1. Decide on your pre-test probability of the disease (choose an approximate probability based on our assessment)
2. Use the likelihood ratio that correlates to your exam.
3. Draw a straight line frm your pre-test probability starting point, to the LR of the feauture/test, take it through to find your post-test probability
4. Use this new post-test probability to help in your decision
Your patient has palatal petechiae, which confers a positive likelihood ratio (LR+) of 2.7
See below how to use this statistic based on your clinical assessment"
Symptoms and signs
Positive LR (95% CI)
Negative LR (95% CI)
Sensitivity (95% CI)
Specificity (95% CI)
Tender cervical nodes
Sibling with sore throat
Tonsillar and/or pharyngeal exudate
Large cervical nodes
Lack of cough
Lack of coryza
Red tonsils and/or pharynx
Documented temperature >38° or >38.5°C
Acute otitis media
History of tonsillectomy
Modified from: Shaikh et al. 2012
Cheung L et al. Throat swab have no influence on the management of patients with sore throats. J Laryngol. 217; 131:977-981.
Ebell MH et al. Rational Clinical Examination: Does This Patient Have Streptococcal Pharyngitis? JAMA. 2000;284(22):2912-2918
Homme JH et al. Duration of Group A Streptococcus PCR positivity following antibiotic treatment of pharyngitis. Diagn Microbiol Infect Dis. 2018 Feb;90(2):105-108.
Nakhoul GN et al. Management of Adults with Acute Streptococcal Pharyngitis: Minimal Value for Backup Strep Testing and Overuse of Antibiotics. J Gen Intern Med. 2013 Jun; 28(6): 830–834.
Oliver J et al. Group A Streptococcus pharyngitis and pharyngeal carriage: A meta-analysis. PLoS Negl Trop Dis. 2018 Mar 19;12(3):e0006335.
Shaikh N, Leonard E, Martin JM. Prevalence of streptococcal pharyngitis and streptococcal carriage in children: a meta-analysis. Pediatrics. 2010 Sep;126(3):e557-64.
Shaikh et al. Accuracy and Precision of the Signs and Symptoms of Streptococcal Pharyngitis in Children: A Systematic Review. J Pediatrics. 2012; 3:487-493.e3
This post and podcast are dedicated to the great K Kay Moody, DO, MPH for her stalwart effort to care for both patient and doctor. Thank you for all that you do to help us to be our best and for promoting #FOAMed #FOAMped and #MedEd.
Churchill NW et al. The first week after concussion: Blood flow, brain function and white matter microstructure. Neuroimage Clin. 2017; 14: 480–489.
Ellis MJ et al. Psychiatric outcomes after pediatric sports-related concussion. J Neurosurg Pediatr. 2015; 16:709-718.
Graham R et al. and the Committee on Sports-Related Concussions in Youth; Board on Children, Youth, and Families; Institute of Medicine; National Research Council. Sports-Related Concussions in Youth: Improving the Science, Changing the Culture. Washington (DC): National Academies Press (US); 2014 Feb 4.
Harmon KG et al. American Medical Society for Sports Medicine position statement: concussion in sport. Br J Sports Med. 2013; 47:15-26.
McCrory P et al. Consensus statement on concussion in sport—the 5th international conference on concussion in sport held in Berlin, October 2016. Br J Sports Med. 2016
Purcell LK et al. What factors must be considered in “return to school” following concussion and what strategies or accommodations should be followed? Br J Sports Med. 2018; 0:1-15.
Wang KK et al. An update on diagnostic and prognostic biomarkers for traumatic brain injury. Exp Rev Molec Gen. 2018; 18(2):165-180.
Wang Y et al. Cerebral Blood Flow Alterations in Acute Sport-Related Concussion. J Neurotrauma. 2016 Jul 1; 33(13): 1227–1236.
Baracco R et al. Pediatric Hypertensive Emergencies. Curr Hypertens Rep. 2014; 16:456.
Belsha CW. Pediatric Hypertension in the Emergency Department. Ann Emerg Med. 2008; 51(3):21-24.
Chandar J et al. Hypertensive crisis in children. Pediatr Nephrol. 2012; 27:741-751.
Dionne JM et al. Hypertension Canada’s 2017 Guidelines for the Diagnosis, Assessment, Prevention, and Treatment of Pediatric Hypertension. Canadian J Cardiol. 2017; 33:577-585
*Flynn JT, Kaelber DC, Baker-Smith CM, et al; SUBCOMMITTEE ON SCREENING AND MANAGEMENT OF HIGH BLOOD PRESSURE IN CHILDREN. Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents. Pediatrics. 2017; 140(3):e20171904
Gilhotra Y et al. Blood pressure measurements on children in the emergency department. Emergency Medicine Australasia. 2006; 18:148-154.
Lurbe E et al. 2016 European Society of Hypertension guidelines for the management of high blood pressure in children and adolescents. J Hypertens. 2016; 34:1-35.
Patel NH et al. Evaluation and management of pediatric hypertensive crises: hypertensive urgency and hypertensive emergencies. Open Access Emergency Medicine. 2012; 4:85-92.
Yang WC et al. Clinical Analysis of Hypertension in Children Admitted to the Emergency Department. Pediatr Neonatol. 2010; 1:44-51.
|Infant to Toddler||Preschool to School Age||Adolescent to Adult|
|Renal disease||Renal disease||Primary hypertension|
|Coarctation of the aorta||Coarctation of the aorta||Medication non-adherence|
|Bronchopulmonary dysplasia||Drug induced/toxicologic||Renal disease|
|Increased intracranial pressure||Increased intracranial pressure||Increased intracranial pressure|
|Congenital adrenal hyperplasia||Primary hypertension||Drug induced/toxicologic|
Adapted from: Constantine E. Hypertension. In: Textbook of Pediatric Emergency Medicine, 6th Ed. Fleischer GR, Ludwig S, Henretig FM (Eds). Lippincott, Williams & Wilkins, Philadelphia. 2010; p315.
