Button cholecystostomy for management of progressive familial intrahepatic cholestasis syndromes

Background/Purpose: 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 1/2 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. Conclusion: 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. (C) 2011 Elsevier Inc. All rights reserved.