Ungewöhnliche Komplikationen des Peutz-Jeghers-Syndroms in zwei Generationen derselben Familie

Zusammenfassung

Das Peutz-Jeghers-Syndrom stellt eine seltene, autosomal dominant vererbte Erkrankung dar, die durch eine hamartomatöse Polyposis des Gastrointestinaltraktes und Pigmentierung der Schleimhaut charakterisiert wird. Komplikationen bei diesen Patienten sind relativ selten und abhängig von der Größe und Lokalisation der Polypen. Wir berichten über zwei Komplikationen des Peutz-Jeghers-Syndroms in zwei Generationen derselben Familie. Es handelte sich um eine Dünndarm-Perforation und eine Invagination. Beide Fällen wurden mit einer Dünndarmresektion behandelt.

Summary

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.

Schlüsselwörter

Peutz-Jeghers-Syndrom - Komplikationen - Perforation - Invagination

Key words

Peutz-Jeghers syndrome - Complications - Perforation - Invagination

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