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家族性腺瘤性息肉病(FAP)是常染色体显性遗传性疾病,如不手术治疗终将发生癌变,本文报告23例,男12例,女11例,年龄19~62岁,35岁以下10例,有家族史13例,手术时有8例癌变。最小癌变年龄23岁。治疗:行全结肠直肠切除回肠造口术1例,只适用于中低位直肠有恶变或全结肠切除回肠直肠吻合术后直肠发生癌变无法保留肛门括约肌者。行全结肠切除回肠直肠吻合术12例,保留段直肠为6~8cm便于术后复查直肠。适应证为直肠下段腺瘤少并能长期随访。行升结肠切除升结肠直肠吻合术1例。行全结肠切除,直肠部分切除,直肠粘膜剥除共9例,其中回肠末端直接与肛管吻合4例,J—pouch肛管吻合4例,S-pouch肛管吻合1例。随访1~18年,平均97个月,脾曲癌变1例术后3年死亡。回肠直肠吻合术12例中有4例直肠腺瘤再发多次手术切除,其中1例直肠腺瘤第3次再发未接受手术切除而致癌变,经局部切除。术后近期排便功能回肠直肠吻合组优于直肠粘膜剥除组。回肠贮袋有无远期排便功能无差别。作者认为应根据病变的范围、程度、有无恶变、能否长期随访、医生的经验等选择手术治疗方法。只要适应证选择合适即能取得良好的疗效。
Familial adenomatous polyposis (FAP) is an autosomal dominant inherited disease. If no surgical treatment is performed, it will eventually become cancerous. This article reports 23 cases, 12 males and 11 females, aged 19 to 62 years, 35 years old and below 10 There were 13 cases of family history and 8 cases of cancer during surgery. The minimum age of cancer is 23 years old. Treatment: One case underwent total colorectal resection and ileostomy. This was only applicable to patients with malignancy in the middle and low rectum, or rectal cancer after rectal bowel anastomosis and inability to retain the anal sphincter. Twelve patients underwent total colectomy and ileal rectal anastomosis. Reserving segment rectum was 6~8cm for rectal examination. Indications for the lower rectal adenoma can be long-term follow-up. One patient underwent ascending colon resection and ascending colorectal anastomosis. Colon resection was performed, partial rectum was removed, and rectum mucosa was stripped in 9 cases, including 4 cases of anastomosis of the ileum directly with the anal canal, 4 cases of J-pouch anal canal anastomosis, and 1 case of S-pouch anal canal anastomosis. Follow-up 1 to 18 years, an average of 97 months, 1 case of splenic ulcer cancer died after 3 years. Four out of 12 cases of ileojejunostomy had recurrent rectal adenomas undergoing multiple resections. One of the 3 rectal adenomas recurred without surgical resection and resulted in canceration and local resection. Postoperative defecation ileal rectal anastomosis was superior to rectal mucosal debridement. The ileal storage bag had no difference in the long-term defecation function. The authors believe that surgical treatment should be based on the extent, extent, presence or absence of malignancy, long-term follow-up, and doctor’s experience. As long as the indications are appropriate, good results can be achieved.