Ambroze W L, Orangio G R, Lucas G
Department of Surgery, Medical College of Georgia, Atlanta.
Semin Surg Oncol. 1995 Nov-Dec;11(6):423-7. doi: 10.1002/ssu.2980110610.
Familial adenomatous polyposis (FAP) is a genetic disorder transmitted in an autosomal dominant pattern. One-half of members of an affected family will carry the gene, and all carriers will succumb to colon cancer or extracolonic manifestations if not detected and treated early. When the diagnosis is made, surgery is indicated. Surgical options include total proctocolectomy with ileostomy, continent ileostomy, total colectomy with ileorectal anastomosis, and total proctocolectomy with ileal pouch anal anastomosis. Many diverse factors, such as extent of rectal disease, the presence and extend of carcinoma, sphincter function, and extracolonic disease, influence which surgical procedure is most appropriate for the individual patient with FAP. This article reviews the surgical options for treating FAP, with emphasis on specific indications, contraindications, and anticipated outcomes.
家族性腺瘤性息肉病（FAP）是一种以常染色体显性模式遗传的遗传性疾病。受影响家族的一半成员会携带该基因，所有携带者如果不及早发现和治疗，都将罹患结肠癌或结肠外表现。确诊后，需进行手术治疗。手术选择包括全直肠结肠切除术加回肠造口术、可控性回肠造口术、全结肠切除术加回肠直肠吻合术以及全直肠结肠切除术加回肠贮袋肛管吻合术。许多不同因素，如直肠疾病的范围、癌的存在及范围、括约肌功能和结肠外疾病等，都会影响哪种手术方式最适合患有FAP的个体患者。本文回顾了治疗FAP的手术选择，重点关注特定的适应证、禁忌证和预期结果。
