目的 探讨Peutz-Jeghers综合征(黑斑息肉病)的诊断、监测与治疗特点.方法 对我院收治并误诊的Peutz-Jeghers综合征1例的临床资料进行回顾性分析.结果 本例为女性,13岁时因多发息肉致肠穿孔行肠穿孔修补术,术后病理报告为Peutz-Jeghers综合征.否认家族中有类似病史患者.此次因急性肠套叠及肠穿孔就诊,伴典型的皮肤、黏膜黑斑,经手术治疗后痊愈,病理检查证实为Peutz-Jeghers综合征(小肠错构瘤样息肉).结论 消化道错构瘤样息肉与皮肤色素沉着是黑斑息肉病的特征性表现.本病应定期复查,一旦发现息肉或出现消化道急重症时,应及时在内镜下切除或手术治疗.%Objective To investigate diagnosis, detection and treatment characteristics of Peutz-Jeghers syndrome. Methods Clinical data of 1 misdiagnosed patient with Peutz-Jeghers disease was retrospectively analyzed. Results A female patient, who underwent kposthesis of intestinal perforation for multiple polyps at age 13, received medical treatment because of acute intussusception and intestinal perforation combined with typical skin and mucous black spots. There was no family history. She was cured after surgery and diagnosed as having Peutz-Jeghers syndrome (small intestinal hamartomatous polyposis) with pathological diagnosis. Conclusion Alimentary canal hamartoma polyps and skin pigmentation are typical characteristics of Peutz-Jeghers syndrome. The patients should be regularly reviewed to guard against the occurrence of severe acute digestive system diseases. When there are polyps or clinical symptoms, the patient should undergo endoscopic polyps'resection or surgery.
展开▼
