- Email: [email protected]
Ductal plate malformation, congenital hepatic fibrosis and Calori's disease are the same entities or not?
Hepatology Research 35 (2006) 77–78
Editorial
Ductal plate malformation, congenital hepatic fibrosis and Calori’s disease are the same entities or not?
Intrahepatic bile ducts develop from primitive hepatocytes precursor cells which ensheathe the mesenchyme of the portal vein. At 9–10 weeks of gestation, these cells become more condensed forming a sleeve around the portal tract. These sleeves are called as the ductal plate. During the development of the liver, structures of peripheral tubules develop in the ductal plates to form mature tubular bile ducts [1]. If these processes are failed or arrested, embryonic bile ducts exceed in a ductal plate configuration. These abnormalities are termed ductal plate malformation (DPM). Congenital hepatic fibrosis (CHF), an autosomal recessive inherited disease and represents DPM of interlobular bile ducts, along with a superimposed destructive type of cholangiopathy associated with scarring fibrosis. An extensive network of thick, collagenous bands, usually continuously, links to adjacent portal tracts. Numerous structures resembling bile ducts, some slightly irregular and dilated, are situated in the fibrous septa and sometimes contain mucin or bile. Although they resemble bile ducts, they actually represent ductal plate remnants. Portal vein branches often appear reduced in size and number, and the sparsity of venous channels may account in part for portal hypertension. The irregular intervening parenchyma frequently exhibits a “jigsaw” pattern reminiscent of that seen in biliary cirrhosis. Caroli’s disease is called as infantile polycystic disease or adult polycystic disease. In infantile type, expression varies from individual to individual. The liver in childhood polycystic disease is enlarged and firm, but cysts are not usually visible grossly. Microscopic sections show numerous irregular, duct-like structures in the portal areas, with apparent branching and irregular, angulated extensions into the acini and a circular arrangement of the ducts, either complete or interrupted, is characteristic of the ductal plate malformation. In adult types, numerous cysts, varying from less
1386-6346/$ – see front matter © 2006 Published by Elsevier Ireland Ltd. doi:10.1016/j.hepres.2006.04.003
than 1 mm to more than 12 cm in size, contain colorless or straw color fluid, unless infected. Microscopically, the cysts appear to originate in the portal areas. They are lined by low columnar to cuboidal epithelium and have a collagenous supporting stroma that can be infiltrated by a few inflammatory cells. Von Meyenburg complexes (biliary microhamartomas) are frequently present and components of complexes lie adjacent to the cysts, which suggests that the cysts evolve from progressive dilation of the biliary channels in the complexes. DPM is now to be the basic component of these diseases and DPM at different levels of the biliary tree induced the wide spectrum of intrahepatic bile duct diseases [2,3]. Gionleme et al. highlighted and emphasized these entities in this issue of Hepatology Research [4]. It has been proposed that virtually all congenital diseases of IHBDs represent DPM and DPM may affect various levels of the intrahepatic biliary tree [5-7]. The DPMs at the different levels of biliary tree determine the different anatomical–clinical entities.
References [1] Terada T, Nakanuma Y. Expression of tenascin, type IV collagen and laminin during human intrahepatic bile duct development and in intrahepatic cholongiocarcinoma. Histopathology 1994;25:143–50. [2] Awasthi A, Das A, Srinivasan R, Joshi K. Morphological and immunohistochemocal analysis of ductal plate malformation: correlation with fetal liver. Histopathology 2004;45:260–7. [3] Desmet VJ. Congenital diseases of intrahepatic bile ducts: variations on the theme ductal plate malformation. Hepatology 1992;16:1069–83. [4] Gionleme O, Nikolaidis N, Tziomalos K, et al. