家族性腺瘤性息肉病外科手术方式的选择

The choise of the type of surgical operation for the patients with FAP

目的 探讨家族性腺瘤性息肉病 (FAP)手术方式的选择和效果。方法 对 17例 FAP的临床资料进行回顾性分析。结果 17例病人中良性 13例 ,癌变 4例。全部病人均施行了全结肠切除 ,采用回肠造口 1例 ,回直肠吻合 2例 ,直肠切除、回肠肛管吻合或回肠储袋、回肠肛管吻合 7例 ,部分直肠切除、直肠粘膜剥脱经直肠肌鞘回肠肛管吻合 7例。并发症 2例 (肠坏死 1例 ,吻合口瘘 1例 )。随访 2～ 2 0年 ,良性病人术后均存活 ,其中 2例发生储袋炎 ,1例术后 5年发生癌变。4例癌变病人中 2例因广泛转移死亡 ,另 2例仍存活。排便功能以回肠造口术较差 ,保留直肠的术式较好。结论 比较各种术式的利弊 ,选择全结肠切除 ,保留直肠肌鞘 ,回肠储袋 。

Objective To explore the choice and effect of surgical management of FAP.Methods 17 patients with FAP who had undergone total colectomy were analyzed retrospectively.Results In 13 patients with benign and 4 patients with malignant adenomatous polyposis,their average age at operation was 23.8 years old.All patients underwent total colectomy,with ileostomy(n=1),ileorectostomy(n=2),ileoanal anastomosis or ileal pouch anal anastomosis(n=7),and rectal mucosectomy,through the muscular sheath of rectum ileoanal/ileal pouch anal anastomosis(n=7) respectively.Postoperation complication included anastomotic fistula(n=1),enteric necrosis(n=1).Follow up time was two to twenty years.All benign patients survived but two had pouchitis and one had canceration in 5 years of postoperation.Two forth malignent patients died of distance metastasis,another two patients survived.It was good in the operation of preserving rectum and poor in ileostom for bowel frequency and quality continence.Conclusions Comparing the advantages and disadvantages of various operations,it was an excellent surgical option to undergo total colectomy,preserving muscular sheath of the rectum ileal pouch anal anastomosis for patients with FAP.[