Peutz-Jeghers症候群：病例報告

Peutz-Jeghers Syndrome: Case Report

林棋民(Chyi-Min Lin) ； 蔡鶴峰(Ho-Fon Tsai) ； 吳志順(Chih-Shun Wu) ； 馬鴻均(Hung-Chun Maa)

《Journal of Medical Sciences》 16卷3期 (1995/12) Pp. 182-188

摘要

Peutz-Jeghers症候群包括粘膜及皮膚色素沈積、腸胃道多發性息肉及具遺傳傾向。黑色素沈積可早在出生或幼年時出現在嘴唇、口腔粘膜、鼻子、眼睛、手指、腳趾、肛門周圍及外陰。腸胃道息肉則是一種缺陷瘤。Peutz-Jeghers症候群偶爾會發生大腸癌或十二指腸癌，其發生率約2－3%。此症候群通常只須內科治療並不主張外科手術，除非有頑固性出血，腸阻塞或套疊時才做外科局部切除。我們報告一21歲病例自四歲時就有嘴唇、口腔粘膜、臉部、手指、足趾黑色素沈積及間歇性腹痛病史。胃鏡、大腸鏡、鋇劑、上腸胃道及小腸攝影檢查，顯示自食道至直腸均有多發性息肉。息肉切除病理診斷是缺陷瘤。經內科保守治療，病人臨床症狀獲得改善。

關鍵字

Peutz-Jeghers症候群 ； 黑色素沈積 ； 息肉 ； 缺陷瘤 ； 息肉切除

並列摘要

The features of the Peutz-Jeghers syndrome are pigmentation, multiple intestinal polyps and a familial incidence. Melanin depositions are present from birth or early childhood. They are almost always present on the lips, buccal mucosa, noses, eyes, fingers, palms, toes, perianal and genital regions. The gastrointestinal polyps in Peutz-Jeghers syndrome are hamartomas. Carcinoma of the colon and duodenum has been reported in patients with Peutz-Jeghers syndrome, but the incidence appears to be no more than 2-3%. Surgical treatment should be as conservative as possible. It is required for intractable bleeding, intestinal obstruction or intussuception. We reported a 21-year-old male with intermittently abdominal pain and pigmentation over lips, buccal mucosa, noses, fingers and toes since 4 years old. Multiple gastro-intestinal polyps from esophagus to rectum were found by fiberoptic gastro-duodenoscope, fiberoptic colonoscope, X-rays studies of Barium enema, upper gastrointestine and small intestine. Polypectomy was done. The pathological reports were hamartomatous polyps. He received medical treatment with good response.

並列關鍵字

Peutz-Jeghers syndrome ； plgmentation ； polyp ； hamartoma ； polypectomy