[Unusual complications of the Peutz-Jeghers-syndrome in two consecutive generations of the same family]

Abstract

The Peutz-Jeghers syndrome is an autosomal dominant inherited disease, characterized by the presence of hamartomatous polyposis of the gastrointestinal tract and perioral mucocutaneous pigmentation. The incidence of surgical complications in these patients is relatively rare, and correlates with the size and location of the polyps. We report on two complications of the Peutz-Jeghers syndrome which occurred in two generations of the same family. There was a perforation and an invagination of the small intestine. Both cases were treated by resection of the small intestine.