This post and podcast are dedicated to Manpreet 'Manny' Singh for his collegiality, collaboration, and overall awesomeness.
[Details in Audio]
This post and podcast are dedicated to Henry Goldstein, B.Pharm, MBBS for his tireless dedication to all things #FOAMed, #FOAMped, and #MedEd. You are awesome. Make sure to visit Don't Forget the Bubbles!
Cohen GM, Albertini LW. Colic. Pediatr Rev. 2012; 33(7):332-3.
Friedman SB et al. The crying infant: diagnostic testing and frequency of serious underlying disease. Pediatrics. 2009; 123(3):841-8
Herman M, Le A. The crying infant. Emerg Med Clin North Am. 2007 Nov;25(4):1137-59.
Poole SR. The infant with acute, unexplained, excessive crying. Pediatrics. 1991; 88 (3): 450-5.
Prentiss KA, Dorfman DH. Pediatric Opthalmology in the Emergency Department. Emerg. Med. Clin. N. Am. 2008; 26: 181-198.
Shope TR, Rieg TS, Kathiria NN. Corneal abrasions in young infants. Pediatrics. 2010 Mar;125(3):e565-9. Epub 2010 Feb 8.
This post and podcast are dedicated to Mads Astvad for sharing his enthusiasm, clinical excellence, and #FOAMed warrior spirit.
Tak, min ven! #SMACConia #Vikingeblod
There is none. The presentation varies so much, we need a rule to live by:
Unilateral pelvic pain in a girl is ovarian torsion until proven otherwise. This includes the cases in which you are concerned about appendicitis. They both can be fake-outs.
Often the pain is severe and abrupt, but trying to tease this out is often not fruitful.
Here are the often-reported signs and symptoms associated with ovarian torsion:
Stabbing pain, 70%
Nausea and vomiting, 70%
Sudden, sharp pain in the lower abdomen, 59%
Pain radiating to the back, flank, or groin, 51%
Peritoneal signs, 3%
Fever, less than 2%
And of course…no pain on presentation…30%...intermittent torsion.
As far as Doppler flow goes, you may see one of several scenarios:
Other things you may see on ultrasound include focal tenderness with the probe, or the whirlpool sign – this is a twisted vascular pedicle.
In children, is there an ovarian size (volume) that rules out torsion?”
In the Journal of Pediatric Radiology, Servaes et al catalogued the ultrasound findings in children with surgically confirmed torsion over a 12 year period. In this case series of 41 patients, the median age was 11. The age range was one month old to 21 years of age. They found that in torsed ovaries, the ovarian volume was 12 x that compared to the normal, non-torsed contralateral ovary.
That is to say, in this case series all torsed ovaries were larger than the normal contralateral ovary.
Sudden unilateral lower abdominal or pelvic pain in a female? Think torsion.
Have a low threshold for investigation.
Know the performance characteristics of ultrasound findings and involve a gynecologist early.
This post and podcast are dedicated to Stephanie Doniger, MD for her enthusiasm, spirit, and expertise in #MedEd #FOAMed #FOAMped #POCUS
Abe M, Sarihan H. Oophoropexy in children with ovarian torsion. Eur. J. Pediatr. Surg. 2004;14:168.
Aziz D, Davis V, Allen L, Langer J. Ovarian torsion in children: Is oophorectomy necessary? J. Pediatr. Surg. 2004;39:750-3.
Bristow RE, Nugent AC, Zahurak ML, et al. Impact of surgeon specialty on ovarian-conserving surgery in young females with an adnexal mass. J. Adolesc. Health 2006;39:411.
Chang YJ, Yan DC, Kong MS, et al. Adnexal torsion in children. Pediatr. Emerg. Care. 2008;24:534-7.
Conforti A, Giorlandino C, Bagolan P. Fetal ovarian cysts management and ovarian prognosis: a report of 82 cases. J. Pediatr. Surg. 2009;44:868; author reply 868-9.
Guthrie BD, Adler MD, Powell EC. Incidence and trends of pediatric ovarian torsion hospitalizations in the United States, 2000-2006. Pediatrics 2010;125:532-8. Epub 2010 Feb 1.
Houry D, Abbott JT. Ovarian torsion: a fifteen-year review. Ann. Emerg. Med. 2001;38:156-9.
Huang TY, Lau BH, Lin LW, Wang TL, Chong CF, Chen CC. Ovarian cyst torsion in a toddler. Am. J. Emerg. Med. 2009;27:632, e1-3.
Hurh PJ, Meyer JS, Shaaban A. Ultrasound of a torsed ovary: characteristic gray-scale appearance despite normal arterial and venous flow on Doppler. Pediatr. Radiol. 2002;32:586-8. Epub 2002 May 25.
Kokoska E, Keller M, Weber T. Acute ovarian torsion in children. Am. J. Surg. 2000;180:462-5.
Oltmann SC, Fischer A, Barber R, Huang R, Hicks B, Garcia N. Cannot exclude torsion – a 15-year review. J. Pediatr. Surg. 2009;44:1212-6; discussion 1217.
Chmitt ER et al. Twist and Shout! Pediatric Ovarian Torsion Clinical Update and Case Discussion. Pediatr Emerg Care. 2013; 29(4):518-523.
Servaes S, Zurakowski D, Laufer MR, Feins N, Chow JS. Sonographic findings of ovarian torsion in children. Pediatr. Radiol. 2007;37:446-51. Epub 2007 Mar 15.
Valsky DV. Added value of the gray-scale whirlpool sign in the diagnosis of adnexal torsion. Ultrasound Obstet. Gynecol. 2010;36:630-4.
Normally metabolized into codeine-6-glucuronide (50-70%) and norcodeine (10-15%). Codeine, codeine-6-glucuronide, and norcodeine have low affinity for the μ (mu) receptor.
However, the most active metabolite of codeine is morphine with 200x the affinity for the mu receptor as the codeine derivates. The problem is, people vary in its metabolism from 0-15% of codeine is metabolized to morphine.
Ok, codeine is lame at best, unpredictable at worst.
