What Lies Beneath? Skin Nodules, Lumps & Bumps

Renee Howard, MD Professor of Dermatology, UCSF Director of Dermatology UCSF Benioff Children’s Hospital Oakland Renee Howard MD

DISCLOSURES I have no relevant relationships with industry Objectives  Learn efficient approach to diagnosis and treatment  Outline differential diagnosis of skin nodules at “high risk hotspots”: nose/brow, scalp, lower back, 5 Facts to Know and Tell

 Who = Other anomalies or systemic issues  What = Morphology  Vascular stain, pigmentary change, pit, aplasia cutis, nodule, mass  When=Congenital, at what age acquired  Where= Hotspots

 How ➡️ high risk or low risk for deep extension How? Management

 Low risk  Observation  High Risk  Imaging  Referral  Neurosurgery  ENT  Plastic Surgery  Pediatric Surgery Where? Developmental hotspots = Embryological fusion

From Kos and Drolet. Neonatal Dermatology 2nd Edition 10 month-old with subtle lump nasal bridge, slowing increasing in size Nasal Midline

 Dermoid cyst/sinus, glioma (5-7%), encephalocele  High risk CNS extension  Common embryologic origin Dermoid Cysts

 Entrapment of surface ectoderm  Midline or over suture lines higher risk  Can rupture, become inflamed or infected, erode bone  Nasal Dermoid rare

J Neurosurg Pediatr. 2017 Jul;20(1):30-34. International Journal of Pediatric Otorhinolaryngology 2015 79, 18-22 102 cases 58 superficial 38 intraosseous 10 intracranial

International Journal of Pediatric Otorhinolaryngology 2015 79, 18-22DOI: (10.1016/j.ijporl.2014.10.020)

Copyright © 2014 Elsevier Ireland Ltd Terms and Conditions Nasal Dermal Sinus Tract (DST)

 Congenital midline pit  Tuft of hair  Oily or clear discharge (CSF!)  10-30% extend deep +/- cyst anywhere along the tract  Complications infectious or aseptic meningitis Nasal Midline Checklist

 Who: healthy newborn or toddler  What: pit, tuft of hair, nodule  When: sinus tract congenital, cyst first few years of life  Where: midline glabella to nasal tip  How  Refer to: ENT + Neurosurgeon  Imaging-MRI with contrast for soft tissue, CT for bony defects prn Lateral Brow Most common location of dermoid cysts

60-68% of craniofacial dermoids on lateral brow Lateral Brow Dermoid Cyst

 Who: healthy infant or toddler  What: slowly growing nodule  When: first few years of life  Where: lateral 1/3 eyebrow  How  No imaging needed as no extension  Observation  ENT or plastic surgeon for endoscopic or simple excision

J Laparoendosc Adv Surg Tech A. 2018 May;28(5):617-621. Developmental hotspots = Embryological fusion lines

From Kos and Drolet. Neonatal Dermatology 2nd Edition Imagine these 2 kids come in…

 There are two patients in two rooms  One requires quick reassurance--low risk  The other needs imaging and/or surgical referral--high risk  Low risk: active nonintervention  Off midline  Soft slow growing nodule  Mobile

 High risk: imaging/refer  Midline membranous aplasia cutis congenita  Hair collar Which scalp lesion is high risk?

 High risk: imaging/refer  Midline membranous aplasia cutis  Hair collar

 Low risk: active nonintervention  Scar like  No other skin or hair changes

Dias et al. Pediatrics 2015; 136:e1105-1119 Scalp Aplasia Cutis Congenita (ACC)

 86% of ACC located on scalp  Most sporadic though can be syndromic  Presents at birth as erosion or scar like area with alopecia  Hair collar sign high risk

Journal of Perinatology (2018) 38, 110–117. Hair Collar Sign

Bessis et al J Am Acad Dermatol 2017;76:478-487. Scalp ACC: When to MRI

 Midline  Membranous  Hair collar YES YES  Vascular stain

YES Which scalp nodule is high risk? Hemangioma vs. Dermoid Cyst

-Natural history, skin changes, firmness -Ultrasound or refer -Active nonintervention Scalp Dermoid Cyst

 2nd most common location after lateral brow (9%)  Presents < 4 years, 40% congenital  Usually asymptomatic, firm, fixed to underlying bone  Slowly enlarge then stabilize

Plast Reconstr Surg. 2020 Jan 9 Pediatr Dermatol 2013; 30:706-11. School age boy with hx of lump and alopecia for years Scalp Dermoid Cyst

 Retrospective studies by neurosurgeons  33-46% eroded or within bone  Higher rate in patients older at time of surgery  Time of surgery 33% <1 year, 49% 1-3 years; all did well  Neurosurgeons: refer for excision at time of presentation World Neurosurg. 2018;120:119-124. J Neurosurg Pediatr. 2017;20:30-34. “Tip of the Iceberg” phenomenon

