[Cholestasis and vomiting: unusual differential diagnosis in a case of Peutz-Jeghers syndrome]

A 54-year-old patient with painless jaundice and vomiting had been diagnosed with a Peutz-Jeghers syndrome 20 years before.

The blood analysis showed a cholestatic constellation as well as increased transaminases. Sonographic, radiological, endoscopic and histological findings indicated multiple hamartomatous polyps of the Peutz-Jeghers' type in the entire small and large bowel with occlusion of the papilla of Vater and the superior gastrointestinal tract by a big polyp.

After an initial percutaneous transhepatic cholangiographic drainage, Whipple's operation and a segmental resection of small and large bowel were performed. A highly differentiated adenocarcinoma of the duodenum was found in the resected specimen.

This case demonstrates the potentially severe complications of a Peutz-Jeghers syndrome that had been neglected for years.