黑斑息肉病31例临床分析  

Clinical analysis of 31 cases of Pcutz-Jeghers syndrome

作　　者：崔潇龙[1] 黄河[1] 席红卫[2] 郭云童[1] 齐自宁[1] 赵瑛[1] 

机构地区：[1]山西医科大学第一医院普外科,太原030001 [2]山西省儿童医院

出　　处：《中国综合临床》2017年第10期923-926,共4页Clinical Medicine of China

摘　　要：目的 探讨黑斑息肉病患者的临床特点、并发症及治疗方法和预后.方法 选择我院1998年1月至2016年12月收治的31例黑斑息肉患者的临床资料进行回顾性分析.结果 本组11例（35%,11/31）有明确家族史.皮肤黏膜色素沉着21例（67.7%,21/31）;胃肠道多发息肉31例（100%, 31/31）,其中癌变8例（25.0%,8/31）;消化道出血24例（77.4%,24/31）;肠套叠20例（64.5%,20/31）;肠穿孔1例（3.2%,1/31）.6例行内镜高频电凝切除术,12例行息肉切除术,17例行病变肠管切除术.3例顽固性肠套叠者行小肠排列术,4例结肠癌变者行全结肠切除术＋所属淋巴结清扫术.结论 黑斑息肉病常诱发顽固性肠梗阻和胃肠道肿瘤,应积极切除病变肠管;对于顽固性小肠套叠,小肠排列术具有一定价值;对于其潜在恶变可能,应严格监控、积极治疗,基因治疗和药物治疗将是未来发展方向.Objective To investigate the clinical features,complications,treatment and prognosis of patients with Pcutz-Jeghers syndrome.Methods The clinical data of thirty-one cases of Pcutz-Jeghers syndrome treated in the First Hospital of Shanxi Medical University from January 1998 to December 2016 was analyzed retrospectively.Results There were11 cases with definite family history（35%,11/31）.There were 21 cases of mucocutaneous pigmentation（67.7%,21/31）,31 cases of gastrointestinal polyps（100%,31/31）, including 8 cases of canceration（25.0%,8/31）,24 cases of digestive tract bleeding（77.4%,24/31）,20 cases of intussusception（64%,20/31）,and 1 case of intestinal perforation（3.2%,1/31）.6 cases were treated with endoscopic high frequency electrocoagulation,12 cases received polypectomy,17 cases underwent resection of intestinal tract.3 patients with intractable intussusception underwent plication of small intestine,and 4 patients with colon cancer underwent total colectomy combined with lymph node dissection.Conclusion Peutz-Jeghers syndrome often induce intractable intestinal obstruction and gastrointestinal tumor,resection of intestinal lesions should be favorable.For small intractable intussusception,the plication of small intestine has certain value; for its potential malignancy,it should be strictly monitored and actively treated.Gene therapy and drug therapy will guide for the future development.

关 键 词：黑斑息肉病 肿瘤 肠排列术 

分 类 号：R656[医药卫生—外科学]