Dias et al. Pediatrics 2015; 136:e1105-1119 Scalp Dermal Sinus Tracts (DST)

 Located parietooccipital  With or without associated cyst  Intracranial extension through midline occipital skull defect into dura

Dias et al. Pediatrics 2015; 136:e1105-1119 Scalp: ACC, Pit, Nodule

 High risk  Low risk  Present at birth  Later onset  Midline, especially vertex, occipital  Off midline  Nodule, membranous ACC, pit  Soft mobile nodule, scar -like ACC  Multiple signs  Other signs  Hair collar  Focal hemangioma, hyperpigmentation  Vascular stain

Pediatr Dermatol. 2018 Jan;35(1):e59-e61. Dias et al. Pediatrics 2015; 136:e1105-1119 8 month old with 2 skin lesions Hotspot: Lumbosacral Spine

 Pits  Aplasia cutis  Nodules and subcutaneous masses  Vascular stains  Segmental hemangiomas  Hypertrichosis Consequences of Undiagnosed LS anomalies

 Infection + vascular stain, mass  Aseptic meningitis Atretic cephalocele  Spinal cord compression  Tethered cord

Dias et al Pediatrics 2015 Oct;136:e1105-19. . Lumbosacral lipoma with intraspinal lipoma and tethered cord

Photos courtesy of Ilona Frieden MD Hypertrichosis

Dias et al Pediatrics 2015 Oct;136:e1105-19. . Dermal sinus tracts Innocent coccygeal pit in presenting as a pit intergluteal cleft

Pediatrics 2015 Oct;136:e1105-19. Dimple Dilemma

 Common, in 2-4% of newborns  Blind-ended dimple/pit not necessarily innocent  Location of the dimple along the craniocaudal axis?  Flat part of at S2 greatest risk  Neurosurgeon’s decision to treat based on presence of a pathologic dimple, regardless of imaging  IF UNSURE THEN REFER

Pediatrics 2015:136:e1105-19. J Neurosurg Pediatr. 2017 Feb;19(2):217-226. Ultrasound Screening Skin Signs OSD

 2.2-7.2% newborns w/skin signs  .5% have operable spinal lesion  Prospective study 475 newborns  Ultrasound abnormal in 39  MRI confirmed OSD in 12  High risk > 1 skin sign  Highest risk dermal sinus  Lowest simple sacral dimple  1.2% required neurosurgery

Ausili E, et al Childs Nerv Syst. 2018 Feb;34(2):285-291. MRI for Occult Spinal Dysraphism (OSD)

 522 patients screened due to cutaneous stigmata over 6 years  Average age 6 months  OSD found in 23%  OSD in 20% of those with dimples  In this series, dimple location did not correlated with OSD

J Neurosurg Pediatr. 2017 Feb;19:217-226. Cutaneous Signs LS Spine  High risk  Low risk  Midline mass, membranous ACC  Off midline  Thick tuft of terminal hair  Diffuse fine hair  Pit over sacrum  Pit low in intergluteal crease (?)  Vascular stains +  Pigment changes, nevus, small  Tail hemangioma  Segmental hemangioma  Isolated deviation crease Checklist for LS Spine

 Who: other GU, GI, midline defects  What: pit, ACC, nodule, vascular stain, tail, hair tuft, segmental hemangioma  When: at birth  Where: midline, above gluteal crease  How: ultrasound if first six months, MRI and… How

Refer to neurosurgery if any chance high risk REGARDLESS of imaging

←Read this 11 year old with slowly enlarging neck nodule

PILOMATRIXOMA Thyroglossal, Branchial Cleft

➡️ ➡️ ➡️

http://www.ghorayeb.com/branchialcleft.html OtolarynologyCurr Opin Otolaryngol Head Neck Surg. 2012 Dec;20:533-9. Neck: Developmental Anomalies

 Cartilaginous rest/wattle  Midline anterior neck inclusion cyst (MANIAC)  Thyroglossal duct cyst  Branchial cleft cyst  Dermoid cyst  Midline cervical cleft  Thymic cyst  Bronchogenic cyst

Figure 9-2 Neonatal Dermatology, 2nd edition Neck: Pits, Tags, Nodules

 High risk  Low risk  Present at birth  Later onset  Midline or just anterior to SCM  Off midline, not anterior to SCM  Nodule, pits, especially with  Firm blue plate like nodule or milia discharge like superficial cyst even if midline

Figure 1

Walsh R Pediatr Dermatol 2018 Jan;35(1):55. What Lies Beneath Remember Importance of “Where”:

Illustration Textbook of Neonatal and Infant Dermatology, 3rd Edition Photos Dias et al. Pediatrics 2015;136:e1105-e1119 Many thanks to Dr. Betts and

 UCSF Benioff Children’s Hospital Oakland pedi derm team  Pediatric trainees & colleagues  UCSF Pediatric dermatology team  Patients and families

Photo courtesy of Ann Petru Dr. Nicole Kittler, me, Anjali Washington PA-C Sport-Related Concussion in Pediatrics

