Peutz-Jeghers症候群の1例

Peutz-Jeghers Syndrome

　Peutz-Jeghers症候群は特有の皮膚，粘膜の色素斑と消化管polyposisのみられる遺伝性疾患である．イレウス症状，腹痛，血便などの消化器症状がみられるが，とりわけ腸重積症は最も多くみられる合併症である．今回われわれは大きさ30mmの空腸polypが原因となり小腸重積症を来したPeutz-Jeghers症候群の1例を経験したので報告する．

　A 10-year-old girl with pigmented spots on her lips was admitted to our hospital due to continuous abdominal pain and vomiting. Abdominal plain x-ray showed a niveau at the umbilical region.

　A segmental resection of the jejunum was performed with a clinical diagnosis of intussusception which revealed one of four polyps obstructing the jejunal canal. Histological study revealed hamartomatous malformation with no malignant lesion. The post-operative clinical course went well, however, a close x-ray examination revealed multiple polyposis over the entire G-I tract.

　The potentiality of malignancy of Peutz-Jeghers hamartomatous polyps has been widely discussed, and recently, several investigators reported that some of hamartomatous polyps showed malignant change in the Peutz-jeghers syndrome. Therefore it is very important to follow up the patients with Peutz-Jeghers polyps carefully.