Intussusception secundary to Peutz-Jeghers syndrome
نویسندگان
چکیده
El síndrome de Peutz-Jeghers es una condición familiar caracterizada por la presencia de manchas cutáneas hiperpigmentadas y poliposis intestinal. La invaginación intestinal es la complicación abdominal más frecuente, pero es muy rara. Éste es el segundo reporte que se comunica en nuestro país. En este artículo presentamos cuatro pacientes que sufrieron el síndrome, que se complicó con invaginación intestinal. Todas fueron del sexo femenino e intervenidas quirúrgicamente. La invaginación estuvo localizada en el ileon y el colon. No hay más de 20 casos de esta asociación referidos en la literatura mundial. Los niños que sufren el síndrome de Peutz-Jeghers tienen altas posibilidades de sufrir una invaginación ileo-ileal o yeyuno-ileal, que con mucha frecuencia requerirá de un procedimiento quirúrgico especial.
منابع مشابه
Jejunoduodenal intussusception caused by a solitary polyp in a woman with Peutz-Jeghers syndrome: a case report
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...
متن کاملPeutz-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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BACKGROUND Duodenal Peutz-Jeghers polyp is a rare cause of duodenal or biliary obstruction. However, a sporadic Peutz-Jeghers polyp leading to simultaneous biliary and duodenal obstruction has not been reported. CASE PRESENTATION We report a case of a 25-year-old Sri Lankan woman presenting with features of recurrent upper small intestinal obstruction and biliary obstruction. She had clinical...
متن کامل[Peutz-Jeghers syndrome].
Peutz-Jeghers syndrome (PJS, MIM 175,2000) is a disease of autosomal dominant inheritance that is characterised by hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. In addition to problems such as intussusception, PJS predisposes to cancers of several sites. The unusual combination of clinical features makes the identification of the defect underlying PJS particularly intere...
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