Celina de Borja, MD Pediatric Musculoskeletal and Sports Medicine Assistant Clinical Professor Division of Pediatric Orthopaedics UCSF Benioff Children’s Hospitals Disclosures

. I have no relevant financial relationships with the manufacturer(s) of any commercial product(s) and/or provider(s) of commercial services discussed in this CME activity . I do not intend to discuss an unapproved/investigative use of a commercial product/device in my presentation

2 Learning Objectives

. Identify, diagnose and evaluate concussions

. Discuss main principles of concussion management

. Tackle common questions that parents raise regarding concussions

3 Case 1

4 Ava“is a 15 year old female cheerleader with a chief complaint of headaches for 5 days. It all started after she got kicked in the head during practice. She denies LOC, but reports headaches and balance issues immediately after the injury. She didn’t tell anyone and finished the last 10 minutes of practice. On the way home, she experienced light sensitivity from the cars on the freeway, which was followed by difficulty concentrating when she tried to finish her homework that evening. She feels fatigued during the day but has difficulty falling asleep at night. Mom reports that she has been more irritable than usual. Physical examination in the office reveals no evidence of focal neurologic deficits.

What is going on? Can this be a concussion? Does she need imaging?

5 Sport-Related Concussion Definition

. “A traumatic brain injury induced by biomechanical forces.” - Either direct blow to the head or indirect force that is transmitted to the head - Rapid onset and often short lived impaired neurologic function that resolves spontaneously, but may evolve over minutes to hours - Functional disturbance rather than structural injury - Results in a range of clinical signs and symptoms (± LOC) - Diagnosis of exclusion - not explained by drug, alcohol, or medication use or other comorbidities (psychological factors or medical conditions)

• Halstead ME, Walter KD, Moffatt K. Sport-Related Concussion in Children and Adolescents. Pediatrics. 2018; 142(6):e20183074 • McCory, P et al. Br J Sports Med 2018.

6 Identify

. Top 3 sports with highest risk: - Boys: Tackle football, lacrosse, ice hockey - Girls: Soccer, lacrosse, field hockey - Also reported in volleyball, cheerleading, gymnastics, track, swimming/diving, etc.

. Female athletes are more likely to report concussive symptoms compared to their male counterparts

. Incidence is higher in competition than in practice except in cheerleading

• Halstead ME, Walter KD, Moffatt K. Sport-Related Concussion in Children and Adolescents. Pediatrics. 2018; 142(6):e20183074

7 Diagnose . Does Ava have a concussion? Yes - Meets clinical criteria  Direct blow to the head  Rapid onset/evolves over time  Range of clinical signs and symptoms

. Clinical tools: - Post Concussion Symptom Scale (PCSS) is useful in tracking recovery https://www.cdc.gov/headsup/providers/tools.html - Consider SCAT 5 immediately after the injury https://bjsm.bmj.com/content/bjsports/early/2017/04 /26/bjsports-2017-097506SCAT5.full.pdf “When in doubt, sit them out”

8 Evaluate

. Does Ava need imaging? No - Concussion symptoms reflect a functional disturbance rather than structural injury . No abnormality is seen on standard neuroimaging studies

- Emergency department head CT use for concussions increased despite a decrease in reported injury severity and conventional neuroimaging often yielding normal results . Providers with variable pediatric experience . Parental imaging expectations

• Halstead ME, Walter KD, Moffatt K. Sport-Related Concussion in Children and Adolescents. Pediatrics. 2018; 142(6):e20183074 • Jennings RM, Burtner JJ, Pellicer JF, et al. Reducing Head CT Use for Children With Head Injuries in a Community Emergency Department. Pediatrics. 2017;139(4):e20161349

9 Evaluate

. Indication for imaging: Suspicion of more severe intracranial injury or structural lesion (eg. Hemorrhage or skull fracture)

 Severe headaches  Significant mental status  Seizures impairment  Focal neurologic deficits  Repeated emesis  LOC > 30 seconds  Significant irritability  Worsening symptoms

. CT scan unlikely to be helpful after 6 hours without deterioration of level of consciousness

• Halstead ME, Walter KD, Moffatt K. Sport-Related Concussion in Children and Adolescents. Pediatrics. 2018; 142(6):e20183074 • Kuppermann, N, et. al. Identification of children at very low risk of clinically-important brain injuries after head trauma: a prospective cohort study. Lancet. 2009; 374:1160-70.

10 Case 2

11 “ Johnny is a 17 year old male soccer athlete who comes in for follow up after being diagnosed with a concussion on Friday evening. He rested at home all weekend and reports that he is feeling somewhat, but not all the way better.

Dad: When can he return to sports? Mom: Should we keep him out of school until he gets better? He’s always on his phone – is that harmful for his recovery?