True. Unless you are hiding a genetic time bomb.
You're an ultra-rapid metabolizer.
Some people have multiple extra copies of the DNA sequence for the CYP2D6 enzyme. Ultra rapid metabolizers funnel a huge proportion of their codeine into morphine metabolism, resulting in a bolus of morphine, ending in apnea.
This combination is no better than placebo -- all of the risks, with no proven benefit. This combination is notoriously abused -- as purple drank or sizzurp. The rapper Pimp C died of this.
Speaking of cough syrups...
The AAP recommends no cough and cold preparations in children under age 6. They have not been adequately studied in young children, and are not recommended for treating the common cold.
What then? You gotta give me something, doctor!
In a study in the Archives of Pediatric and Adolescent Medicine, Dr Paul and colleagues published: Effect of honey, dextromethorphan, and no treatment on nocturnal cough and sleep quality for coughing children and their parents. They compared a buckwheat honey, honey-flavored dextromethorphan (DM) and no treatment 30 min before bed for children with upper respiratory tract infections.
Of the three, honey, dextromethorphan, and no treatment – honey scored the best for symptom improvement and cough frequency.
Over age 1? Cough and cold? Honey. There is no concern about accidental overdose, parents are doing something with a proven effect, and compliance is pretty much 100% -- and Grandma approves.
No proven benefit over placebo. Also widely abused, in pill form ("Skittles") and/or liquid form mixed in alcoholic beverage ("robotripping").
Allow the child to speak for himself whenever possible. After acknowledging the parent’s input, perhaps try “I want to make sure I understand how the pain is for you. Tell me more.”
Engage parents and communicate the plan to them. Elicit their expectations, and give them of preview of what to expect in the ED.
Opioids are meant for pain caused by acute tissue injury, for the briefest period of time feasible. Older school-aged children and adolescents are increasingly at risk for opioid dependence and addiction.
Give detailed advice on how to manage pain at home. Set expectations. Let them know you understand and will help them through your good advice that will carry them through this difficult time. Patients and families often just need a plan. Map it out clearly.
Just say no to: codeine, promethazine with codeine, and dextramethorphan.
This post and podcast are dedicated to Bryan Hayes, PharmD for his practical approach to pharmacologic conundrums and to David Juurlink, MD, PhD for his steadfast dedication to patient safety and clinician education. Check out Bryan's helpful blog and clinical resource, PharmERToxGuy. Check out David anywhere one utters the word Tra-ma-dol.
This post and podcast are dedicated to Kevin Klauer, DO, EJD, FACEP for his dedication to education, and for his unique balance of safety and keeping it real. Thank you.
For intractable epilepsy; sends retrograde signal up corona radiata
Also may be used in: depression, bulimia, Alzheimer, narcolepsy, addiction, and others
Used to infuse basal rate of drug, usually baclofen for spasticity, but pump may contain morphine, bupivicaine, clonidine. Also used for severe MS, stroke, TBI, chronic pain. Verify the medication and identify the toxidrome if symptomatic.
May be left ventricular assist, right ventricular assist, or biventricular assist device.
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Ruffoli R, Giorgi FS, Pizzanelli C et al. The chemical neuroanatomy of vagus nerve stimulation. Journal of Chemical Neuroanatomy; 2011; 42: 288–296.
Borowski A, Littleton AG, Borkhuu B et al. Complications of Intrathecal Baclofen Pump Therapy in Pediatric Patients. J Pediatr Orthop. 2010; 30:76–81.
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Yang TF, Wang JC, Chiu JW et al. Ultrasound-guided refilling of an intrathecal baclofen pump—a case report. Childs Nerv Syst. 2013; 29:347–349.
Yeh RN, Nypaver MM, Deegan TJ, Ayyangar R. Baclofen Toxicity in an 8-year-old with an Intrathecal Baclofen Pump. J Emerg Med. 2004; 26(4): 163–167.
Blume ED, Naftel DC, Bastardi HJ et al. for the Pediatric Heart Transplant Study Investigators. Outcomes of Children Bridged to Heart Transplantation With Ventricular Assist Devices: A Multi-Institutional Study. Circulation. 2006; 113: 2313-2319.
Colón JE, Laborde ME, Nossaman BD. Case Report: Left Ventricular Assist Device in a 12 Year Old Child as a Bridge to Heart Transplantation. Section of Congenital Cardiac Anesthesia, Ochsner Medical Center, New Orleans, Louisiana. 2012.
Fan Y, Weng YG, Huebler M et al. Predictors of In-Hospital Mortality in Children After Long-Term Ventricular Assist Device Insertion. J Amer Coll Cardiol. 2011; 58(11):1183–90
Fraser CD, Jaquiss RDB, Rosenthal DN et al. Prospective Trial of a Pediatric Ventricular Assist Device. N Engl J Med. 2012;367:532-41.
Gazit AZ, Gandhi SK, Canter CC. Mechanical Circulatory Support of the Critically Ill Child Awaiting Heart Transplantation. Current Cardiology Reviews. 2010; 6: 46-53.
VanderPluym CJ, Fynn-Thompson F, Blume ED. Ventricular Assist Devices in Children Progress With an Orphan Device Application. Circulation. 2014;129:1530-1537.
One of our largest responsibilities in the Emergency Department is sorting out benign from surgical or medical causes of abdominal pain. Morbidity and mortality varies by age and condition.
Neu J, Walker A. Necrotizing Enterocolitis. N Eng J Med. 2011; 364(3):255-264.
Niño DF et al. Necrotizing enterocolitis: new insights into pathogenesis and mechanisms. Nature. 2016; 13:590-600.
Walsh MC et al. Necrotizing Enterocolitis: A Practitioner’s Perspective. Pediatr Rev. 1988; 9(7):219-226.
Malrotation with Midgut Volvulus
Applegate KE. Intestinal Malrotation in Children: A Problem-Solving Approach to the Upper Gastrointestinal Series. Radiographics. 2006; 26:1485-1500.
