Duodeno-biliary obstruction in Peutz-Jeghers syndrome

Peutz-Jeghers syndrome (PJS) is a pathology with autosomal dominant inheritance characterized by the presence of hamartomatous polyposis and mucocutaneous pigmentation. We present a case report from the Hospital General of Mexico.

We present the case of a 28-year-old male. During physical examination we noted hyperpigmented dermatosis of the oral mucosa and lips. The same condition was seen in both palms. The condition evolved with intolerance to oral feeding and progressive obstructive jaundice. Panendoscopy reported pangastric sessile polyps, as well as being pylorus passable. In the second duodenal portion occupying the region of the ampoule of Vater was a sessile polyp that deformed the region. Exit of bile was not observed through the ampoule. Ultrasound and computed tomography of the abdomen corroborated dilatation of the extrahepatic biliary tract. Two endoprostheses were placed in the bile duct by endoscopic cholangiography, with improvement of biliary obstruction. Roux-en-Y astrojejunoanastomosis was performed because of obstruction of the duodenum by polyps between the second and third portion. Jejunal enterotomy was necessary because of the presence of intraluminal injury formed by a conglomerate of polyps. The patient had a satisfactory evolution. Pathological study reported hamartomatous polyps.

Duodenal obstruction secondary to biliary tract obstruction is a rare manifestation associated with PJS. In these cases, the treatment of choice is polyp resection using endoscopic and/or surgical approach as well as management of the biliary tract obstruction.