12 Management

. Initial phase - REST (Cognitive and physical) . Limit to 24 - 48 hours . Be careful with “rest overdose” . Prolonged rest is associated with longer recovery

• Thomas DG, et al. Benefits of strict rest after acute concussion: a randomized controlled trial. Pediatrics 2015

13 Management

. Three central principles 1. Prevent new head injury 2. Minimize school interruption 3. Prevent deconditioning (physical, social/psychological)

14 Management

1. Prevent new head injury - Restrict from all contact/collision sports and high risk activities until medically cleared

Dad: When can he return to sports? When he is medically cleared

http://leginfo.legislature.ca.gov/faces/codes_displaySection.xhtml?sectionNum=124235.&lawCode=HSC

15 Management 2. Minimize school interruption (Academic accommodations)  Shorten school day  Reduce workload  Provide print outs (limit screen time)  Allow extended time to complete tasks  Allow rest breaks  Postpone tests

- Athletes who did not reduce their cognitive load at all took longest to recover

Mom: Should we keep him out of school until he gets better? No • Brown NJ, Mannix RC, O'Brien MJ, et al. Effect of cognitive activity level on duration of post-concussion symptoms. Pediatrics 2014

16 Management

3. Prevent deconditioning (Physical) - Growing body of evidence supporting benefits of physical activity - Improves symptoms, speeds up recovery and restores function - Not harmful

• Leddy JJ, et al. Exercise is medicine for concussion. Curr Sports Med Rep 2018. • Leddy JJ, et al. A preliminary study of subsymptom threshold exercise training for refractory post- concussion syndrome. Clin J Sport Med 2010. • Gagnon I, et al. (2009). Active rehabilitation for children who are slow to recover following sport- related concussion, Brain injury 23:12, 956-964. • Baker et al. (2012). Return to full functioning after graded exercise assessment and progressive exercise treatment of post concussion syndrome, rehabilitation research and practice, vol 2012. McCory, P et al. Br J Sports Med 2018.

17 Management

3. Prevent deconditioning (Social/Psychological) - Discourage blanket recommendations completely avoiding use of electronic devices . May only result in social isolation, depressive and anxious symptoms

Parents: Should we take his gadgets away? No

• Halstead ME, Walter KD, Moffatt K. Sport-Related Concussion in Children and Adolescents. Pediatrics. 2018; 142(6):e20183074

18 Return to Play Criteria

Majority of pediatric athletes will take 4 weeks to fully recover

 Complete resolution of symptoms  Successfully returned to full academics  Completed graduated return to sport protocol

• Halstead ME, Walter KD, Moffatt K. Sport-Related Concussion in Children and Adolescents. Pediatrics. 2018; 142(6):e20183074 • McCory, P et al. Br J Sports Med 2018.

19 Return to Play

. Computerized neurocognitive tests - Validity and reliability are currently being questioned - May be helpful in overall assessment (compare baseline) - Should not be used as sole determining factor in RTP decisions

• Halstead ME, Walter KD, Moffatt K. Sport-Related Concussion in Children and Adolescents. Pediatrics. 2018; 142(6):e20183074

20 Prolonged Symptoms

. Consider rehabilitation for: - Cervical strain - Vestibular injuries - Oculomotor disorders - Sleep cycle disturbance - Developing anxiety/depression/attention deficit

• Halstead ME, Walter KD, Moffatt K. Sport-Related Concussion in Children and Adolescents. Pediatrics. 2018; 142(6):e20183074

21 “ Johnny fulfilled his return to play criteria and is ready to return to sports. He is looking forward to participating in football this coming season.

What can we do to prevent this from happening again? Do you recommend specific equipment or supplements?

22 Prevention

. Education/ Awareness . Mouth guards/ Helmets . Game rule changes - Helps with maxillofacial/dental trauma . Neck strengthening - Reduce severe injury (skull fractures, intracranial hemorrhage) - Does NOT prevent or lessen severity of concussion . Biomarkers/ Supplements - No evidence to support

• Halstead ME, Walter KD, Moffatt K. Sport-Related Concussion in Children and Adolescents. Pediatrics. 2018; 142(6):e20183074

23 24 25 26 27 California State Laws

. AB 25 Concussion Law 2012 - 3 parts (education, remove from play, written medical note to return) . AB 1451 Coaches Concussion Training Law 2013 - Mandatory education every 2 years . AB 2127 Concussion Safety Law 2015 - Limit FB full-contact practices - Mandatory RTP protocol of no less than 7 days from the diagnosed date of concussion - RTP under the supervision of LHCP . AB 2007 Concussion Mgmt in Youth Sports Act 2016 - Requires youth sports participants to undergo the same safety protocols as high school athletes

28  Concussion Information Sheet  Acute Concussion Notification Form  Graded Concussion Symptom Checklist  Physician Letter to School After Concussion Visit  Concussion Return to Learn (RTL) Protocol  Physician Recommended School Accommodations Following Concussion  Concussion Return to Play (RTP) Protocol

http://cifstate.org/sports-medicine/concussions/index

29 Thank You!

[email protected]