Kapfer SA, Rappold JF. Intestinal Malrotation – Not Just the Pediatric Surgeon’s Problem. J Am Coll Surg. 2004; 199(4):628-635.
Lee HC et al. Intestinal Malrotation and Catastrophic Volvulus in Infancy. J Emerg Med. 2012; 43(1):49-51.
Martin V, Shaw-Smith C. Review of genetic factors in intestinal malrotation. Pediatr Surg Int. 2010; 26:769-781.
Nehra D, Goldstein AM. Intestinal malrotation: Varied clinical presentation from infancy through adulthood. Surgery. 2010; 149(3):386-391.
Amiel J, Sproat-Emison E, Garcia-Barcelo M, et al. Hirschsprung disease, associated syndromes and genetics: a review. J Med Genet 2008; 45:1.
Arshad A, Powell C, Tighe MP. Hirschsprung's disease. BMJ 2012; 345:e5521.
Aworanti OM, McDowell DT, Martin IM, Quinn F. Does Functional Outcome Improve with Time Postsurgery for Hirschsprung Disease? Eur J Pediatr Surg 2016; 26:192.
Clark DA. Times of first void and first stool in 500 newborns. Pediatrics 1977; 60:457.
Dasgupta R, Langer JC. Evaluation and management of persistent problems after surgery for Hirschsprung disease in a child. J Pediatr Gastroenterol Nutr 2008; 46:13.
De Lorijn F, Reitsma JB, Voskuijl WP, et al. Diagnosis of Hirschsprung's disease: a prospective, comparative accuracy study of common tests. J Pediatr 2005; 146:787.
Doig CM. Hirschsprung's disease and mimicking conditions. Dig Dis 1994; 12:106.
Khan AR, Vujanic GM, Huddart S. The constipated child: how likely is Hirschsprung's disease? Pediatr Surg Int 2003; 19:439.
Singh SJ, Croaker GD, Manglick P, et al. Hirschsprung's disease: the Australian Paediatric Surveillance Unit's experience. Pediatr Surg Int 2003; 19:247.
Suita S, Taguchi T, Ieiri S, Nakatsuji T. Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years. J Pediatr Surg 2005; 40:197.
Sulkowski JP, Cooper JN, Congeni A, et al. Single-stage versus multi-stage pull-through for Hirschsprung's disease: practice trends and outcomes in infants. J Pediatr Surg 2014; 49:1619.
Aspelund G, Langer JC. Current management of hypertrophic pyloric stenosis. Semin Pedaitr Surg. 2007; 16:27-33.
Dias SC et al. Hypertrophic pyloric stenosis: tips and tricks for ultrasound diagnosis. Insights Imaging. 2012; 3:247-250.
Kawahara H et al. Medical treatment of infantile hypertrophic pyloric stenosis: should we always slice the olive? J Pediatr Surg. 2005; 40:1848-1851.
Mack HC. Adult Hypertrophic Pyloric Stenosis. Arch Inter Med. 1959; 104:78-83.
Meissner PE et al. Conservative treatment of infantile hypertrophic pyloric stenosis with intravenous atropine sulfate does not replace pyloromyotomy. Pediatr Surg Int. 2006; 22:1021-1024.
Mercer AE, Phillips R. Can a conservative approach to the treatment of hypertrophic pyloric stenosis with atropine be considered a real alternative to pyloromyotomy? Arch Dis Child. 2013; 95(6): 474-477.
Pandya S, Heiss K, Pyloric Stenosis in Pediatric Surgery.Surg Clin N Am. 2012; 92:527-39.
Peters B et al. Advances in infantile hypertrophic pyloric stenosis. Expert Rev Gastroenterol Hepatol. 2014; 8(5):533-541.
Apelt N et al. Laparoscopic treatment of intussusception in children: A systematic review. J Pediatr Surg. 2013; 48:1789-1793.
Applegate KE. Intussusception in Children: Imaging Choices. Semin Roentgenol. 2008; 15-21.
Bartocci M et al. Intussusception in childhood: role of sonography on diagnosis and treatment. J Ultrasound. 2015; 18 Gilmore AW et al. Management of childhood intussusception after reductiion by enema. Am J Emerg Med. 2011; 29:1136-1140.:205-211.
Chien M et al. Management of the child after enema-reduced intussusception: hospital or home? J Emerg Med. 2013; 44(1):53-57.
Cochran AA et al. Intussusception in traditional pediatric, nontraditional pediatric, and adult patients. Am J Emerg Med. 2011; 523-527.
Loukas M et al. Intussusception: An Anatomical Perspective With Review of the Literature. Clin Anatomy. 2011; 24: 552-561.
Mendez D et al. The diagnostic accuracy of an abdominal radiograph with signs and symptoms of intussusception. Am J Emerg Med. 2012; 30:426-431.
Whitehouse et al. Is it safe to discharge intussusception patients after successful hydrostatic reduction? J Pediatr Surg. 2010; 45:1182-1186.
Amin P, Chang D. Management of Complicated Appendicitis in the Pediatrc Population: When Surgery Doesn’t Cut it. Semin Intervent Radiol. 2012; 29:231-236
Blakely ML et al. Early vs Interval Appendectomy for Children With Perforated Appendicitis. Arch Surg. 2011; 146(6):660-665.
Bundy DG et al. Does This Child Have Appendicitis? JAMA. 2007; 298(4):438-451.
Cohen B et al. The non-diagnostic ultrasound in appendicitis: is a non-visualized appendix the same as a negative study? J Pediatr Surg. 2015 Jun;50(6):923-7
Herliczek TW et al. Utility of MRI After Inconclusive Ultrasound in Pediatric Patients with Suspected Appendicitis. AJT. 2013; 200:969-973.
Janitz et al. Ultrasound Evaluation for Appendicitis. J Am Osteopath Coll Radiol. 2016; 5(1):5-12.
Kanona H et al. Stump Appendicitis: A Review. Int J Surg. 2012; 10:4255-428.
