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Peutz-Jeghers syndrome complicated by intussusception

Case contributed by Mustafa Hammad
Diagnosis almost certain

Presentation

Abdominal pain.

Patient Data

Age: 40 years
Gender: Female

Axial CT demonstrates a “sausage” mass (pseudokidney sign) with central low-attenuation mesenteric fat along with vessels. Coronal CT demonstrates the intussusception mass.

There is duodenal, jejunal, ileal and colonic polyposis, compatible with Peutz-Jeghers syndrome.

Case Discussion

This patient had a known history of Peutz-Jeghers syndrome, which is a rare autosomal dominant condition characterized by mucocutaneous pigmentation and gastrointestinal hamartomatous polyps.

The condition is associated with an increased risk of adenocarcinoma, mainly of the gastrointestinal tract, although the polyps themselves are not premalignant. Many patients experience abdominal symptoms in particular because of obstruction and intussusception.

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