Familial Adenomatous Polyposis-Related Desmoids Presenting with Air-Fluid Level: A Clinical Review and Management Algorithm | oneFAPvoice

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Familial Adenomatous Polyposis-Related Desmoids Presenting with Air-Fluid Level: A Clinical Review and Management Algorithm

key information

source: Diseases of the colon and rectum

year: 2012

authors: Santosh Bhandari, Pravin Ranchod, Ashish Sinha, Arun Gupta, Robin K. S. Phillips, Susan K. Clark

summary/abstract:

BACKGROUND : 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.

OBJECTIVE : 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.

DESIGN : This is a retrospective study of prospectively maintained polyposis registry database.

SETTING : This study was conducted at a tertiary referral center specializing in familial adenomatous polyposis and desmoid disease.

PATIENTS : Nine patients with intra-abdominal desmoid and air-fluid level were analyzed for the purpose of this study.

RESULTS : 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.

LIMITATION : This study was limited by the small number of patients.

CONCLUSIONS : 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.

organisation: St Mark's Hospital Harrow

DOI: 10.1097/DCR.0b013e318257fa93

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