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Multiple Intussusceptions as Primary Manifestation of Peutz-Jeghers Syndrome: Report of a Case

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Author(s): Mohammad-Reza Motie | Ahmad Amouzeshi | Ali Najib Jalali

Journal: Iranian Journal of Pediatrics
ISSN 2008-2142

Volume: 19;
Issue: 3;
Start page: 313;
Date: 2009;
Original page

Keywords: Peutz-Jeghers syndrome | Multiple intussusceptions | Appendiceal intussusception | Hamartomatous polyps

ABSTRACT
Background: Peutz-Jeghers syndrome is a rare hereditary disorder characterized by hamartomatous polyps in the gastrointestinal tract and typical pigment lesions. It is a rare cause of multiple intussusceptions. Previous studies on Peutz-Jeghers syndrome reported only one case of multiple intussusceptions. We describe a case of appendiceal and multiple small intestine intussusceptions presenting as peritonitis in a patient with Peutz-Jeghers syndrome. Case Presentation: A 17-year-old girl presented with an 8 day history of a sharp, non-radiating periumbilical pain. She underwent surgery with the diagnosis of peritonitis. Intraoperative findings included appendiceal and multiple small intestine intussusceptions. The final pathological evaluation of the specimen confirmed the diagnosis of Peutz-Jeghers syndrome. Conclusion: Multiple intussusceptions may occur as the primary manifestation of Peutz-Jeghers syndrome. Because of its complications, in view of the presence of multiple polyps, early intervention is strongly recommended.
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