家族性腺瘤性息肉病相关硬纤维瘤病患者的小肠梗阻。

Small bowel obstruction in patients with familial adenomatous polyposis related desmoid disease.

机构信息

Department of Colorectal Surgery, Sanford R. Weiss Center for Hereditary Colorectal Neoplasia, Digestive Diseases Institute, Cleveland Clinic Foundation, Cleveland, Ohio, USA.

出版信息

Colorectal Dis. 2013 Dec;15(12):1489-92. doi: 10.1111/codi.12416.

DOI:10.1111/codi.12416

PMID:24020802

Abstract

AIM

Intra-abdominal desmoid disease is one of the most common extra-intestinal manifestations of familial adenomatous polyposis. Small bowel obstruction occurs frequently in affected patients and is notoriously difficult to treat. The aim of this study was to review the management and outcome of desmoid-related small bowel obstruction.

METHOD

This was a retrospective, descriptive study of patients with familial adenomatous polyposis and intra-abdominal desmoid disease who developed small bowel obstruction. Demographic data and data concerning the presentation, diagnosis and treatment of the bowel obstructions were abstracted from the polyposis database or patients' records. Patients with obstruction unrelated to desmoid disease were excluded.

RESULTS

There were 47 patients (30 women and 17 men). Median age at first bowel obstruction was 24.2 (interquartile range 19.2-34.2) years. The median time from index surgery to first bowel obstruction was 4.1 (interquartile range 1.5-9.0) years. Twenty-two patients had a colectomy and ileorectal anastomosis and 21 a proctocolectomy and ileoanal pouch. Obstruction was treated medically in 29% of cases and surgically in 69%. Thirteen patients had total parental nutrition. Thirty (63.8%) had a second episode of small bowel obstruction at a mean of 5.3 years after the first, 50% of which were treated medically. Eighteen (37.5%) patients had more than two episodes of bowel obstruction. There were 118 operations, including lysis of adhesions (29), small bowel resection (14), bypass (12), ileostomy (12), desmoid excision (9) and stricturoplasty (2).

CONCLUSION

Desmoid-related small bowel obstruction in familial adenomatous polyposis patients requires multiple surgical strategies to restore a patent gastrointestinal tract. WHAT DOES THIS PAPER ADD TO THE LITERATURE?: This is the only series in the literature specifically addressing small bowel obstruction in patients with familial adenomatous polyposis and intra-abdominal desmoid disease. The data show that small bowel obstruction is common, tends to recur, but can be successfully managed by a combination of medical and well selected surgical treatment.

摘要

目的

腹内纤维瘤病是家族性腺瘤性息肉病（familial adenomatous polyposis，FAP）最常见的肠外表现之一。受影响的患者常发生小肠梗阻，且治疗极具挑战性。本研究旨在回顾与纤维瘤病相关的小肠梗阻的处理和结局。

方法

这是一项回顾性描述性研究，纳入了患有 FAP 和腹内纤维瘤病并发生小肠梗阻的患者。从息肉病数据库或患者记录中提取人口统计学数据以及与肠阻塞的表现、诊断和治疗相关的数据。排除与纤维瘤病无关的梗阻患者。

结果

共有 47 名患者（30 名女性和 17 名男性）。首次发生肠阻塞的中位年龄为 24.2 岁（四分位距 19.2-34.2）。从首次手术到首次发生肠阻塞的中位时间为 4.1 年（四分位距 1.5-9.0）。22 例患者接受了结肠切除术和回直肠吻合术，21 例患者接受了直肠结肠切除术和回肠肛管吻合术。29%的病例采用药物治疗，69%的病例采用手术治疗。13 例患者接受了全胃肠外营养。30 例（63.8%）患者在首次梗阻后平均 5.3 年发生第二次小肠梗阻，其中 50%采用药物治疗。18 例（37.5%）患者发生了两次以上的肠阻塞。共有 118 例手术，包括粘连松解术（29 例）、小肠切除术（14 例）、旁路手术（12 例）、回肠造口术（12 例）、纤维瘤切除术（9 例）和狭窄成形术（2 例）。