Case: Tommy is a 6yo boy coming in for a sick appointment. His mother states that he has had a limp for “a while.” He is an active kid and she thought he had bruised himself and didn’t think too much of it. Now, 2 weeks later, he has not improved. After a complete history and physical, an x-ray of the hips is obtained:
Frog leg view
Diagnosis: Legg-Calve-Perthes Disease
Radiology Findings (courtesy of Radiopaedia): “The left superior femoral epiphysis is markedly flattened, sclerotic and appear to be undergoing fragmentation. Alignment is unremarkable and the pelvis and acetabulum appear normal. The Right hip appears normal.”
- Legg-Cavle-Perthes Disease is an idiopathic avascular necrosis of the femoral head
- Generally affects children between 2-12 years of age and tends to be more common in boys as opposed to girls (Hermann et al, 2015)
- Bilateral in 10-20% of patients (Nigrovic, 2016)
- Children tend to present with limp and limited range of motion of the hip.
- Children may also initially present with knee pain or thigh pain
- Plain films of the hips help make the diagnosis, though tend to normal early in the course [unlike case above, which likely details later stage findings] (Nigrovic, 2016)
- MRI helpful in diagnosis early in disease process as well as detailing prognosis (Dillman et al, 2009)
- findings include: proximal femoral necrosis, delayed contrast enhancement, abnormalities in the proximal femoral physis
- Referral to orthopedic surgeon important for long term management
- Once diagnosed, children should be made nonweight bearing until seen by orthopedist (Nigrovic, 2016)
- Ongoing management is symptomatic (anti-inflammatory meds, physical therapy, limited activities), though there is a paucity of evidence to guide recommendations.
Faculty Reviewer: Alison Riese, MD
Case courtesy of Dr. Bruno Di Muzio, Radiopaedia.org. From the case rID: 12153
Dillman JR et al. “MRI of Legg-Calve-Perthes Disease.” American Journal of Radiology. 2009;193(5)1394- 1407.
Herman MJ et al. “The Limping Child.” Pediatrics in Review. 2015;36(5)184-197.
Nigrovic PA. “Overview of hip pain and childhood.” https://www.uptodate.com/. Accessed October, 2016.
While evaluating an otherwise asymptomatic, healthy adolescent in clinic, you note an irregular rhythm on exam. Given this finding, you order an EKG (shown below). What do you think?
EKG Courtesy of Life in the Fast Lane: http://lifeinthefastlane.com/ecg-library
Diagnosis: AV block: 2nd degree, Mobitz 1 (Wenckebach Phenomenon)
What is it?
- Progressively lengthening of the PR interval and eventual “dropped QRS” complex, caused by increased refractory period of the AV node . The entire process tends to occur over 3-6 cardiac cycles (Doniger et al, 2006)
- Following “dropped” beat, there is a diastolic pause, and cycle continues
- Characteristically thought of as a “benign rhythm” found in asymptomatic individuals and there is low risk of progressing to third degree heart block. In fact, up to 10% of healthy will episodes of type 1, second degree heart block (Dickinson, 2005).
- However, this may be seen in myocarditis, congenital heart disease, and following cardiac surgery
Created by: Jason Mandell, MD
Reviewed by: Brian Lee, MD
Dickinson DF. “The Normal ECG in Childhood and Adolescence.” Heart. 2005;91:1626-1630
Doniger SJ et al. “Pediatric Dysrhythmias.” Pediatric Clinics of North America. 2006;53(1)85-105
Life in The Fast Lane: http://lifeinthefastlane.com/ecg-library/basics/wenckebach/
A 16 year-old male presents to the ED after a syncopal episode. He currently feels well and wants to go home. Below is his EKG, what do you think?
The above presentation (syncopal event) paired with this EKG is concerning for the Brugada syndrome.
- The Brugada syndrome was first described in 1992 by Drs. Pedro and Josep Brugada in a case report of 8 patient with aborted sudden cardiac death (SCD), though cases of sudden unexplained nocturnal death (SUND) had been described previously in various southeast Asian populations (Juang JM et al, 2004).
- Some estimate that 4-12% of SCD can be attributed to the syndrome, with a higher prevalence in SE Asian populations (Gourraud JB et al, 2016)
- Median age of presentation is 41, though has be reported in neonates through the elderly.
- While many mutations have been described, the Brugada syndrome appears to result from mutations in the Na+ channel, affecting the SCN5A gene (Behere SP et al, 2015)
- Note: Only 20% of patients with Brugada syndrome have a documented mutation in the SCN5A gene. Studies show a autosomal dominant transmission with incomplete penetrance (Juang JM et al, 2004).
