Juvenile xanthogranuloma: diverse presentations of noncutaneous disease.

Juvenile xanthogranulomas (JXGs) are benign cutaneous lesions of childhood that often spontaneously involute. They rarely present as a noncutaneous tumors. However, JXG tumors have been described in numerous noncutaneous anatomic sites, presenting with a variety of symptoms. The severity of symptoms and accurate preoperative diagnosis of JXG should determine operative and nonoperative treatment options of these uncommon, benign, and self-limiting tumors. We report 3 cases of symptomatic, noncutaneous JXG from disparate anatomic sites all treated with aggressive surgical resection.

Complex spontaneous bile duct perforation: an alternative approach to standard porta hepatis drainage therapy.

INTRODUCTION: Spontaneous perforation of the bile duct (SPBD) is a rare abnormality of the extrahepatic biliary tree in infants. Limited porta hepatis exploration and drainage of the bile-soiled peritoneum are often sufficient treatment. Here, we describe three cases of SPBD, one of which required complex multi-disciplinary interventions for restoration of biliary continuity. METHODS: Three infants with bilious ascites from spontaneous biliary perforation were reviewed. Metrics included age, presenting symptoms, diagnostic tests, interventions performed, and outcomes. RESULTS: The presenting symptoms of all three infants were bilious ascites, sepsis, lethargy, anorexia, fever and persistent emesis. SPBD was confirmed pre-operatively by HIDA scan (hepatobiliary scintigraphy) in each case. Intra-operative, trans-cholecystic cholangiogram confirmed SBDP in two cases. Two of the infants were successfully treated with placement of a cholecystostomy tube and porta hepatis drains. The third infant, having failed multiple similar drainage procedures, required percutaneous transhepatic drainage of a persistent porta hepatis biloma. Ultimately percutaneous, transhepatic cannulation of the extra-hepatic biliary tree for prolonged stenting was required to successfully treat this biliary perforation. CONCLUSION: Complex spontaneous biliary perforation may require extensive interventions if the perforation fails to resolve with standard porta hepatis drainage. Access of the biliary tree via Interventional Radiology procedures for complex biliary disease of this type is novel and presents an alternative to traditional open surgical treatment and control of spontaneous biliary perforations.