Surgical aspects of intussusception secondary to Peutz-Jeghers syndrome.

Peutz-Jeghers syndrome (PJS) is a familial condition characterized by the presence of pigmented mucocutaneous spots and intestinal polyposis. Intussusception is the most frequent abdominal complication, but it is very rare. In our country, this is the second known report. In this article we present four cases of PJS associated with intussusception. All the patients were female and all underwent a surgical procedure. The intussusception was located in the ileum and colon. There are no more than 20 reported cases in the world where these two entities are associated. Children with PJS have a high risk of suffering from ileo-ileal or jejuno-ileal intussusception that will frequently require a special surgical procedure.