UCSF Benioff Children’s Hospital 1825 4th St, San Francisco, CA 94158 (415) 353-2967

UCSF Benioff Children’s Hospital Oakland 747 52nd St, Oakland, CA 94609 (510) 428-3238 Chronic Traumatic Encephalopathy (CTE)

. Case series of 202 football players whose brains were donated for research . Mean 15.1 years of football participation . CTE was neuropathologically diagnosed in 177 (87%) players - 3/14 (21%) high school - 48/53 (91%) college - 9/14 (64%) semi professional - 7/8 (88%) Canadian football league - 110/111 (99%) NFL . Conclusion: CTE may be related to prior participation in football • Mez J, Daneshvar DH, Kiernan PT, et al. Clinicopathological Evaluation of Chronic Traumatic Encephalopathy in Players of American Football. JAMA. 2017;318(4):360–370. doi:10.1001/jama.2017.8334

31 Research Gaps and Controversies in CTE

. Selection bias . No definite criteria for defining clinical syndrome . Absence of in vivo biomarkers . Confounding variables: premorbid and comorbid conditions that influence normal or expected cognitive functioning

• Asken BM, Sullan MJ, DeKosky ST, Jaffee MS, Bauer RM. Research Gaps and Controversies in Chronic Traumatic Encephalopathy: A Review. JAMA Neurol. 2017;74(10):1255– 1262. doi:10.1001/jamaneurol.2017.2396

32 33 Pediatric Airway Basics

Claire Hargrove, MD Attending Anesthesiologist UCSF Benioff Children’s Hospital Oakland “Children are not small adults”

• The pediatric airway differs significantly from the adult airway

• The pediatric airway begins to approximate the adult airway at about age 8

• Pediatric physiology leads to more rapid desaturation during apnea Airway. Airway. Airway.

• Number one priority is maintaining oxygen delivery to tissues

• Number one requirement is a patent airway

• Cardiac arrest in children is rare but usually due to hypoxia secondary to respiratory failure

• Rescuing an airway and restoring oxygenation can literally bring a child back to life Major anatomic differences

• Occiput

• Tongue

• Larynx

• Epiglottis

• Subglottis

• Airway caliber

• Relatively large occiput tends to flex and occlude infant airway

• A shoulder roll can help open the airway

Larynx • The cricoid cartilage is the narrowest part of the pediatric airway

• A tube that passes easily through the vocal cords (glottic opening) can be tight at the level of the cricoid cartilage (subglottis)

• Pressure on the subglottic mucosa can lead to injury and scarring (subglottic stenosis) Epiglottis Airway caliber

• Smaller airways mean greater resistance • Small changes in airway diameter mean large changes in resistance (Poiseuille’s Law) Ventilation strategies

• Natural airway (+/- supplemental O2)

• Optimize positioning

• Oral/nasal airway

• Bag-mask ventilation

• LMA (laryngeal mask airway)

• Endotracheal tube

• Surgical airway Open airway by lifting tongue away from posterior pharynx

Oral Airway (Oropharyngeal airway; OPA; Guedel airway)

* not well-tolerated in awake patients Nasal airway (Nasopharyngeal airway; nasal trumpet)

* more comfortable in an awake patient Mask ventilation Mask ventilation

• Can be challenging but is extremely important!

• Don’t forget about oral and nasal airways

• May require 2 people—one to hold mask and give jaw thrust, one to squeeze bag Mask ventilation, infant LMA

• Easy to place

• Fairly comfortable for patient

• Not a protected airway

• Should be considered in “Can not ventilate, can not intubate” situation

LMA positioning Endotracheal tube (ETT)

• Traditionally uncuffed ETT were used for children; now almost exclusively cuffed ETT, except for preemies and neonates

• Ideally should have an air leak with cuff down

• Depth is approx 3x/ETT size, but check for bilateral breath sounds ETT sizing

• Preemie 3.0 uncuffed

• Newborn 3.5 uncuffed or 3.0 cuffed

• 6 month-old 3.5 cuffed

• 12-month old 4.0 cuffed

Then Age/4 + 4 and subtract half size for cuffed tube Infant ETT Direct laryngoscope • Miller blade lifts the epiglottis

• Macintosh blade sits in the vallecula in front of the epiglottis Keep it simple:

• Birth-12 months: Miller 1

• 1 y.o to 5 y.o: Mac 2

• Everyone else: Mac 3 Video Laryngoscope

Glide scope Video laryngoscope

C-MAC Last but not least:

• Always confirm air exchange

Chest rise and fall

Condensation in mask or ETT

Auscultation

Capnography

• Always have a backup plan

• Don’t panic! Questions??

