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

Abstract

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. 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. 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). 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.