Kao LS et al. Antibiotics vs Appendectomy for Uncomplicated Acute Appendicitis. Evid Based Rev Surg. 2013;216(3):501-505.
Petroianu A. Diagnosis of acute appendicitis. Int J Surg. 2012; 10:115-119.
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Schizas AMP, Williams AB. Management of complex appendicitis. Surgery. 2010; 28(11):544-548.
Shogilev DJ et al. Diagnosing Appendicitis: Evidence-Based Review. West J Emerg Med. 2014; 15(4):859-871.
Wray CJ et al. Acute Appendicitis: Controversies in Diagnosis and Management. Current Problems in Surgery. 2013; 50:54-86
Babl FE et al. Does nebulized lidocaine reduce the pain and distress of nasogastric tube insertion in young children? A randomized, double-blind, placebo-controlled trial. Pediatrics. 2009 Jun;123(6):1548-55
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Myocardial infarction (MI) in children is uncommon, but underdiagnosed. This is due to two main factors: the etiologies are varied; and the presenting symptoms are “atypical”.
Two main presentations of MI due to congenital lesions: novel and known. The novel presentation is at risk for underdiagnosis, due to its uncommonness and vague, atypical symptoms. There are usually some red flags with a careful H&P. The known presentation is a child with a history of congenital heart disease, addressed by corrective or palliative surgery. This child is at risk for expected complications, as well as overdiagnosis and iatrogenia. Risk stratify, collaborate with specialists.
Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) is an example of what can go wrong during fetal development: any abnormality in the number, origin, course, or morphology of the coronary arteries can present as a neonate with sweating during feeds (steal syndrome), an infant in CHF, or an older child with failure to thrive or poor exercise tolerance.
Normal coronary arteries run along the epicardial surface of the heart, with projections into the myocardium. If part of the artery’s course runs within the myocardium (i.e. the artery weaves into and/or out of the myocardium), then there is a myocardial bridge of the coronary artery. With every systolic contraction, the artery is occluded.
Although a myocardial bridge may not cause symptoms (especially at distal portions), the area it supplies is at risk.
With any minor trauma or exertion, demand may outpace supply, resulting in ischemia.
Diagnosis is made on coronary angiography.
The child with single ventricle physiology may have a Norwood procedure at birth (creation of a neoaorta, atrial septectomy, and Blalock-Taussig shunt), a Bidirectional Glenn procedure at 3-6 months (shunt removed, superior vena cava connected to pulmonary arteries), and a Fontan procedure at about 2-3 years of age (inferior vena cava blood flow is shunted into the pulmonary arteries).
These children depend on their preload to run blood passively into the pulmonary circuit; afterload reduction is also important to compensate for a poor left ejection fraction, as well as to avoid the development of pulmonary hypertension. They are typically on an anticoagulant (often aspirin), a diuretic (e.g. furosemide), and an afterload reduction agent (e.g. enalapril).
Any disturbance in volume status (hyper- or hypovolemia), anticoagulation, or afterload may cause myocardial strain or infarction. Take the child s/p Fontan seriously and involve his specialists early with any concerns.
The body’s inflammatory-mediated reaction to a real or perceived insult can cause short- and long-term cardiac sequelae. Find out how well the underlying disease is controlled, and what complications the child has had in the past.
The red, hot, crispy, flaky child: acute Kawasaki disease
Kawasaki disease (KD) is an acute systemic vasculitis, diagnosed by the presence of fever for five or more days accompanied by four or more criteria: bilateral conjunctival injection, mucositis, cervical lymphadenopathy, polymorphous rash, and palmar or sole desquamation. The criteria may occur (and disappear) at any time during the illness.
Infants are under double jeopardy with Kawasaki Disease. They are more likely to have incomplete KD (i.e. not fulfill strict criteria) and if they have KD, they are more likely to suffer the dangerous consequences of aneurysm formation (chiefly coronary arteries, but also brain, kidney). Have a low threshold for investigation.
Treatment includes 2 g/kg/day IVIG and high-dose aspirin (30-50 mg/kg/day) acutely, then low-dose aspirin (5 mg/kg/day) for weeks to months. Regular and long-term follow-up with Cardiology is required.
The family and child with a history of KD may have psychological trauma and continuous anxiety about the child’s risk of MI. Approximately 4.7% of children who were promptly diagnosed and correctly treated will go on to have cardiac sequelae.
Children who have no detected cardiac sequelae by 8 weeks, typically continue to be asymptomatic up to 20 years later.
Smaller aneurysms tend to regress over time, especially those < 6 mm.
Thrombi may calcify, or the lumen may become stenotic due to myofibroblast proliferation. Children with any coronary artery dilatation from KD should be followed indefinitely.
Giant aneurysms (≥8 mm) connote the highest risk for MI.
Parents often are concerned about recurrence, and any subsequent fever can be distressing. There is a low rate of recurrence for KD: approximately 2%. Infants who have coronary aneurysms are at the highest risk for recurrence.
Up to 15% of cases of SLE begin in childhood. Adult criteria are used, with the caveat that the diagnosis of SLE in children can be challenging; many children only manifest a few of the criteria initially before going on to develop further systemic involvement.
The Systemic Lupus International Collaborating Clinics (SLICC) revised the criteria in 2012. The patient should have ≥4/17 clinical and/or immunologic criteria. The clinical criteria are: acute cutaneous (malar); chronic cutaneous (discoid); oral; alopecia; synovitis; serositis; renal; neurologic; hemolytic anemia; leukopenia; or thrombocytopenia. The immunologic criteria are: ANA; anti-dsDNA; anti-Sm; antiphospholipid; low complement; and/or Direct Coombs (in absence of hemolytic anemia). At least one criterion should be clinical, and at least one should be immunologic.
Children with antiphospholipid syndrome (APS) may occur with or without SLE. Patients are at risk for venous and arterial thrombi formation. APS may also cause structural damage, such as valvular thickening and valvular nodes (Libman-Sacks endocarditis). Mitral and aortic valves are at the highest risk.