Claire Hargrove, MD [email protected] Pediatric Circulation & Shock

v. 1.6b 3-6-2020

Mandeep Singh Chadha, M.D. Pediatric Intensivist Assumptions

• Review of fundamental principles of sepsis, cardiovascular physiology and pharmacology is useful for the early responder, ED/Trauma personnel, and inpatient providers – Practical bedside application – Improve communication among MDs, RNs and other staff Outline • The meaning of life • Shock (and sepsis) • Drugs • Scenarios

The meaning of life

• O2 delivery (DO2) = O2 consumption (VO2) O2 delivery

DO2 = CO • CaO2 CO = HR • SV

CaO2 = (1.34•Hg•SaO2)+(0.003•PaO2)

DO2 = HR•SV•[(1.34•Hg•SaO2)+(0.003•PaOHR•SV•[(1.34•Hg•SaO2)+(0.003•PaO2)]

Preload Contractility Afterload “CO”

“Preload” O2 consumption

• Consciousness • Movement • Breathing • Circulation • Digestion and substrate utilization • Temperature control • Tissue repair • Immune function

Shock

• Unbalanced O2 delivery and consumption – Perfusion variations (pressure and flow) affect baroreceptors in carotid body, aortic arch and kidneys – Changes in NaCl filtration affect renal macula densa – Oxygen variations and cellular acidosis affect peripheral chemoreceptors and vascular smooth muscle – Cellular hypoxia affects gene expression • Compensated shock – Increased ventilation and cardiac output • Increased sympathetic tone • Renin angiotensin activation • Humoral responses • Autotransfusion – Switch from aerobic to anaerobic Organmetabolism function is preserved, – Improvedbut if vascularization left untreated… – Enhanced oxygen carrying capacity • Uncompensated – Organ function deteriorates (MOD and MOF) – Hypotension • Irreversible – Apoptosis – Necrosis Types of shock

• Hypovolemic – Hemorrhage – Dehydration • Distributive – Anaphylaxis – Neurogenic – Toxic – Inflammatory • Septic • Cardiogenic – Myocardial ischemia – Cardiomyopathy • Dilated • Hypertrophic – Dysrhythmia – Septic – Traumatic – Congestive heart failure – Ductal dependent CHD • Obstructive – Pneumothorax/hemothorax – Tamponade – Dissection – Outflow tract obstructiuon • Dissociative – Heat – CO – Cyanide – Endocrine Special cases

• Septic shock – Warm (distributive) – Cold (cardiogenic) • Neonatal shock – Congenital adrenal hyperplasia – Inborn error of metabolism – Obstructive left sided lesion • Traumatic shock – Hemorrhagic – Neurogenic – Cardiogenic – Obstructive Children are not small adults

• Less myocardial mass = smaller SV • Lower calcium stores • Lower catecholamine stores • Inability to fully compensate by increasing HR Evidence for shock

• Physical exam – Cool extremities – Pallor – Decreased pulses – Altered mental status – Delayed capillary refill

Evidence for shock (VS)

• Tachycardia • Hyperpnea • Pulse pressure

• SaO2 – Unreliable in low perfusion state • Noninvasive cuff versus invasive arterial BP monitoring? • Shock index (SIPA)? – HR/SBP • Decreased UOP Laboratory evaluation

• Acid base disturbance – Base deficit? • Lactate • Central venous saturation • Organ function – LFTs – Coagulopathy • Tissue oximetry • Thromboelastography (TEG) Imaging

• CXR • Focused abdominal sonography for trauma (FAST) • CT – Most useful in Trauma? • Echo OrganA note on sepsisfunction terminology based (Goldstein criteria? et al 2005) Does it matter?

• Shock is uncommon? • PICU mortality is very low? – Not for septic shock – Not necessarily for patients with initial high or rising SIPA • “The Golden Hour” – Odds of mortality doubles for every hour in shock (Han et al 2003) • Adherence to guidelines makes a difference in kids (Han et al 2003) What do we do about it?

• Treat underlying disease process – Diagnostic tests (e.g. cultures) – Prompt antibiotics – Surgical intervention • Improve oxygen delivery • Reduce metabolic demands • Consensus guidelines for septic shock

Start with basics

DO = HR•SV•[(1.34•Hg•SaO2)+(0.003•PaO )] • Airway 2 2 Preload Contractility Afterload – Position – Oral or NP airway – Intubate? • Drugs? • Cardiopulmonary interactions? • Breathing

– 100% O2? – Noninvasive versus invasive ventilation? Positive pressure ventilation

• Reduces systemic ventricular strain and improves work, reduces afterload • May reduce preload and stroke volume • May reduce pulmonary blood flow (and possibly stroke volume) in patients with Glenn or Fontan physiology

Circulation

• What, how and where are you measuring? • Fluid resuscitation • Vascular access • Drugs Non-invasive blood pressure

• Manual – Artery compressed on bone by air-filled cuff – Auscultation of Korotkoff sounds – Read pressure from manometer • Automated (e.g. GE Dinamap) – Pressure transducer measures amplitude of pressure oscillations – Rapid rise of oscillations = SBP – Point of maximum oscillation = MAP (most reliable measurement) – DBP calculated NIBP errors

• Wrong cuff size • Wrong height • DBP calculated (spuriously low) • Edema • Irregular pulse • Arterial stenosis

Fluid resuscitation

• Route • Amount and speed – Cardiac compliance – Valvar insufficiency • Composition – Crystalloid vs colloid vs other – Expiring blood products?