- In the original description of the syndrome, the Brugada brothers noted that all patients presented with syncope, with 5/7 progressing immediately to cardiac arrest and the other 2 progressing to cardiac arrest over the next 2 weeks
- Registry data indicates that almost 1/3 present with syncope, 6% with SCD, though >60% were asymptomatic at the time of diagnosis (Probst V et al, 2010)
- NOTE: Registry listed above excluded children, so interpret with caution
- Sustained Polymorphic Ventricular Tachycardia is the most common rhythm noted to precipitate SCD
EKG Findings and Diagnosis
- ST elevation in V1-3 with right bundle block morphology is common, with “coved pattern” in V1-3 negative T wave (referred to as “Type 1;” this is the only pathognomonic EKG pattern)
- In most cases, no reciprocal ST-depression noted (Juang JM et al, 2004).
- EKGs in Brugada syndrome tend to be variable and at times are even normal
- In patients with normal EKG, class Ia or Ic antiarrhythmic drugs ( potently blocks Na channel) may unmask the Brugada pattern (Juang JM et al, 2004).
- Two other patterns have been described, though are considered less significant
- To be considered diagnostic of Brugada syndrome the EKG findings must be combined with the following clinical findings:
- Polymorphic ventricular tachycardia
- ventricular fibrillation
- Family history of early sudden cardiac death (<45)
- Workup must also exclude other causes that lead to ST-elevation (e.g. acute pericarditis, arrhythmogenic right ventricular dysplasia (ARVD), Prinzmetal angina, etc).
- Implantable cardioverter-defibrillator (ICD) is the only strategy to prevent SCD
- Patients should also be advised to avoid alcohol, certain drugs, and treat fevers aggressively (Refaat MM, 2016).
Resident Reviewer: Vanessa Tommey, MD
- Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a multicenter report. J Am Coll Cardiol 1992;20:1391–6
- Juang JM et al. “Brugada syndrome–an under-recognized electrical disease in patients with sudden cardiac death.” Cardiology. 2004;101(4):157-69
- Gourraud JB et al. “The Brugada Syndrome: A Rare Arrhythmia Disorder with Complex Inheritance.” Front Cardiovasc Med. 2016 Apr 25;3:9.
- Behere SP et al. “Inherited arrhythmias: The cardiac channelopathies.” Ann Pediatr Cardiol. 2015 Sep-Dec; 8(3): 210–220.
- Probst V et al. “Long-Term Prognosis of Patients Diagnosed With Brugada Syndrome.” Circulation. 2010;121(5):635-643.
- Alley P. “What is Brugada Syndrome?” http://lifeinthefastlane.com/what-is-brugada-syndrome/
Created on 8/3/2016 by Vanessa Hand, MD
A 17 year-old male in obvious distress is brought the ED by his sister. She states that this morning he woke up with a fever and a sore throat. However, over the next few hours his voice has been changing and is now more “hoarse.” She notes that during this time he also developed difficulty breathing. Below is an x-ray obtained upon presentation. What are you most concerned about?
Case courtesy of Dr Maxime St-Amant, Radiopaedia.org. From the case rID: 26840
Radiology Findings: Typical findings of epiglottitis with enlarged epiglottis and aryepiglottic folds.
Diagnosis: Acute Epiglottitis
- Combination of sore throat, dysphagia, “hot potato” voice and high fevers classically described
- Difficulty with breathing may be most common chief complaint (Mayo-Smith et al, 1995)
- Symptoms progress rapidly, usually over hours (Stroud et al, 2001)
- Physical Exam Shows:
- Vitals: Febrile, Tachypnea
- Visibly Distressed Child; “Tripoding” position
- Muffled or hoarse voice
Epidemiology (Shah at al, 2004)
- Historically caused by H. Influenza type B, however vaccination has largely shifted etiology to other organisms
- Rate dropped from 5/100,000 to 0.6-0.8/100,000 (immunized)
- Increased age of presentation from 3 yo to 6-12 yo
- H. influenzae, penicillin resistant S. pneumoniae, S. Aureus, β-hemolytic strep
- Minimize stimuli, stressful procedures
- Maintain airway, anesthesia/ENT intubate in OR
- Antibiotics: Ceftriaxone or Ampicillin/Sulbactam; add Vancomycin or Clindamycin if concern for MRSA
Faculty Reviewer: Brian Alverson, MD
Mayo-Smith MF et al. “Acute Epiglottitis. An 18-year experience in Rhode Island.” Chest. 1995;108(6):1640-7
Shah RK et al. “Epiglottitis in the Hemophilus influenzae Type B Vaccine Era: Changing Trends.” The Laryngoscope. 2004;114(3): 557-60
Stroud RH et al. “An update on inflammatory disorders of the pediatric airway: epiglottitis, croup, and tracheitis.” Am J Otolaryngology. 2001;22(4):268-275
A 6 month-old male presents to the emergency room with progressively increasing work of breathing. A chest x-ray obtained shows the following:
Case courtesy of Dr Jeremy Jones, Radiopaedia.org. From the case rID: 24086
What are you concerned about?
This X-ray demonstrates cardiomegaly with prominent lung markings, which is suggestive of heart failure as the etiology of this infant’s respiratory distress.
