M'Koma A E, Herline A J, Adunyah S E
Department of Biochemistry and Cancer Biology, Meharry Medical College School of Medicine, Nashville, TN ; Department of Surgery, Vanderbilt Medical Center, Nashville, TN ; Vanderbilt-Ingram Cancer Center, Vanderbilt University School of Medicine, Nashville, Tennessee.
Department of Surgery, Vanderbilt Medical Center, Nashville, TN ; Vanderbilt-Ingram Cancer Center, Vanderbilt University School of Medicine, Nashville, Tennessee.
World J Colorectal Surg. 2013;3(2).
Familial adenomatous polyposis (FAP) is an autosomally dominant disease characterized by the early development of colorectal adenomas and carcinoma in untreated patients. Patients with FAP may develop rectal cancer at their initial presentation (primary) or after prophylactic surgery (secondary). Controversies exist regarding which surgical procedure represents the best first-line treatment. The options for FAP are ileorectal anastomosis (IRA) or a restorative proctocolectomy (RPC) with either a handsewn or a stapled ileal pouch-anal anastomosis (IPAA), with or without mucosectomy. The purpose of these surgeries is to stop progression to an adenoma-cancer sequence by eradicating the colon, a disease prone organ. Unfortunately, these surgical procedures, which excise the entire colon and rectum while maintaining transanal fecal continence, do not guarantee that patients still won't develop adenomas. Based on the available literature, we therefore reviewed reported incidences of pouch-related adenomas that occurred post prophylactic surgery for FAP. The review consists of a collection of case, descriptive, prospective and retrospective reports.
To provide available data on the natural history of subsequent adenomas after prophylactic surgery (by type) for FAP.
A review was conducted of existing case, descriptive, prospective and retrospective reports for patients undergoing prophylactic surgery for FAP (1975 - August, 2013). In each case, the adenomas were clearly diagnosed in one of the following: the ileal pouch mucosa (above the ileorectal anastomosis), within the anorectal segment (ARS) below the ileorectal anastomosis, or in the afferent ileal loop.
A total of 515 (36%) patients with pouch-related adenomas have been reported. Two hundred and eleven (211) patients had adenomas in the ileal pouch mucosa, 295 had them in the ARS and in 9 were in the afferent ileal loop. Patients with pouch adenomas without dysplasia or cancer were either endoscopically polypectomized or were treated with a coagulation modality using either a Nd:Yag laser or argon plasma coagulation (as indicated). Patients with dysplastic pouch adenomas or pouch adenomas with cancer had their pouch excised (pouchectomy).
In patients with FAP treated with IRA or RPC with IPAA, the formation of adenomas in the pouch-body mucosa or ARS/anastomosis and in the afferent ileal loop is apparent. Because of risks for adenoma recurrence, a life time endoscopic pouch-surveillance is warranted.
家族性腺瘤性息肉病(FAP)是一种常染色体显性疾病,其特征为未经治疗的患者会早期出现结直肠腺瘤和癌。FAP患者可能在初次就诊时(原发性)或预防性手术后(继发性)发生直肠癌。关于哪种手术方式是最佳一线治疗方案存在争议。FAP的手术选择包括回直肠吻合术(IRA)或采用手工缝合或吻合器行回肠袋肛管吻合术(IPAA)的保留直肠结肠切除术(RPC),可选择或不选择黏膜切除术。这些手术的目的是通过切除结肠这一易患疾病的器官来阻止疾病进展至腺瘤 - 癌序列。不幸的是,这些切除整个结肠和直肠同时维持经肛门排便自控的手术并不能保证患者不会再发生腺瘤。基于现有文献,我们因此回顾了FAP预防性手术后发生的与袋相关腺瘤的报告发病率。该综述包括一系列病例、描述性、前瞻性和回顾性报告。
提供FAP预防性手术后后续腺瘤自然史(按类型)的可用数据。
对1975年至2013年8月接受FAP预防性手术患者的现有病例、描述性、前瞻性和回顾性报告进行了综述。在每个病例中,腺瘤在以下部位之一被明确诊断:回肠袋黏膜(回直肠吻合术上方)、回直肠吻合术下方的肛管直肠段(ARS)或输入袢回肠。
共报告了515例(36%)与袋相关腺瘤患者。211例患者的腺瘤位于回肠袋黏膜,295例位于ARS,9例位于输入袢回肠。无发育异常或癌的袋腺瘤患者接受了内镜下息肉切除术或根据情况采用Nd:Yag激光或氩离子凝固术进行凝固治疗。有发育异常的袋腺瘤或袋腺癌患者接受了袋切除(袋切除术)。
在接受IRA或RPC加IPAA治疗的FAP患者中,袋体黏膜或ARS/吻合口以及输入袢回肠中腺瘤的形成很明显。由于腺瘤复发风险,终身内镜下袋监测是必要的。