title: Button cholecystostomy for management of progressive familial intrahepatic cholestasis syndromes.
abstract: OBJECTIVE
  Progressive familial intrahepatic cholestasis syndromes are characterized by impaired bile acid secretion resulting in pruritus, coagulopathy, diarrhea, and malnutrition leading to progressive liver failure and death in childhood. Partial internal or external biliary drainage can relieve symptoms and slow the progression of the disease. Objections to partial external biliary drainage include the need for a permanent biliary stoma with all the inherent complications of a stoma. We propose a novel approach to these diseases--placement of a "button" cholecystostomy tube.
  
  
  METHODS
  Under general anesthesia and through a small right subcostal incision, a MIC-KEY button (Kimberly-Clark Worldwide, Inc, Draper, UT) is inserted into the mobilized fundus of the gallbladder and secured with 2 purse-string sutures. Time of drainage is adjusted to relieve pruritus.
  
  
  RESULTS
  Three children with progressive familial intrahepatic cholestasis achieved adequate bile drainage via the cholecystostomy button to relieve pruritus for 1, 2, and 2 ½ years postoperatively, with drainage periods of 12 to 14 hours per day. There were no episodes of cholangitis. Dislodged tubes can be replaced, or stones can be retrieved via the tract that is formed. Patient (parent) acceptance has been excellent.
  
  
  CONCLUSIONS
  Button cholecystostomy is simple to perform, relieves pruritus with intermittent (nighttime) drainage, avoids complications of a permanent stoma, avoids an enteric anastomosis, and is accepted by parents.
authors.affiliation: Division of Pediatric Surgery, Emory University School of Medicine/Children's Healthcare of Atlanta, Atlanta, GA 30322, USA.
authors.forename: Matthew S
authors.initials: MS
authors.lastname: Clifton
authors.forename: Rene
authors.initials: R
authors.lastname: Romero
authors.forename: Richard R
authors.initials: RR
authors.lastname: Ricketts
mesh_terms: D001294:Attitude to Health; D002648:Child; D002675:Child, Preschool; D002767:Cholecystostomy / Q000295:instrumentation / Q000379:methods*; D002780:Cholestasis, Intrahepatic / Q000601:surgery; D004322:Drainage / Q000295:instrumentation / Q000379:methods; D005260:Female; D005704:Gallbladder / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D010290:Parents / Q000523:psychology; D010342:Patient Acceptance of Health Care / Q000523:psychology; D011537:Pruritus / Q000628:therapy; D054047:Surgical Stomas; D016896:Treatment Outcome