Although most children with chest pain will not have MI, those with comorbidities should be investigated carefully.
Direct, blunt trauma to the chest can cause myocardial stunning, dysrhythmias, or an asymptomatic rise in Troponin I. However, some children are at risk for disproportionate harm due to a previously unknown risk factor. Clinically significant cardiac injury occurs in up to 20% of patients with non-penetrating thoracic trauma.
The motor vehicle collision: blunt myocardial injury
Direct trauma (steering wheel, airbag, seatbelt), especially in fast acceleration-deceleration injury, may cause compression of the heart between the sternum and the thoracic spine.
Electrocardiography (ECG) should be performed on any patient with significant blunt chest injury. A negative ECG is highly consistent with no significant blunt myocardial injury.
Any patient with a new abnormality on ECG (dysrhythmia, heart block, or signs of ischemia) should be admitted for continuous ECG monitoring.
Elevation in troponin is common, but not predicted. A solitary elevated troponin without ECG abnormality is of unclear significance. Author’s advice: obtain troponin testing if there is an abnormal ECG, more than fleeting suspicion of BCI, and/or the child will be admitted for monitoring.
Direct trauma (e.g. MVC, baseball, high-velocity soccer ball) may cause damage to the left anterior descending artery or left circumflex artery, at the highest risk due to their proximity to the chest wall. Thrombosis and/or dissection may result, often presenting in a focal pattern of ischemia on the ECG.
Echocardiography may reveal valvular damage related to the injury, as well as effusion and ejection fraction. Since there is often a need to investigate the coronary anatomy, percutaneous coronary intervention (PCI) is recommended.
As mentioned in the congenital section (above), a known variation of a coronary artery’s course involves weaving in and out of the myocardium, creating a baseline risk for ischemia. Even minor trauma in a child with a myocardial bridge may cause acute thrombus, or slow stenosis from resulting edema. Unfortunately, the presence of myocardial bridging is often unknown at the time of injury. Approximately 25% of the population may have myocardial bridging, based on autopsy studies. Take the child seriously who has disproportionate symptoms to what should be a minor injury.
Coagulopathic and thrombophilic states may predispose children to focal cardiac ischemia. The best documented cormorbidity is sickle cell disease, although other pro-thrombotic conditions also put the child at risk.
The child with sickle cell disease and chest pain: when it’s not acute chest syndrome
Sickle cell disease (SCD) can affect any organ system, although the heart is traditionally considered a lower-risk target organ for direct sickling and ischemia. The major cardiac morbidity in sickle cell is from strain, high-output failure and multiple, serial increases in myocardial demand, causing left ventricular hypertrophy and congestive heart failure.
However, there is mounting evidence that acute myocardial ischemia in sickle cell disease may be underappreciated and/or attributed to other causes of chest pain.
Other cardiac sequelae from SCD include pulmonary hypertension, left ventricular dysfunction, right ventricular dysfunction, and chronic iron overload.
Evidence of myocardial ischemia/infarction in children with SCD has been demonstrated on single-photon emission computed tomography (SPECT) scan.
Children who suffer from nephrotic syndrome lose proteins that contribute to the coagulation cascade. In addition, lipoprotein profiles are altered: there is a rise in the very low-density lipoproteins (LDL), contributing to accelerated atherosclerosis. Typically nephrotic patients have normal levels of high-density lipoproteins (HDL), unless there is profuse proteinuria.
Children with difficult-to-control nephrotic syndrome (typically steroid-resistant) may form accelerated plaques that rupture, causing focal MI, as early as school age.
Asymptomatic patent foramen ovale (PFO) is the cause of some cases of cryptogenic vascular disease, such as stroke and MI. However, the presence of PFO alone does not connote higher risk. When paired with an inherited or acquired thrombogenic condition, the venous thrombus may travel from the right-sided circulation to the left, causing distal ischemia. Many of these cases are unknown until a complication arises.
A family history of adult-onset hypercholesterolemia is not necessarily a risk factor for early complications in children, provided the child does not have the same acquired risk factors as adults (e.g. obesity, sedentary lifestyle, smoking, etc). Parents may seek help in the ED for children with chest pain and no risk factors, but adult parents who have poor cholesterol profiles.
The exception is the child with familial hypercholesterolemia, who is at risk for accelerated atherosclerosis and MI.
Myocarditis has varied etiologies, including infectious, medications (chemotherapy agents), immunologic (rheumatologic, transplant rejection), toxins (arsenic, carbon monoxide, heavy metals such as iron or copper), or physical stress (electrical injury, heat illness, radiation).
In children, the most common cause of myocarditis is infectious (viruses, protozoa, bacteria, fungal, parasites). Of these, viral causes are the most common (adenovirus, enterovirus, echovirus, rubella, HHV6).
The verbal child may complain of typical chest complaints, or may come in with flu-like illness and tachycardia or ill appearance out of proportion to presumed viral illness.
The most common presenting features in children with myocarditis are: shortness of breath, vomiting, poor feeding, hepatomegaly, respiratory distress, and fever.
Beware of the poor feeding, tachycardic, ill appearing infant who “has a cold” because everyone else around him has a ‘cold’. That may very well be true, but any virus can be invasive with myocardial involvement. Infants are only able to increase their cardiac output through increasing their heart rate; they cannot respond to increased demands through ionotropy. Look for signs of acute heart failure, such as hepatomegaly, respiratory distress, and sacral edema.
The child with tachycardia out of proportion to complaint: myocarditis
The previously healthy child with “a bad flu” may simply be very symptomatic from influenza-like illness, or he may be developing myocarditis. Look for chest pain and tachycardia out of proportion to presumed illness, and constant chest pain, not just associated with cough.
Acute heart failure may mimic viral pneumonia. Look for disproportionate signs and symptoms.
Younger children may get into others’ medications, be given dangerous home remedies, take drugs recreationally, have environmental exposures (heavy metals), suffer from a consequence of a comorbidity (iron or copper overload) or have adverse events from generally safe medications.
