RESUMO
We report a case of a 16-yr-old white female patient with acute abdominal pain due to visceral involvement of Degos' disease that required extensive small bowel resection. Skin manifestations of her disease had been present for 2 yr before the correct diagnosis. She died as a result of central nervous system involvement from Degos' disease.
Assuntos
Abdome Agudo/etiologia , Enteropatias/etiologia , Dermatopatias Papuloescamosas/complicações , Dermatopatias Vasculares/complicações , Adolescente , Doenças do Sistema Nervoso Central/etiologia , Evolução Fatal , Feminino , Humanos , Enteropatias/cirurgiaRESUMO
We describe a young patient with a family history of hereditary pancreatitis who developed extensive pancreatic necrosis complicated by pancreatic ascites. Because of failure of medical management, he was successfully treated with operative necrosectomy and primary wound closure over peripancreatic drains. A postoperative low-output pancreaticocutaneous fistula resolved with time. Pancreatic ascites, as a result of pancreatic duct disruption, is more common in chronic rather than acute pancreatitis and is exceedingly uncommon in the context of necrotizing pancreatitis. When it complicates the latter, treatment should be guided by the principles of management of necrotizing pancreatitis. However, when true pancreatic ascites persists, the pancreatic duct anatomy and site of leak should be defined with endoscopic retrograde pancreatography (ERP). Treatment options include endoscopic duct dilatation and stent placement (if a stricture exists proximal to the leak), onlay pancreaticojejunostomy, or distal pancreatectomy (especially if the leak is located in the distal pancreas or in an enterically isolated distal pancreas).