家族性腺瘤性息肉病的手术治疗:附45例报告

目的　总结家族性腺瘤性息肉病 (FAP)的临床诊治经验和教训。方法　回顾性分析手术治疗 45例FAP的临床病例资料。行全大肠切除回肠造瘘术 2 5例 ,次全结肠切除 直肠息肉电灼术15例 ,结肠全切除、直肠黏膜剥除、回肠经直肠肌鞘脱出及回肠肛管吻合术 5例。结果　患者首诊平均年龄 3 3 .5岁 ,3 5例有家族史。便血、腹泻、腹痛是常见临床表现。息肉数量均在 10 0个以上。术前漏误诊 10例 (2 2 .2 % )。癌变者 2 8例 (62 .2 % ) ,未癌变者 10例 (2 2 .2 % ) ,不典型增生 7例 (15 .6% )。癌变组与未癌变组间年龄相差有显著意义 (P <0 .0 5 )。 40例获定期复查随访。无癌变者存活 2～ 2 0a ;肝转移是癌变者死亡的主要原因 ,其存活时间 2 .5～ 4.5a。结论　FAP缺乏特征性临床表现 ,容易漏诊误诊 ;根据患者具体情况 ,早期手术治疗是防止FAP癌变的关键。

Surgical treatment of familial adenomatous polyposis:a report of 45 cases

Objective To summarize the clinical data of 45 cases of familial adenomatous polyposis(FAP) and the experiences of treatment and diagnosis of FAP. Methods Retrospectively analyzed of the clinical data was made on 45 FAP patients.The choice of operative procedure included total colectomy ileostomy in 25 cases,subtotal colectomy rectal polyposis electrocautery in 15,and total colectomy with ileo-anal anastomosis in 5. Results Average age of patients at first diagnosis was 33.5 years. A family history was found in 35 patients. Bleeding, diarrhea, pain are common clinical presentations, and the numbers of polyposis were all more than 100. All patients without malignant change survived for 3-21years;and metastasis of liver was the main cause of death in patients with malignant change. There were age differences between malignant and non malignant group. Conclusions FAP lacks typical clinical presentation, and misdiagnosis is not uncommon. Surgical therapy should be done on an individual basis and according to the actual condition of patients.