DO2 = HR•SV•[(1.34•Hg•SaO2)+(0.003•PaO2)]

Preload Contractility Afterload Vascular Access

• PIV versus CVL – Consider lumen size, length and resistance – Maximum flow rates are a function of number of lumens, inner diameter, length, and fluid viscosity – Risk of extravasation injury? • Carrier flow rates? • Proximity to heart? • Cardiac lines? – Risk of thromboembolism with common atrium or other right to left shunt 5Fr 1.7mm

4Fr 1.3mm

3Fr 1.0mm

Infants Mild 5% Moderate 10% Severe 15% (50 ml/kg) (100 ml/kg) (150 ml/kg) Enough fluid? • Physical exam • Data – Skin turgor – Weight – Mucous membranes – Vital signs – Urine volume – Capillary refill – Urine SG – Liver – Total fluid balance (Epic) – Gallop – CVP? ? – Crackles – Wedge pressure? – Edema – Mixed venous saturation? – Pulse contour analysis? – Pulsus paradoxus? – Restoration of BP and perfusion after bolus? Straight leg raise? Intravascular vs extravascular? Pulsus paradoxus Trauma considerations

• Risks of aggressive fluid resuscitation • Hypertension • Hemodilution • Where’s the bleeding? – Imaging versus surgery – Intraperitoneal versus retroperitoneal • How do I stop the bleeding? – Compression versus tourniquet – Massive transfusion protocol (MTP) – Antifibrinolytic therapy (e.g. TXA)

Cardiovascular drugs

• Inotropes increase contractility/SV • Pressors increase SVR • Chronotropes increase HR • Lusitropes increase diastolic filling resulting in increased contractility/SV • Other! Adrenergic agents

• Dopamine • Epinephrine • Norepinephrine

Signal transduction of cardiac myocyte contraction and its regulation by cardiac adrenergic receptors (ARs; see text for details).

• Neonates • Blood products • Low Mg, high Phos

Anastasios Lymperopoulos et al. Circ Res. 2013;113:739- 753

Copyright © American Heart Association, Inc. All rights reserved. Dopamine

• At low doses (1–5 mcg/kg/min), stimulates dopaminergic receptors, increases renal blood flow • At moderate doses (5–10), stimulates release of endogenous catecholamines and metabolized

• At high doses, principally α1 agent • Typically starts at 5 mcg/kg/min, titrated 1–5 at a time

DO2 = HR•SV•[(1.34•Hg•SaO2)+(0.003•PaO2)]

Preload Contractility Afterload • Increases CO, SVR, HR • Does NOT appear to be a selective renal vasodilator • Toxicity includes functional immunosuppression, tachycardia, tachyarrythmias, thyroid dysfunction, prolactin inhibition Evidence

• First line therapy? • Independent risk factor for mortality in adults in septic shock? • Dopamine associated with lymphocyte depletion and worse outcome in pediatric septic shock and MOF (Felmet et al 2005) Epinephrine

• At low doses (0.01–0.1 mcg/kg/min),

stimulates β1/2 >> α1

• At moderate doses (0.1–0.2), stimulates β1/2 = α1 • At high doses, (0.2–1 mcg/kg/min), stimulates

α1 >> β1/2 • Typically starts at 0.02 mcg/kg/min, titrated 0.01 at a time

DO2 = HR•SV•[(1.34•Hg•SaO2)+(0.003•PaO2)]

Preload Contractility Afterload • At low doses, inotrope and vasodilator • At higher doses, progressively more vasoconstrictive and chronotropic • Toxicity includes anxiety, tremors, tachycardia, tachyarrythmias, increased

myocardial O2 consumption, decreased splanchnic/hepatic perfusion, insulin antagonist causing hyperglycemia and lactic acidemia

Norepinephrine

• At almost all doses (0.01–1 mcg/kg/min),

stimulates α1 >> β1/2 • Typically starts at 0.02 mcg/kg/min, titrated 0.01 at a time • Increases SVR • Toxicity includes profound vasoconstriction and increased afterload

DO2 = HR•SV•[(1.34•Hg•SaO2)+(0.003•PaO2)]

Preload Contractility Afterload “Leave ‘em dead?”

• Norepinephrine superior to dopamine in adults with septic shock (De Backer et al 2010) • Pediatric community acquired septic shock usually cold, responsive to epi, versus hospital (CVL) acquired usually warm, responsive to NE (Brierley et al 2016) Consider…

• Positive pressure ventilation • Calcium in neonates • PGE for ductal dependent lesions • Steroids in adrenal insufficiency • iNO for PAH • Vasopressin for vasoplegia • Milrinone for myocardial dysfunction • Sodium bicarbonate – Doesn’t affect intracellular milieu

– Acidosis may improve myocardial performance and O2 delivery • Antibiotics or debridement/drainage for infectious source control • Massive transfusion protocol and/or antifibrinolytic therapy for trauma • Dialysis or other clearance strategies for toxins Reduce oxygen consumption!