Heart Failure in Children: The Basics
- In the US, the most common cause of pediatric heart failure is structural congenital heart disease. In children with structurally normal hearts, cardiomyopathy is the most common cause of heart failure. [Hsu et al, 2009]
- Heart failure presents when the metabolic demands of the body exceed cardiac output.
- Clinical Presentation is Diverse & Age related. [Madriago et al, 2010]
- Neonates: Feeding Difficulties and ultimately Failure to Thrive
- Older Children: Fatigue, GI symptoms, and exercise intolerance
- Physical Exam:
- “Pulmonary”: Tachypnea, Respiratory Distress (retractions, grunting), crackles on auscultation
- “Cardiac”: Tachycardia, gallop rhythm
- “GI”: hepatomegaly
Faculty Reviewer: Kristin Lombardi, MD
Radiology Reviewer: Laura Sternick, MD
Hsu DT et al. “Heart Failure in Children, Part I: History, etiology, and pathophysiology.” Circ Heart Fail. 2009;2:63-70
Madriago E et al. “Heart Failure in Infants and Children.” Pediatrics in Review. 2010;31(1)4-12.
As you are starting your morning ED shift, your first patient is a 2-year-old boy, previously healthy, who is coming in with 5 days of fevers at home. Fevers have occurred daily and have been as high as 39.5ºC. His mother states that during this time he has been more fussy than usual and has been not been eating or drinking very well. Vital signs demonstrate a temperature of 38.9ºC, HR of 150, RR is 20. His BP is 110/60. On exam, he is fussy and appears ill. His conjunctiva are injected and you appreciate limbic sparing. Oropharyngeal exam is shown below. You also note a diffuse macular rash. What are you most concerned about?
Kole A, Chandakole D. N Engl J Med 2015;373:467-467.
Answer: The picture above shows a strawberry tongue with fissured lips, which in conjunction with 5 days of fever, rash and bilateral conjunctival injection is concerning for Kawasaki Disease (KD). KD is an acute vasculitis of still unknown etiology affecting primarily infants and children. KD affects coronary arteries, and without IVIG, 20% of children will go on to have coronary artery aneurysms (Newburger et al). Diagnosis of KD is primarily clinical, and requires 5 or more days of fever in conjunction with 4/5 clinical criteria (see below). For those children with persistent fever, but who lack all 4 criteria (child mentioned in case has 3), diagnosis utilizes lab and ECHO findings (listed below). In children with classic Kawasaki, as well as those with incomplete disease, the mainstay of acute management is IVIG paired with high-dose aspirin. For more information, please review the attached references.
Diagnostic Criteria of Kawasaki Disease (requires at least 5 days of fever)
- Changes in extremities: Acute: Erythema and edema of hands and feet Convalescent: Desquamation of fingertips
- Polymorphous exanthema
- Bilateral, painless bulbar conjunctival injection without exudate
- Changes in lips and oral cavity: Erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosae
- Cervical lymphadenopathy (≥1.5 cm in diameter), usually unilateral
Diagnostic Criteria for children who have at least 5 days of fever and only 2-3 findings mentioned above:
- Obtain Labs: CRP/ESR, CBC, LFTs, and UA
- If CRP < 3 mg/dl AND ESR <40
- If fever continues, re-evaluate
- If fever defervesces, no follow-up required (EXCEPTION: in children who develop desquamation, an ECHO should be obtained).
- If CRP ≥ 3 mg/dl and/or ESR ≥40, obtain ECHO
- If > 3 supplementary lab findings (See Below), treat with IVIG and high dose ASA
- If < 3 supplementary Findings
- ECHO positive (see below for criteria) –> TREAT
- ECHO Negative
- If fever resolves, Kawasaki is unlikely
- If fever persists, obtain 2nd ECHO, consult specialist
Important Lab or Imaging Findings
- Supplementary Lab Findings (Need 3 or more)
- LFTs: Albumin ≤ 3 g/dL or elevated ALT (> 50 units/L)
- CBC: WBC ≥ 15,000 or PLT≥ 450,000 after 7 days, or anemia for age [normochromic, normocytic]
- UA: ≥10WBC per HPF
- ECHO findings
- Any of the Following
- z score of LAD or RCA ≥2.5
- coronary arteries meet Japanese Ministry of Health criteria for aneurysms
- ≥3 other suggestive features exist:
- perivascular brightness
- lack of tapering
- decreased LV function
- mitral regurgitation
- pericardial effusion,
- zscores in LAD or RCA of 2–2.5
From: AHA Scientific Statement on Kawasaki Disease 2004.
Faculty Reviewer: Erica Chung, MD
- “Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease.” Circulation. 2004; 110(17)2747-2771
- Son MB and Newburger JW. “Kawasaki Disease.” Pediatrics in Review. 2013; 24(4)151-161
- Newburger JW et al. “Kawasaki Disease.” Journal of the American College of Cardiology. 2016;67(14)1738-49.