Abstract
Progression to cirrhosis despite restoration of bile flow by successful portoenterostomy suggests that factors other than obstruction alone are operative in biliary atresia. This paper describes pathologic changes at all levels of the hepatobiliary secretory apparatus from hepatocyte to common duct, exhibited in liver biopsies, resected portions of liver adjacent to the porta hepatis, and extrahepatic duct remnants from 17 patients operated upon for relief of biliary atresia. In both intra- and extrahepatic bile ducts, there were epithelial changes interpreted as occurring in a sequence of vacuolar degeneration, necrosis, inflammatory infiltration, and proliferative epithelial repair that precede obliterative fibrosis. From their distribution, it is inferred that these cytopathologic changes may not be simply due to distal obstruction. These ductal epithelial changes are similar to those that occur in livers of some patients with severe viral hepatitis. The association of these changes with giant cell transformation in all cases suggests that they reflect a common etiology for ductal and hepatocellular damage in the neonatal hepatitis-biliary atresia spectrum. The persistence of these changes in liver biopsies following portoenterostomy suggests that they may be of pathogenetic significance in the progressive liver disease seen in most patients with biliary atresia.
Résumé
L'aggravation de la cirrhose après une hépaticoentérostomie rétablissant l'écoulement de bile suggère que la pathogénie de l'atrésie biliaire n'est pas uniquement faite d'obstruction biliaire. Nous décrivons les lésions observées, chez 17 malades opérés d'atrésie biliaire, aux divers étages de l'arbre biliaire, depuis l'hépatocyte jusqu'au cholédoque (biopsies hépatiques, fragments de tissu hépatique prélevés dans le hile, résidus de l'arbre biliaire extra-hépatique). Tant dans les canaux biliaires intra- qu'extra-hépatiques, on trouve des altérations épithéliales que l'on peut interpréter comme une succession de dégénérescence vacuolaire, nécrose, infiltrat inflammatoire, prolifération épithéliale réparatrice et finalement fibrose oblitérante. La distribution de ces lésions cytologiques indique qu'elles ne sont peut-être pas dues uniquement à une obstruction distale. Les altérations de l'épithélium des canalicules biliaires ressemblent à ce que l'on observe dans la foie de certains cas d'hépatite virale. Dans tous les cas, les lésions s'accompagnent de formation de cellules géantes, ce qui suggère une étiologie commune aux lésions canalaires et hépatocellulaires de l'hépatite néonatale et de l'atrésie biliaire. La persistance des lésions dans les biopsies prélevées après hépaticoentérostomie indique qu'elles peuvent jouer un rôle pathogène dans la progression de l'atteinte hépatique observée dans la majorité des cas d'atrésie biliaire.
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Haas, J.E. Bile duct and liver pathology in biliary atresia. World J. Surg. 2, 561–569 (1978). https://doi.org/10.1007/BF01556047
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DOI: https://doi.org/10.1007/BF01556047