• Mechanical ventilation • Sedation • Analgesia • Paralysis • Euthermia • Avoid catabolism • Manage infection

Case 1

• 13mo with 4d hx URI, fever, poor feeding, presents to ED for respiratory distress, lethargy

• VS: P140, RR45, BP 88/52, SaO2 93% • PE: sleepy but arousable, moderately labored breathing, coarse ronchi and occasional wheezes, no murmur, abd soft without organomegaly, cool extremities with fair pulses, CR 4 secs • Dx: bronchiolitis, hypoxemic respiratory failure, compensated septic shock

• Tx: FM O2, PIV, fluid bolus Case 2

• 7yo with 3wk hx cough fever, malaise, presents to ED somnolent, tachycardic, poorly perfused. Placed on

O2, cxs drawn, given abx, 40ml/kg fluid bolus, CXR reveals RML consolidation.

• VS: P140, RR35, BP 88/52, SaO2 98% • PE: arousable with sternal rub, moderately labored breathing, right sided crackles, no murmur, abd soft without organomegaly, cool extremities with poor pulses, CR 4sec • Dx: community acquired bacterial pneumonia, hypoxemic respiratory failure, uncompensated cold septic shock

• Tx: FM O2, consider intubation, repeat fluid bolus, prep for CVL and initiation of inotrope • IV falls out – IO, peripheral epi Case 3

• 14yo AML s/p chemo with port, admitted yesterday for F&N. Cxs sent, started broad spectrum abx, became hypotensive overnight, received 2L NS boluses.

• VS: P140, RR35, BP 88/31, SaO2 100% • PE: awake, alert, tachypneic but unlabored, CTA, hyperdynamic with 2/6 SEM, abd soft without organomegaly, warm extremities with bounding pulses, CR <1sec • Dx: line associated bacteremia, warm uncompensated septic shock

• Tx: FM O2, place PIV and repeat fluid bolus, start NE in port • Warm shock persists and lactate rising despite increasing NE to 0.2 – Increase NE, check cortisol, consider steroids Case 4

• 4yo with hx URI 3wk ago, now with malaise, tachypnea, poor perfusion. CXR shows cardiomegaly.

• VS: P140, RR35, BP 68/45, SaO2 94% • PE: irritable, tachypneic, labored breathing, bilateral crackles, gallop rhythm, hepatomegaly, cool extremities with poor pulses, CR 5sec, peripheral edema • Dx: viral myocarditis, dilated CM, uncompensated cardiogenic shock

• Tx: FM O2, place PIV, start milrinone, consider diuretics Case 5

• 2wo well TNB with 1d hx poor feeding, sleepy, floppy, cold.

• VS: P190, RR40, BP 45/30, SaO2 75% • PE: minimally responsive, agonal resp, limp, abnml facies, poor resp effort, shallow BS, no murmur, mild hepatomegaly, cool extremities with absent femoral pulses, CR 6sec • Dx: cyanotic CHD with left sided obstruction (e.g. IAA), closed ductus, uncompensated cardiogenic shock

• Tx: FM O2, intubate, place PIV or UVC, start PGE, order stat echo Case 6

• 4yo girl in high speed car accident, wearing a lap belt, brought by EMS with Trauma activation, awake, crying

• VS: P140, RR45, BP 90/65, SaO2 99% • PE: responsive, labored breathing, bruise across lower and tender on palpation, cool extremities with fair pulses but delayed CR • FAST reveals free fluid, CBC and T/S pending • Dx: blunt abdominal trauma with hemorrhage, compensated hypovolemic shock

• Tx: FM O2, place PIV, fluid bolus, considering further imaging and blood transfusion • HR increases, BP drops, Hg 8 – Stat O- transfusion, consider surgery Summary

• O2 delivery = O2 consumption • Shock is common and important to recognize and treat quickly • Airway and breathing before circulation • Cardiovascular medications may help restore

adequate O2 delivery to tissues – Use dopamine if you must, give peripherally if you must • Consider transfusion and surgery in bleeding trauma patient • Successfully treating shock starts with recognition which starts with YOU • Call us for advice or help and we will come –855-CHO-KIDS (855-246-5437) • Bread is bliss References

• Fuhrman BP, Zimmerman J, editors. 2006. Pediatric critical care. 3rd ed. Philadelphia (PA): Mosby Elsevier. • Nichols DG, ed. 2008. Roger’s textbook of Pediatric Intensive Care. 4th ed. Philadelphia (PA): Lippincott. • Surviving sepsis guidelines. • Too many articles to count!

Thanks

• Joe Carcillo • Every PICU, ED, Trauma and transport team I’ve ever worked with