Surgical aspects of intussusception secondary to Peutz-Jeghers syndrome.
نویسندگان
چکیده
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.
منابع مشابه
Peutz-Jeghers syndrome: a new understanding.
Peutz-Jeghers syndrome is an autosomal dominant inherited disorder characterized by hamartomatous polyps in the small bowel and mucocutaneous pigmentation. Patients with Peutz-Jeghers syndrome often present as surgical emergencies with complications of the polyps, such as intussusception, bowel obstruction and bleeding. Furthermore, repeated operations may be needed in some patients, which may ...
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INTRODUCTION Peutz-Jeghers syndrome is a rare autosomal dominant disorder characterized by hamartomatous polyps and characteristic mucocutaneous pigmentation. The hamartomatous polyps of Peutz-Jeghers syndrome can cause intestinal occlusion, especially in the small intestine. Intussusception is seen frequently in children, but rarely in adults. CASE PRESENTATION We present the case of a 21-ye...
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Hamartomatous polyps of Peutz-Jeghers are mostly found in patients affected by Peutz-Jeghers syndrome (PJS), but they can be rarely encountered in the general population. It is unclear whether a solitary Peutz-Jeghers polyp (PJP) is an incomplete form of PJS or a separate entity. We report a case of solitary PJP in a paediatric patient in whom the other features of PJS were absent. The patient ...
متن کاملPeutz-Jeghers syndrome: an unusual cause of recurrent intussusception in a 7-year-old boy.
Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps in association with mucocutaneous pigmentations. Main symptoms of PJS in childhood are abdominal pain, obstruction, intussusception, and bleeding from hamartomatous polyps. PJS carries a high risk of gastrointestinal cancer with advancing years. Although, intussusception has...
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ورودعنوان ژورنال:
- Cirugia y cirujanos
دوره 73 2 شماره
صفحات -
تاریخ انتشار 2005