ILEAL EXCLUSION FOR BYLERS-DISEASE - AN ALTERNATIVE SURGICAL APPROACHWITH PROMISING EARLY RESULTS FOR PRURITUS

Background/Purpose: Progressive familial intrahepatic cholestasis (Byl er's disease) is often characterized by pruritus-induced self-mutilati on with minimal response to medical therapy. The causative cholestasis is likely to progress to cirrhosis necessitating transplantation. Par tial external biliary diversion has been used with promising results f or the jaundice and debilitating pruritus but all the potential compli cations and aesthetic concerns of long-term stomas attend this approac h. Methods: The authors describe a terminal ileal exclusion that was f irst developed for patients who had previously undergone cholecystecto my. Over a 3-year period, we identified for study seven children with liver histology characteristic of Byler's disease accompanying a clini cal picture of chronic cholestasis without a defined metabolic or anat omic abnormality. The first two patients underwent a cholecystojejunal cutaneous stoma, until now, the recommended treatment for this condit ion. The third had previously undergone cholecystectomy so an ileocolo nic anastomosis was performed excluding the distal 15% of the small bo wel. This child had complete relief of pruritus without evidence of di arrhea. Two more terminal ileal exclusions were performed with similar results before standardizing this approach. The authors approximated small intestinal length using Siebert's graph relating crown-heel leng th to small intestinal length. The midpoint between the mean and one s tandard deviation below the mean was determined, Fifteen percent of th e estimated small bowel length was measured back from the ileocecal va lve and then divided using a linear stapling device. A stapled anastom osis was created between the proximal ileum and the cecum, bypassing t he terminal ileum. Results: Four of five children have had relief from their pruritus and self-mutilation with no evidence of diarrhea. Term inal ileal bypass offers a stoma-free, completely reversible ''biliary diversion.'' Conclusion: Early results on a few patients are promisin g, but long-term evaluation of growth, development, and liver function and histology is needed before advocating this as the primary therapy for Byler's disease. Copyright (C) 1998 by W.B. Saunders Company.