Baeza-Herrera Carlos, García-Cabello Luis Manuel, Nájera-Garduño Heladio M, Sánchez-Fernández Luis Angel, Mora-Hernández Francisco, Ortiz-Zúñiga Alejandra I
Academia Mexicana de Cirugía, UNAM, Departamento de Cirugía, Hospital Pediátrico Moctezuma, Mexico.
Cir Cir. 2005 Mar-Apr;73(2):91-5.
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.
佩-杰综合征（PJS）是一种家族性疾病，其特征为色素沉着性黏膜皮肤斑和肠道息肉病。肠套叠是最常见的腹部并发症，但非常罕见。在我国，这是第二篇已知报道。在本文中，我们呈现了4例与肠套叠相关的PJS病例。所有患者均为女性，且均接受了外科手术。肠套叠位于回肠和结肠。世界上报道的这两种情况相关的病例不超过20例。患有PJS的儿童患回肠-回肠或空肠-回肠套叠的风险很高，这通常需要特殊的外科手术。
