CLINICAL CASES   

Chirurgia 2006 April;19(2):175-9

Copyright © 2006 EDIZIONI MINERVA MEDICA

language: English

Reconstruction of the abdominal wall following its complete destruction by giant desmoid tumours in Gardner’s syndrome Report of a case

Finco B., Luongo B., Sarzo G., Savastano S., Vecchiato M., Merigliano S.

Department of Medical and Surgical Sciences Unit of Surgery, Colon-Proctology, University of Padua S. Antonio Hospital, Padua, Italy

Desmoid tumours are locally malignant neoplasias with a high rate of recurrence following removal, especially where the surgery is not radical. They are associated with familial adenomatous polyposis in Gardner syndrome. There are several treatment options, but are all associated with a high failure and recurrence rate. The role of surgery is limited to complicated disease and failure of conservative measures. We present a case of compressive abdominal syndrome in a patient with giant desmoids of the abdominal wall and intramesenteric desmoids with retroperitoneal fibrosis resistant to conservative therapy affected by Gardner syndrome, who had already undergone a subtotal colectomy for familial adenomatous polyposis. The patient underwent surgery with removal of 4 giant desmoids necessitating almost complete destruction of the abdominal wall and subsequent total reconstruction using a gore prosthesis. At 6 month follow-up, the patient’s general health and the aesthetic and functional results of the surgery are satisfactory. in conclusion surgery in this case represents the only therapeutic option, notwithstanding the fact that it is by necessity highly destructive and not without significant peri and postoperative complications. The prognosis is determined by progression of intramesenteric desmoids, which are not suitable for surgery for the high risk of mesenteric vascular lesions.