家族性腺瘤性息肉病相关硬纤维瘤病中的输尿管梗阻。

Ureteric obstruction in familial adenomatous polyposis-associated desmoid disease.

机构信息

Sanford R. Weiss Center for Inherited Colorectal Neoplasia, Digestive Disease Institute, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.

出版信息

Dis Colon Rectum. 2010 Mar;53(3):327-32. doi: 10.1007/DCR.0b013e3181c52894.

DOI:10.1007/DCR.0b013e3181c52894

PMID:20173481

Abstract

PURPOSE

Intra-abdominal desmoid disease is the second leading cause of death in familial adenomatous polyposis patients. The aim of this study was to identify the incidence, management, and outcomes for familial adenomatous polyposis associated intra-abdominal desmoids causing ureteric obstruction.

METHODS

Clinical data were abstracted from an institutional review board-approved, prospectively maintained familial polyposis registry.

RESULTS

Of 107 patients identified with familial adenomatous polyposis related desmoid disease, 30 (28%) had documented CT scan evidence of ureteric obstruction. There was a 1:2.3 female predominance. Preceding surgery was the most prominent risk factor for development of desmoid disease (28 of 30 patients); 2 patients were diagnosed with desmoids before abdominal surgery. Overall, 11 patients had ureteric obstruction at the time of diagnosis. In the other 19 patients, median time from desmoid diagnosis to ureteric obstruction was 2 years. Pharmacologic management alone was effective in 8 patients. Eighteen patients (60%) underwent retrograde ureteric stent insertion. Five patients (17%) required percutaneous nephrostomy tubes. Three patients (10%) underwent autotransplant of 4 kidneys, and 4 patients (13%) required nephrectomy. One patient underwent ureterolysis, and another underwent ureteric resection with reimplantation. One-third of patients required more than one urologic procedure, and 63% had extensive small-bowel involvement with desmoid.

CONCLUSIONS

The majority of patients with familial adenomatous polyposis associated desmoid disease who develop hydronephrosis require stenting. Complete obstruction may necessitate a nephrostomy. Renal autotransplant is an option for persistent symptomatic obstruction. Physicians treating patients with familial adenomatous polyposis and desmoid disease must be aware of the potential for development of ureteric obstruction and available treatment options.

摘要

目的

腹内型硬纤维瘤病是家族性腺瘤性息肉病患者的第二大死亡原因。本研究旨在确定家族性腺瘤性息肉病相关的腹内型硬纤维瘤病导致输尿管梗阻的发病率、处理方法和结局。

方法

临床数据从机构审查委员会批准的、前瞻性维护的家族性息肉病登记处中提取。

结果

在 107 例诊断为家族性腺瘤性息肉病相关硬纤维瘤病的患者中，有 30 例（28%）有明确的 CT 扫描证据表明存在输尿管梗阻。女性患者明显居多（男女比为 1:2.3）。先前的手术是发生硬纤维瘤病的最显著危险因素（30 例患者中有 28 例）；2 例患者在腹部手术前就被诊断为硬纤维瘤病。总体而言，11 例患者在诊断时就存在输尿管梗阻。在另外 19 例患者中，从硬纤维瘤病诊断到输尿管梗阻的中位时间为 2 年。单独药物治疗对 8 例患者有效。18 例患者（60%）接受了逆行输尿管支架置入术。5 例患者（17%）需要经皮肾造口管。3 例患者（10%）接受了 4 个肾脏的自体移植，4 例患者（13%）需要进行肾切除术。1 例患者接受了输尿管松解术，另 1 例患者接受了输尿管切除和再植术。三分之一的患者需要进行不止一次的泌尿科手术，63%的患者存在广泛的伴有硬纤维瘤的小肠受累。