Gardner's syndrome: a spectrum of clinical presentations
Steger, A.C.; Galland, R.B.; Burdett-Smith, P.; Spencer, J.
Journal of the Royal College of Surgeons of Edinburgh 31(5): 289-292
1986
ISSN/ISBN: 0035-8835 PMID: 3795148 Document Number: 280924
Gardner's syndrome is a rare condition affecting approximately 10% of patients with polyposis coli. The nonintestinal features of the disease can present varying problems of management. 5 cases have been seen and followed in 12 years (4 women and one man). Presenting features were intestinal polyps in 3 cases and soft tissue tumours in 2. Metachronous lesions presented over many years following the initial manifestation of the disease. 3 patients have had a colectomy with ileorectal anastomosis for polyposis coli; subsequent dysplastic changes in the rectum of one necessitated a proctectomy. 3 patients have had multiple laparotomies to treat fibromatosis, 2 intra-abdominal and one involving the anterior abdominal wall. In all 3 fibromatosis remains a problem. Patients who present with abdominal wall or mesenteric desmoids need thorough investigation to exclude intestinal polyps, for these have a high malignant potential. As our management of polyposis has improved, desmoid tumours have become relatively more important in terms of patient morbidity and mortality.