratory distress in the chest mass. The infant was transferred to our institution at 9 days neonate. It is usually secondary to other causes such as diaphragmatic of life for further evaluation. At this time, the infant was symptom hernia. We present a case of a newborn with primary hypoplasia of the right free without tachypnea, cyanosis, or cough. The infant otherwise upper lobe who was later found to also have tracheobronchomalacia. We appeared normal without any dysmorphic features. Review of the describe the clinical course through early childhood. chest radiograph series and chest CT scan confirmed the diagnosis Journal of Perinatology (2004) 24, 667–670. doi:10.1038/sj.jp.7211156 of unilateral right upper lobe pulmonary hypoplasia without any evidence for a chest mass. A magnetic resonance image (MRI) of the chest (Figure 4) was consistent with hypoplasia of the right upper lobe. An assessment of pulmonary venous drainage was not possible secondary to technical problems.
    [Show full text]