title: Ureteric obstruction in familial adenomatous polyposis-associated desmoid disease.
abstract: OBJECTIVE
  Intra-abdominal desmoid disease is the second leading cause of death in familial adenomatous polyposis patients. The aim of this study was to identify the incidence, management, and outcomes for familial adenomatous polyposis associated intra-abdominal desmoids causing ureteric obstruction.
  
  
  METHODS
  Clinical data were abstracted from an institutional review board-approved, prospectively maintained familial polyposis registry.
  
  
  RESULTS
  Of 107 patients identified with familial adenomatous polyposis related desmoid disease, 30 (28%) had documented CT scan evidence of ureteric obstruction. There was a 1:2.3 female predominance. Preceding surgery was the most prominent risk factor for development of desmoid disease (28 of 30 patients); 2 patients were diagnosed with desmoids before abdominal surgery. Overall, 11 patients had ureteric obstruction at the time of diagnosis. In the other 19 patients, median time from desmoid diagnosis to ureteric obstruction was 2 years. Pharmacologic management alone was effective in 8 patients. Eighteen patients (60%) underwent retrograde ureteric stent insertion. Five patients (17%) required percutaneous nephrostomy tubes. Three patients (10%) underwent autotransplant of 4 kidneys, and 4 patients (13%) required nephrectomy. One patient underwent ureterolysis, and another underwent ureteric resection with reimplantation. One-third of patients required more than one urologic procedure, and 63% had extensive small-bowel involvement with desmoid.
  
  
  CONCLUSIONS
  The majority of patients with familial adenomatous polyposis associated desmoid disease who develop hydronephrosis require stenting. Complete obstruction may necessitate a nephrostomy. Renal autotransplant is an option for persistent symptomatic obstruction. Physicians treating patients with familial adenomatous polyposis and desmoid disease must be aware of the potential for development of ureteric obstruction and available treatment options.
authors.affiliation: Sanford R. Weiss Center for Inherited Colorectal Neoplasia, Digestive Disease Institute, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
authors.forename: Myles
authors.initials: M
authors.lastname: Joyce
authors.forename: Emilio
authors.initials: E
authors.lastname: Mignanelli
authors.forename: James
authors.initials: J
authors.lastname: Church
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000150:complications* / Q000601:surgery; D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D018222:Desmoid Tumors / Q000150:complications* / Q000601:surgery; D006801:Humans; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D009403:Nephrostomy, Percutaneous; D010166:Palliative Care; D011446:Prospective Studies; D012042:Registries; D012737:Sex Factors; D015607:Stents; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014517:Ureteral Obstruction / Q000209:etiology* / Q000601:surgery