Polysplenia syndrome revealed in adulthood by pancreatic and vascular malformations: a case report.

Polysplenia syndrome (PSS) is a rare congenital disease that associates multiple spleens to other malformations, most frequently cardiac, vascular, visceral, and biliary malformations. Most patients with PSS die in the early neonatal period because the disease is often accompanied by serve cardiac and biliary abnormalities. However, some patients have only mild cardiovascular malformations or anomalies in the abdominal organs, which are typically diagnosed incidentally in adulthood. We report the case of a 54-year-old woman who consulted for chronic atypical diffuse abdominal pain. The clinical examination was normal. Abdominal computed tomography showed a total of 5 spleens with vascular and pancreatic malformations as part of polysplenia syndrome. Symptomatic treatment was instituted with good evolution. No specific therapeutic indication was indicated in our case discovered incidentally in adulthood.

Endoscopic retrograde cholangiopancreatography in ruptured liver hydatid cyst.

One of the most common and serious complications of hepatic hydatid cyst disease is communication between the cyst and the biliary tree. Surgical management of biliary fistula is associated with high morbidity and mortality. We retrospectively reviewed the effectiveness of endoscopic treatment of ruptured hydatid cyst into intrahepatic bile ducts. Diagnosis of intrabiliary rupture of hydatid cyst was mostly suspected by acute cholangitis, jaundice, pain, and/or persistent external biliary fistula after surgery. The diagnosis was confirmed by radiology and endoscopic retrograde cholangiopancreatography (ERCP) findings. We retrospectively reviewed clinical, laboratory, imagery, and ERCP findings for all patients. The therapeutic methods performed were endoscopic sphincterotomy, extraction by balloon or Dormia basket, stenting, or nasobiliary drainage. Sixteen patients with ruptured hepatic hydatid cyst into bile ducts were seen in 9 years. Nine of 16 patients had a surgical history of hepatic hydatid cyst and three patients had a percutanous treatment history. We carried out ERCP with sphincterotomy and extraction of hydatid materials (extraction balloon n = 11; Dormia basket n = 5) or biliary drainage (nasobiliary drainage n = 1; biliary stenting n = 1). The fistula healed in 80 % of patients with a median time of 6 weeks [range, 1-12] after endoscopic treatment. ERCP was an effective method of treatment for hepatic hydatid cyst with biliary fistula.