家族性腺瘤性息肉病17例的诊断与外科治疗

目的：总结家庭性腺瘤性息肉病（FAP）的诊断和外科治疗经验。方法：回顾分析17例FAP的临床病理资料。结果：17例患者中男7例，女10例，平均年龄23．8岁，良性13例，平均年龄19．6岁，癌变4例，平均年龄37．8岁，13例有家庭史。家系调查发现1个家系至少有2例患者，最多可达7例，其中，6个家系中有7人已死于息肉癌变，全部患者均施行了全结肠切除，并分别采用回肠造口，回肠储袋，回肠肛管吻合等术式，随访2－20年，13例良性患者均存活。其中1例术后5年发生癌变，癌变者中1例术后5年发生肺部转移，行肺叶切除术后2年死亡，1例术后3年因广泛转移死亡，另2例为术后2年和6年，仍存活 。结论：FAP易癌变应尽早手术，手术宜选全结肠切除，直肠粘膜剥脱，回肠储袋，回肠肛管吻合术。

Diagnosis and surgical treatment of familial adenomatous polyposis

ObjectiveTo summarize the experience in the diagnosis and surgical treatment of familial adenomatous polyposis(FAP).MethodsSeventeen patients with FAP who had undergone total colectomy were analyzed retrospectively. ResultsThe average age of 13 benign patients was 19.6 years and that of 4 malignant was 37.8 years. 13 patients had positive family history,with a familial incidence of 2 to 7 cases. Seven familial members out of 6 families had died of colorectal cancer. All patients underwent total colectomy plus ileostomy or ileorectostomy/ileoanal pouch anastomosis. All benign patients are survival at follow up but one had canceration 5 years postoperation.Two of 4 patients with canceration died of distant metastasis.and the remaining two patients were still alive at follow up two and six years respectively postoperation. Conclusion FAP has strong proved tendency of canceration unless total colon was resected. Total colectomy,mucosal resection of the rectum and ileal pouch anal anastomosis is the choice of therapy for FAP.