Abstract
Arteriohepatic dysplasia is a congenital syndrome associated with life-long cholestasis. Because of symptoms suggesting extrahepatic biliary tract obstruction, we studied three patients with this syndrome by endoscopic retrograde cholangiopancreatogram (ERCP). All patients showed a decrease in the number of intrahepatic ducts. In addition, the intrahepatic ducts show attenuation with focal areas of dilatation. In one subject, this latter finding appeared to be a localized process. The extrahepatic ducts were also narrowed. One patient in this series was found to have gallstones and another cirrhosis. Although the relationship of these anomalies to the cholestasis seen in these patients is unclear, arteriohepatic dysplasis can be added to the list of processes associated with biliary tract abnormalities.
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Riely CA, LaBrecque DR, Ghent C, Horwich A, Klatskin G: A father and son with cholestasis and peripheral pulmonic stenosis: A distinct form of intrahepatic cholestasis. J Pediatr 92:406–411, 1978
Greenwood RD, Rosenthal A, Crocker AC, Nadas AS: Syndrome of intrahepatic biliary dysgenesis and cardiovascular malformations. Pediatrics 58:243–247, 1976
Alagille D, Odiévre M, Gautier M, Dommergues JP: Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur. J Pediatr 86:63–71, 1975
Riely CA, Cotlier E, Jensen PS, Klatskin G: Arteriohepatic dysplasia: A benign syndrome of intrahepatic cholestasis with multiple organ involvement. Ann Intern Med 91:520–527, 1979
Aagennaes D, Henricksen T, Sørland S: Hereditary neonatal cholestasis combined with vascular malformations.In Liver Disease in Infancy and Childhood. SR Berenberg (ed). Baltimore, Williams and Wilkins, 1976, pp 199–206
Riely CA: Familial intrahepatic cholestasis: An update. Yale J Biol Med 52:89–98, 1979
Rosenfield NS, Kelley MJ, Jensen PS, Cotlier E, Rosenfield AT, Riely CA: Arteriohepatic dysplasia: Radiologic features of a new syndrome. Am J Roentgenol 135:1217–1223, 1980
Thaler MM, Gellis SS: Studies in neonatal hepatitis and biliary atresia IV. Diagnosis. Am J Dis Child 116:280–284, 1968
Kum LS, Wong KP, Chan PKW, Ngan H, Ong GB: Recurrent pyogenic cholangitis: A study by endoscopic retrograde cholangiography. Gastroenterology 74:1196–1203, 1978
Chapman RWG, Arborgh BAM, Rhodes JM, Summerfield JA, Dick R, Sheuer PJ, Sherlock S: Primary sclerosing cholangitis: A review of its clinical features, cholangiography and hepatic histology. Gut 21:870–877, 1980
Altemeier WA, Gall EA, Culbertson WR, Inge WW: Sclerosing carcinoma of the intrahepatic bile ducts. Surgery 60:191–200, 1966
Tanaka H, Sakumoto K, Kosihara T: Diseases of the biliary system.In Endoscopic Retrograde Cholangiopancreatography. T Takemoto, T Kasugai (eds). New York, Ingaku-Shoin, 1979, pp 306–321
Summerfield JA, Elias E, Hungerford GD, Nikapota VLB, Dick R, Sherlock S: The biliary system in primary biliary cirrhosis: A study by endoscopic retrograde cholangiopancreatography. Gastroenterology 70:240–243, 1976
Kocoshis SA, Cottrill CM, O'Connor WN, Haugh R, Johnson GL, Noonan JA: Congenital heart disease, butterfly vertebrae, and extrahepatic biliary atresia: A variant of arteriohepatic dysplasia. J Pediatr 99:436–439, 1981
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Gorelick, F.S., Dobbins, J.W., Burrell, M. et al. Biliary tract abnormalities in patients with arteriohepatic dysplasia. Digest Dis Sci 27, 815–820 (1982). https://doi.org/10.1007/BF01391375
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DOI: https://doi.org/10.1007/BF01391375