Cronkhite-Canada综合征2例报告并国内外文献评价

Two cases of Cronkhite-Canada syndrome reports with worldwide literature review

目的总结Cronkhite-Canada综合征(CCS)的临床表现、消化道息肉特点、病理改变及诊断治疗,以提高临床医生对本病的认识。方法对我院诊断明确的2例CCS病例及1990年以来72篇文献报告的31例病例的临床资料回顾性研究。结果符合CCS诊断标准的入组病例33例,男20例,女13例;年龄17～77岁,中位年龄51岁。临床表现为脱发、指(趾)甲改变、皮肤色素沉着、腹部不适及低蛋白血症。全部患者均有消化道多发息肉,累及胃、小肠、大肠。息肉病理无特异性,其中8例癌变。治疗方法包括激素治疗17例,内镜下息肉摘除2例,因癌变、消化道梗阻及蛋白丢失性肠病手术11例(3例同时激素治疗)和其他治疗6例,其中1年内激素治疗有效14例,最长1例随访7年无复发。结论Cronkhite-Canada综合征(CCS)是以消化道多发息肉伴外胚层改变为主要临床特点的综合征,诊断较困难,激素治疗能改善症状,长期疗效仍待进一步研究。

Objective To know more about Cronkhite-Canada syndrome （CCS） by analyzing its clinical manifestation, features of gastrointestinal polyp, pathological changes, diagnosis and treatment. Methods Two cases of CCS in Chinese PLA General Hospital were collected, and a retrospective study on the literature pertaining to the rare syndrome was performed. Results From the observation of 33 cases of CCS, median age was 51 （ 17 - 77） and characteristics of CCS were alopecia, onychodystrophy, cutaneous pigmentation, abdominal discomfort and hypoalbuminemia. For CCS polyps located in stomach, small intestine and large bowel, pathological results were non-specific. Treatments for CCS were mainly consist of corticosteroid therapy for 17 of the cases, endoscopic polypectomy for 2 of the cases, surgery for 11 of the cases due to eancerization, gastrointestinal obstruction and protein-losing enteropathy. With treatment of corticosteroid, 14 cases of CCS relieved obviously without polyp-recurrence following up one year and the longest following up period was seven years. Conclusion CCS is a rare form of nonhereditary gastrointestinal polyposis accompanied by ectodermal changes with poor diagnosis. Corticosteroid can alleviate symptom, yet long-term therapeutic efficacy awaits further study.