At. Gentile et al., COMMON BILE-DUCT OBSTRUCTION RELATED TO INTESTINAL POLYPOSIS IN A CHILD WITH PEUTZ-JEGHERS SYNDROME, Journal of pediatric surgery, 29(12), 1994, pp. 1584-1587
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.