Abstract
Thirteen out of 268 children (<18 years old) underwent hepatic transplantation (OLT) for end-stage liver disease (ESLD) associated with arteriohepatic dysplasia (AHD). Seven children are alive and well with normal liver function. Six children died, four within 11 days of the operation and the other two at 4 and 10 months after the OLT. Vascular complications with associated septicemia were responsible for the deaths of three children. Two died of heart failure and circulatory collapse, secondary to pulmonary hypertension and congenital heart disease. The remaining patient died of overwhelming sepsis not associated with technical complications. Seven patients had a portoenterostomy or portocholecystostomy early in life; five of these died after the OLT. Severe cardiovascular abnormalities in some of our patients suggest that complete hemodynamic monitoring with invasive studies should be performed in all patients with AHD, especially in cases of documented hypertrophy of the right ventricle. The improved quality of life in our surviving patients confirms the validity of OLT as a treatment of choice in cases of ESLD due to AHD.
Similar content being viewed by others
References
Alagille D, Odievre M (1979) Cholestasis in children. In: Alagille D, Odievre M (eds) Liver and biliary tract disease in children. Wiley and Sons, New York, pp 163–195
Alagille D, Odievre M, Gautier M, Dommergues JP (1975) Hepatic ductular hypoplasia associated with characteristic faces, vertebral malformations, retarded physical, mental and sexual development and cardiac murmur. J Pediatr 86: 63–71
Alagille D, Estrada A, Hadchonel M, Gautier M, Odievre M, Dammergues JP (1987) Syndromic paucity of interlobular bile duct (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases. J Pediatr 110:195–200
Dixon WJ (1985) BMDP Statistical software manual. University of California Press, Los Angeles
Esquivel CO, Iwatsuki S, Gordon RD, Marsh WJ Jr, Koneru B, Makowka L, Tzakis AG, Todo S, Starzl TE (1987) Indications for pediatric liver transplantation. J Pediatr 3: 1039–1045
Esquivel CO, Koneru B, Karrer F, Todo S, Iwatsuki S, Gordon RD, Makowka L, Marsh WJ, Starzl TE (1987) Liver transplantation under one year of age. J Pediatr 110: 545–548
Kam I, Lynch S, Todo S, Dewolf A, McSteen F, Jakab F, Ericzon BG, Takaya S, Starzl TE (1986) Low flow venovenous bypasses in small dogs and pediatric patients undergoing replacement of the liver. Surg Gynecol Obstet 163: 33–36
Markowitz J, Daum F, Kahn EI, Schneider KM, So HB, Altman RP, Aiges HW, Alperstein G, Silverberg M (1983) Arteriohepatic dysplasia. I. Pitfalls in diagnosis and management. Hepatology 3: 74–76
Perrault J (1981) Paucity of interlobular ducts. Dig Dis Sci 26: 481–484
Starzl TE, Demetris AJ (1990) Liver transplantation: a 31-year perspective. Part I. Curr Probl Surg 27: 96–98
Starzl TE, Iwatsuki S, Thiel DH van, Gartner JC, Zitelli BJ, Malatack JJ, Schade RR, Shaw BW Jr, Hakala TR, Rosenthal JT, Porter KA (1982) Evolution of liver transplanration. Hepatology 2:614–636
Starzl TE, Iwatsuki S, Esquivel CO, Todo S, Kam I, Lynch S, Gordon RD, Shaw BW Jr (1985) Refinements in the surgical technique of liver transplantation. Semin Liver Dis 5: 349–356
Tzakis A, Todo S, Starzl TE (1989) Piggyback orthotopic liver transplantation with preservation of the inferior vena cava. Ann Surg 210: 649–652
Watson GH, Miller V (1973) Arteriohepatic dysplasia, familial pulmonary stenosis with neonatal liver disease. Arch Dis Child 48: 459–466
Author information
Authors and Affiliations
About this article
Cite this article
Marino, I.R., ChapChap, P., Esquivel, C.O. et al. Liver transplantation for arteriohepatic dysplasia (Alagille's syndrome). Transplant Int 5, 61–64 (1992). https://doi.org/10.1007/BF00339217
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00339217