Attention deficit hyperactivity disorder (ADHD) is growing in rate of diagnosis and use of medications. As the only medical diagnosis based on self-reported criteria, many children are given stimulants regardless of actual neurologic disorder; with a higher proportion of children exposed to stimulants, adverse effects are seen more commonly.
Methylphenidate is related to amphetamine, and they both are dopaminergic drugs. Their mechanisms of action are different, however. Methylphenidate increases neuronal firing rate. Methamphetamine reduces neuronal firing rate; cardiovascular sequelae such as MI and CHF are more common in chronic methamphetamine use.
Although methylphenidate is typically well tolerated, risks include dysrhythmias such as ventricular tachycardia.
Some anti-epileptic agents, such as carbamazepine, promote a poor lipid profile, leading to atherosclerosis and early MI. Case reports include school-aged children on carbamazepine who have foamy cells in the coronary arteries, aorta, and vasa vasorum on autopsy. It is unclear whether this is a strong association.
The spice trader: synthetic cannabinoids
Synthetic cannabinoids are notoriously difficult to regulate and study, as the manufacturers label them as “not for human consumption”. Once reports surface of abuse of a certain compound, the formula is altered slightly and repackaged, often in a colorful or mysterious way that is attractive to teenagers.
The misperceptions are: are a) synthetics are related to marijuana and therefore safe and b) marijuana is inherently “safe”. Both tend to steer unwitting teens to take these unknown entities. Some suffer MI as a result.
Exposure to tetrahydrocannabinol (THC) in high-potency marijuana has been linked to myocardial ischemia, ventricular tachycardia, and ventricular fibrillation. Marijuana can increase the heart rate from 20-100%, depending on the amount ingested.
K2 (“kush 2.0”) or Spice (Zohai, Genie, K3, Bliss, Nice, Black Mamba, fake weed, etc) is a mixture of plant leaves doused in synthetic chemicals, including cannabinoids and fertilizer (JWH-108), none of which are tested or safe for human consumption.
Synthetic cannabinoids have a higher affinity to cannabinoid receptors, conferring higher potency, and therefore worse adverse effects. They are thought to be 100 to 800 times more potent as marijuana.
Bath salts (Purple Wave, Zoom, Cloud Nine, etc) can be ingested, snorted, or injected. They typically include some form of cathinone, such as mephedrone, similar to the substance found in the naturally occurring khat plant. Hallucinations, palpitations, tachycardia, MI, and dysrhythmias have been reported from their use as a recreational drug.
Chest pain with marijuana, synthetic cannabinoid, or bath salt ingestion should be investigated and/or monitored.
Riding that train: high on cocaine
Cocaine is a well-known cause of acute MI in young people. In addition to the direct stimulant causes acutely, such as hypertension, tachycardia, and impaired judgement (coingestions, risky behavior), chronic cocaine use has long-term sequelae. Cocaine causes accelerated atherosclerosis. That, in conjunction with arterial vasospasm and platelet activation, is a recipe for acute MI in the young.
Methamphetamine is a highly addictive stimulant that is relatively inexpensive and widely available. Repeated use causes multiple psychiatric, personality, and neurologic changes. Risky behavior, violence, and motor vehicle accidents are all linked to this drug.
Like cocaine, methamphetamine may cause fatal dysrhythmias, acute MI from demand ischemia, and long-term sequelae such as congestive heart failure.
Acute MI is a challenging presentation in children:
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This post and podcast are dedicated to Edwin Leap, MD for his sanity and humanity in the practice of Emergency Medicine. Thank you, Dr Leap for all that you do.
When a baby is born with jaundice, it’s always bad. This is pathologic jaundice, and it’s almost always caught before the baby goes home. Think about ABO-incompatbility, G6PD deficiency, Crigler-Najjar, metabolic disturbances, and infections to name a few. Newborns are typically screened and managed.
Physiologic jaundice, on the other hand, is usually fine, until it’s not.
All babies have some inclination to develop jaundice. Their livers are immature. They may get a little dehydrated, especially if mother’s milk is late to come in. In today’s practice, we are challenged to catch those at risk for developing complications from rising bilirubin levels.
Hyperbilirubinemia is the result of at least one of three processes: you make too much, you don’t process it enough, or you don’t get rid of it fast enough.
Bilirubin mostly comes from the recycling of red blood cells. Heme is broken down in in the liver and spleen to biliverdin then bilirubin.
Normal, full term babies without jaundice run a little high -- bilirubin production is two to three times higher than in adults, because they are born with a higher hematocrit. Also, fetal hemoglobin is great at holding on to oxygen, but has a shorter life span, and high turn-over rate, producing more bilirubin.
Think of bilirubin as your email. Unconjugated bilirubin is your unread email. To process it or get rid of it – you have to open it. Of course, the more unread messages that accumulate, the more unwell you feel.
Conjugated bilirubin is your opened and processed email. So much easier to sort out, deal with, and get rid of.
Both unread email and unconjugated bilirubin continue to float around in your inbox. Unconjugated bilirubin keeps getting reabsorbed in the intestinal mucosa through enterohepatic circulation.
Processed email and conjugated bilirubin are easier to sort out. Conjugated bilirubin is water soluble, so it goes right into the read folder in your gallbladder, and is excreted off your inbox. Later on down the line in the intestine, conjugated bilirubin can’t be reabsorbed through the intestinal mucosa. Like when you open an email and forget about it – it passes on through, out of your system.
Newborns are terrible at answering emails. There is a lot of unread unconjugated bilirubin is floating around. The liver and spleen are just not able to keep up.
Also, newborns have a double-whammy administrative load. Normally, bacteria in the gut can further break down conjugated bilirubin to urobilin and get excreted in the urine. The infant’s gut is relatively sterile, so no admin assistance there. Just to add to the workload a poor little newborn has to do – he is being sabotaged by extra beta-glucuronidase which will take his hard-earned conjugated bilirubin and unconjugate it again, then recycle it, just like email you “mark as unread”.
