Partial internal biliary diversion for patients with progressive familial intrahepatic cholestasis type 1.
ABSTRACT We herein report a case of progressive familial intrahepatic cholestasis with partial internal biliary diversion (PIBD). Although by using PIBD an external stoma can be avoided, exposure of the ileocecal junction to bile reflux as well as the effects of the direct bile flow on the colonic mucosa require further investigation.
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ABSTRACT: Progressive familial intrahepatic cholestasis (PFIC) presents in early childhood with pruritus, jaundice, hepatomegaly, and growth failure. Medical therapy is unsuccessful, with progression from cholestasis to hepatic fibrosis, cirrhosis, and ultimately death before the age of 10 years. Because of evidence that biliary diversion can arrest or reverse progression to hepatic fibrosis, we have used partial biliary diversion (PBD) as primary therapy in PFIC, reserving orthotopic liver transplantation (OLT) for children who have progressive disease or established cirrhosis. Seventeen children with PFIC (aged 2 months to 19 years) have been treated. PBD was performed in eight cases. In these procedures, a 10-cm properistaltic jejunal segment was anastomosed to the side of the gallbladder, terminating as an end stoma for the collection and discard of bile. Eleven patients with hepatic insufficiency (or end-stage cirrhosis) received OLT using standard techniques, at the average age of 4 years. Six of the eight children treated with PBD had complete resolution of clinical symptoms and remain well 1 to 13 years postoperatively. These six patients have conjugated bilirubin values of less than 0.3 mg/dL, normal transaminases, and a serum bile salt concentration of less than 10 nmol/mL. All have had either reversal or no progression of the hepatic fibrosis. Postoperative bleeding complications occurred in two (25%), which required reoperation. One patient had an adhesive intestinal obstruction that was managed surgically 9 months postoperatively. Two patients had no benefit from PBD, and all of them had severe bridging fibrosis (1) or cirrhosis (3). These and nine others with cirrhosis at the time of presentation received orthotopic liver transplantation; of these, eight are alive (1 to 5 years postoperatively). These results show the importance of establishing a correct diagnosis in children with cholestasis. Clinical symptoms often are severe in children with PFIC before the development of irreversible hepatic fibrosis. Because several patients who appear to have been cured with PBD initially were scheduled for OLT, it is important that transplant surgeons recognize the feasibility of this approach.Journal of Pediatric Surgery 01/1996; 30(12):1635-41. · 1.38 Impact Factor
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ABSTRACT: Partial diversion of bile flow to an external stoma was performed in 6 patients with chronic intrahepatic cholestasis with severe pruritus that had been refractory to medical measures. Four patients with progressive intrahepatic cholestasis and 2 with arteriohepatic dysplasia were treated. Follow-up has been 3-8 yr. Patients with progressive intrahepatic cholestasis have been free of itching since surgery. Serum bile salt concentrations fell from 218-275 microM (normal less than 10) before to less than 10 microM after surgery. Biochemical tests of liver function and histology returned to normal or near normal. Patients with arteriohepatic dysplasia had persistent mild pruritus after surgery. Serum bile salt concentrations fell from 153-317 to 25-37 microM. There was little or no improvement in biochemical tests or histology. Bile volume and bile salt diverted were higher in patients with progressive intrahepatic cholestasis (7.3-13.0 ml/kg.day and 83-137 mumol/kg.day, respectively) than those with arteriohepatic dysplasia (3.2-4.5 ml/kg.day and 21-36 mumol/kg.day). The quality of life since surgery has been excellent in patients with progressive intrahepatic cholestasis, but not as optimal in those with arteriohepatic dysplasia. These findings suggest that partial external biliary diversion can provide effective relief from pruritus and perhaps reversal of liver disease in patients with progressive intrahepatic cholestasis. It should be used in patients with arteriohepatic dysplasia only in those with disabling pruritus.Gastroenterology 08/1988; 95(1):130-6. · 12.82 Impact Factor
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ABSTRACT: Progressive familial intrahepatic cholestasis (Byler's disease) is often characterized by pruritus-induced self-mutilation with minimal response to medical therapy. The causative cholestasis is likely to progress to cirrhosis necessitating transplantation. Partial external biliary diversion has been used with promising results for the jaundice and debilitating pruritus but all the potential complications and aesthetic concerns of long-term stomas attend this approach. The authors describe a terminal ileal exclusion that was first developed for patients who had previously undergone cholecystectomy. Over a 3-year period, we identified for study seven children with liver histology characteristic of Byler's disease accompanying a clinical picture of chronic cholestasis without a defined metabolic or anatomic abnormality. The first two patients underwent a cholecystojejunal cutaneous stoma, until now, the recommended treatment for this condition. The third had previously undergone cholecystectomy so an ileocolonic anastomosis was performed excluding the distal 15% of the small bowel. This child had complete relief of pruritus without evidence of diarrhea. 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 length to small intestinal length. The midpoint between the mean and one standard deviation below the mean was determined. Fifteen percent of the estimated small bowel length was measured back from the ileocecal valve and then divided using a linear stapling device. A stapled anastomosis was created between the proximal ileum and the cecum, bypassing the terminal ileum. Four of five children have had relief from their pruritus and self-mutilation with no evidence of diarrhea. Terminal ileal bypass offers a stoma-free, completely reversible "biliary diversion." Early results on a few patients are promising, 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.Journal of Pediatric Surgery 03/1998; 33(2):220-4. · 1.38 Impact Factor