COMMON BILE-DUCT OBSTRUCTION RELATED TO INTESTINAL POLYPOSIS IN A CHILD WITH PEUTZ-JEGHERS SYNDROME

Peutz-Jeghers syndrome is characterized by hamartomatous polyposis of the small and large bowel and mucocutaneous pigmentation. The authors describe a 9-year-old girl with small bowel obstruction related to duo denal intussusception caused by polyposis in the fourth portion of the duodenum. Operative reduction of the intussusception and excision of the polyps were performed, at which time the pancreas appeared to have mild pancreatitis. A liver biopsy specimen showed mild portal fibrosi s and ductal proliferation. The patient did well postoperatively, but later presented with symptoms consistent with biliary obstruction. Per cutaneous transhepatic cholangiography showed pancreatic and biliary d uct dilatation as well as obstruction of the common bile duct, which e xtended into the left upper quadrant. Exploration showed ampullary obs truction several centimeters proximal to the line of resection. Sphinc teroplasty was performed. The postoperative course was uncomplicated. The authors conclude that Peutz-Jeghers syndrome with polyps in the du odenum can markedly distort duodenal and ductal anatomy and can lead t o ampullary obstruction.