The recommended followup is 48 hours after discharge from the nursery for a routine bilirubin check, often in clinic, and often via the transcutaneous route.
|Infant Discharged||Should Be Seen by Age|
|Before age 24 h||72 h|
|Between 24 and 48 h||96 h|
|Between 48 and 72 h||120 h|
The neonate will end up in your ED off hours, if there is concern, if his status deteriorates, or simply by chance. We need to know how to manage this presentation, because time is of the essence to avoid complications if hyperbilirubinemia is present.
Assess risk for developing severe hyperbilirubinemia.
|Risk Factors for Developing Hyperbilirubinemia|
|Total serum bilirubin/Transcutaneous bilirubin in high-risk zone|
|Jaundice in first 24 hours|
|ABO incompatibility with positive direct Coombs, known hemolytic disease, or elevated ETCO|
|Gestational age 35-36 weeks|
|Prior sibling had phototherapy|
|Cephalohematoma or bruising|
|Exclusive breastfeeding, especially with poor feeding or weight loss|
|East Asian Race|
Check bilirubin and match this with how old the child is -- in hours of life -- at the time of bilirubin measurement.
Use the Bilitool or Bhutani Nomogram (below).
In general, babies at low-risk and low-intermediate risk can go home (see below). Babies at high-intermediate or high risk are admitted (see below).
Assess risk for developing subsequent neurotoxicity.
The neonate who is safe to go home is well appearing, and not dehydrated. His total bilirubin is in the low to low-intermediate risk for developing severe hyperbilirubinemia, and he is not at high risk for neurotoxicity based on risk factors.
Babies need to stay hydrated. Breast feeding mothers need encouragement and need to offer feeds 8-12 times/day – an exhausting regimen. The main message is: stick with it. Make sure to enlist the family's help and support to keep Mom hydrated, eating well, and resting whenever she can. Supplementing with formula or expressed breast milk is not routinely needed. Be explicit that the neonate should not receive water or sugar water – it can cause dangerous hyponatremia. A moment of solid precautionary advice could avert a disaster in the making.
The child’s pediatrician will help more with this, and you can remind nursing mothers of the excellent La Leche League – an international group for breastfeeding support. They have local groups everywhere, including a hotline to call.
If the baby is at high intermediate or high risk for hyperbilirubinemia, then he should be admitted for hydration, often IV. Most babies with hyperbilirubinemia are dehydrated, which just exacerbates the problem.
Bililights or biliblankets, provide the baby with the right blue spectrum of light to isomerize bilirubin to the more soluble form. Traditionally, we have thought them to be more effective or safer than filtered sunlight. A recent randomized control trial by Slusher et al. in the New England Journal of Medicine compared filtered sunlight versus conventional phototherapy for safety and efficacy in a resource-poor environment. These were all term babies with clinically significant jaundice in Nigeria. To standardize the intervention, they used commercial phototherapy canopies that remove most UV rays. None of them became dehydrated or became sunburned. The filtered sunlight resulted in a 93% successful treatment versus 90% for conventional phototherapy. My take away: we now have some evidence basis for using filtered sunlight as an adjunct for babies well enough to go home.
Although rare, the critically ill neonate with hyperbilirubinemia requires immediate intervention.
He will be dehydrated – possibly in shock. He will be irritable.
Or, he may just have a dangerously high bilirubin level – at any minute he could develop bilirubin induced neurologic dysfunction, or BIND, especially when bilirubin concentrations reach or surpass 25 mg/dL (428 micromol/L). The bilirubin is so concentrated that it leeches past the blood brain barrier and causes neuronal apoptosis. BIND is a spectrum from acute bilirubin encephalopathy to kernicterus, all involving some disorder in vision, hearing, and later gait, speech, and cognition.
Acute bilirubin encephalopathy starts subtly. The neonate may be sleepy but hypotonic or have a high-pitched cry; he maybe irritable or inconsolable, jittery or lethergic.
The dehydration and neurologic dysfnction from the hyperbilirubinemia may even cause fever. Check the bilirubin in any neonate you are working up for sepsis.
Acute bilirubin encephalopathy may progress to an abnormal neurologic exam, seizures, apnea, or coma.
Kernicterus is the final, permanent result of bilirubin encephalpathy. The child may have choreoathetoid cerebral palsy with chorea, tremor, ballismus, and dystonia. He may have sensorineural hearting loss, or cognitive dysfunction.
It is for this reason that any child sick enough to be admitted should be considered for exchange transfusion. Most babies need just a little gentle rehydration and bililights, but to be sure, the admitting team will look at a separate nomogram to gage the child’s risk and decide whether to pull the trigger on exchange transfusion. For our purposes, a ballpark estimate is that if the total serum bilirubin is 5 mg/dL above the phototherapy threshold, or if they have any red flag signs or symptoms, then exchange transfusion should be started.
Exchange transfusion involves taking small aliquots of blood from the baby and replacing them with donor blood. It’s often a manual procedure, done with careful monitoring. It can be done with any combination of umbilical arteries or veins with peripheral arteries or veins. In general, arteries are the output, veins are for transfusion. The baby may need a double-volume exchange, which ends up replacing about 85% of circulating blood, a single-voume exchange, replacing about 60% of blood, or any fraction of that with apartial volume exchange. It is a very delicate procedure that requires multiple hours and often multiple staff.
For our pruposes, just be aware that the jaundiced baby in front of you may need escalation of his care.
Find out the hour of life of the baby at the time of bilirubin measurement. Identify risk factors for developing severe hyperbilirubinemia and/or neurotoxicity
The child with low to low-intermediate risk may be a good outpatient candidate provided he is well, not dehydrated, and follow-up is assured.
The child with high-intermediate to high-risk for developing severe hyperbilirubinemia should be admitted for hydration, bililights, and/or assessment for exchange transfusion.
The unwell child with or without current neurologic findings should have immediate exchange transfusion.
This post and podcast are dedicated to Gita Pensa, MD, for her commitment to #FOAMed and passion for asynchronous learning and education innovation.