Juvenile xanthogranuloma (JXG) is generally a benign, self-limited histiocytic disorder of the skin. We report two cases of multisystem JXG presenting with clinical features more commonly seen in Langerhans cell histiocytosis (LCH), including diabetes insipidus and lytic bony lesions. Histologically, the skin lesions demonstrated a histiocytic dermal infiltrate that stained for CD-68, but S-100 and CD1a stains were negative. Treatment according to LCH-based chemotherapy regimens resulted in prompt resolution of symptoms. A literature review of multisystem JXG cases treated with chemotherapy suggests that symptomatic patients can successfully be treated with LCH-based regimens that include both corticosteroids and vinca alkaloids.

No abstract

Objective To determine the causes of sudden cardiac arrest (SCA) in apparently healthy children in a single center in the era of primary prevention (screening questionnaire, SQ) and secondary prevention (automated external defibrillator, AED and automated implantable cardioverter defibrillator, AICD). Study Design Any child (0–18 year’s age) without prior known disease except for attention deficit disorder who underwent out-of-the hospital cardiopulmonary resuscitation was included in the study as SCA. Using retrospective chart review we evaluated the efficacy of the SQ, electrocardiogram (EKG), chest roentgenogram (CXR) and an echocardiogram. Results We found 44 out of 6,656 children admitted to intensive care with SCA; an AED was used in 39%, AICD placed in 18% and survival to hospital discharge was 50%. The etiology for SCA was identified in 57% of the cases, mostly in those above one year of age and among these the majority of had a cardiac etiology (50%) while 7% had rupture of an arteriovenous malformation. Stimulant medication use was seen in 11% of the SCA. In the best case scenario of hypothesized primary prevention, a prior SQ, CXR, EKG and echocardiogram may have detected 18%, 9%, 23% and 16% of at-risk cases respectively and 32% may have been detected with EKG and SQ together. Based on a historical control cohort, a positive EKG was significantly higher in children with SCA (p = 0.014). Conclusion An EKG along with a screening SQ may be more effective in identifying children who are potentially at-risk for SCA than a SQ alone.

Rupture of aneurysm of sinus of Valsalva into the right atrium mimicking tricuspid valve endocarditis is a rare presentation. We review a case of spontaneous rupture of aneurysm of sinus of Valsalva into the right atrium presenting as a murmur. Transthoracic echocardiogram showed a mobile mass that appeared to be attached to the tricuspid valve leaflet with moderate tricuspid regurgitation suggestive of tricuspid valve endocarditis. The diagnosis was confirmed as spontaneous rupture of noncoronary sinus in to the right atrium by transesophageal echocardiogram. Patient recovered completely after surgical repair.