Familial adenomatous polyposis-related desmoids presenting with air-fluid level: a clinical review and management algorithm.
ABSTRACT Familial adenomatous polyposis-related desmoid tumors can present with a liquefied center containing gas, accompanied by abdominal pain and sepsis. To date the optimal management of such patients has not been documented.
The aim of this study was to review our experience of managing these desmoids grouped together as "intra-abdominal desmoids with air-fluid level" and present a management algorithm.
This is a retrospective study of prospectively maintained polyposis registry database.
This study was conducted at a tertiary referral center specializing in familial adenomatous polyposis and desmoid disease.
Nine patients with intra-abdominal desmoid and air-fluid level were analyzed for the purpose of this study.
Two hundred and forty-six patients were identified with desmoid tumor. Of these, a total of 9 patients had an intra-abdominal desmoid with air-fluid level; 7 were women. Age range at diagnosis was 20 to 41 years. The median time from primary surgery to desmoid tumor development was 24 months (range, 0-48 months), and the median time for further progression to air-fluid level was 24 months (range, 0-226 months). Desmoid tumor size ranged from 10 cm to greater than 20 cm in diameter. Two patients were successfully managed with antibiotics alone, and 2 patients were managed with percutaneous drainage and antibiotics. The other 5 patients required surgical intervention involving either excision or drainage with or without proximal defunctioning/exclusion. There was a single 30-day mortality.
This study was limited by the small number of patients.
The majority of intra-abdominal desmoids with an air-fluid level require surgical intervention. Antibiotics and percutaneous drainage are only successful in a limited number of patients. We present our current treatment algorithm based on this experience.
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ABSTRACT: Enterocutaneous fistula is a severe complication of intra-abdominal desmoid disease. It is hard to repair because of the presence of the desmoid itself, the possibility of distal obstruction, and the complexity of multiple laparotomies. Here we report the outcome of a series of patients presenting with abdominal desmoid disease and associated enterocutaneous fistula. this is a retrospective, descriptive study of patients presenting to a hereditary colorectal cancer registry with familial adenomatous polyposis-related intra-abdominal desmoid disease and associated enterocutaneous fistulas. Patients were identified through the registry database and aspects of their treatment and outcome were abstracted. 16 patients (11 women, 5 men) were treated. The mean age at index surgery was 25.2 years and mean time to first fistula was 115.6 (+/- 92.7 standard deviation) months from index surgery. Index surgery included restorative proctocolectomy with ileal pouch (n=9), colectomy with ileorectal anastomosis (n=4) and proctocolectomy with end ileostomy (n=1). One patient had only a small bowel bypass and another did not have any index surgery. Ten patients underwent laparotomy for the enterocutaneous fistula; eight had a repair +/- resection, one had a diversion, and one a bypass. All eight patients who had a repair healed and the bypassed fistula was successfully palliated. Three fistulas recurred and two were successfully repaired at a second procedure. One patient was explored but nothing could be done for the fistula. Two surgery patients died of causes unrelated to the fistula. Six patients received medical treatment, four of whom died. Selected desmoid-related enterocutaneous fistulas can be repaired successfully. This article is protected by copyright. All rights reserved.Colorectal Disease 07/2013; · 2.08 Impact Factor