Tulchinsky Hagit, Keidar Andrei, Strul Hana, Goldman Gideon, Klausner Joseph M, Rabau Micha
Colorectal Unit, Department of Surgery B, Tel Aviv Sourasky Medical Center, affiliated with the Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
Arch Surg. 2005 Feb;140(2):159-63; discussion 164. doi: 10.1001/archsurg.140.2.159.
Extracolonic manifestations have a major effect on the morbidity and mortality of patients with familial adenomatous polyposis following proctocolectomy.
Case review study.
Colorectal unit, university-affiliated hospital.
Fifty patients (25 males and 25 females) with familial adenomatous polyposis WHO underwent proctocolectomy between January 1988 and October 2003.
Ileal pouch-anal anastomosis (n = 41), Kock pouch (n = 1), end ileostomy (n = 6). Two patients underwent total colectomy with an ileorectal anastomosis.
Clinical follow-up and telephone interview; contact with clinicians following up patients elsewhere.
The patients' median age at surgery was 33 years. The mean length of follow-up was 74 months. Four patients were lost to follow-up. Extracolonic manifestations were diagnosed in 38 patients (76%). Twelve patients had 14 desmoid tumors: 7 were treated surgically and 7 medically (these patients received celecoxib and tamoxifen citrate therapy). Of the 41 patients who underwent upper gastrointestinal tract endoscopy, 11 developed duodenal and/or ampullary adenomas. Three patients had endoscopic polypectomy and 1 underwent a Whipple operation. Among the 29 patients who underwent pouchoscopy, 5 had pouch adenomas and 3 had adenomas that were found in the rectal stump. Two patients died--one of a huge mesenteric desmoid tumor and the other of an aggressive mesenteric malignant fibrous histiocytoma.
Long-term morbidity and mortality were strongly related to the development of mesenteric tumors and ampullary-duodenal polyps. Early detection of desmoid tumors, duodenal, pouch, and rectal cuff adenomas by periodic computed tomography, gastroduodenoscopy, and pouchoscopy, respectively, may allow control by medical therapy, endoscopy, or limited surgical procedures. In most patients control of desmoid tumors was achieved using a combination of celecoxib and tamoxifen citrate therapy.
结外表现对家族性腺瘤性息肉病患者直肠结肠切除术后的发病率和死亡率有重大影响。
病例回顾研究。
大学附属医院的结直肠科。
50例家族性腺瘤性息肉病患者（25例男性和25例女性），于1988年1月至2003年10月期间接受了直肠结肠切除术。
回肠袋肛管吻合术（n = 41），Kock袋（n = 1），末端回肠造口术（n = 6）。两名患者接受了全结肠切除术并进行了回肠直肠吻合术。
临床随访和电话访谈；与在其他地方对患者进行随访的临床医生联系。
患者手术时的中位年龄为33岁。平均随访时间为74个月。4例患者失访。38例患者（76%）被诊断有结外表现。12例患者有14个硬纤维瘤：7例接受了手术治疗，7例接受了药物治疗（这些患者接受了塞来昔布和枸橼酸他莫昔芬治疗）。在41例行上消化道内镜检查的患者中，11例发生了十二指肠和/或壶腹腺瘤。3例患者接受了内镜下息肉切除术，1例接受了惠普尔手术。在29例行袋内镜检查的患者中，5例有袋腺瘤，3例在直肠残端发现腺瘤。2例患者死亡——1例死于巨大的肠系膜硬纤维瘤，另1例死于侵袭性肠系膜恶性纤维组织细胞瘤。
长期发病率和死亡率与肠系膜肿瘤和壶腹-十二指肠息肉的发生密切相关。分别通过定期计算机断层扫描、胃十二指肠镜检查和袋内镜检查早期发现硬纤维瘤、十二指肠、袋和直肠残端腺瘤，可能通过药物治疗、内镜检查或有限的外科手术进行控制。在大多数患者中，使用塞来昔布和枸橼酸他莫昔芬联合治疗实现了对硬纤维瘤的控制。
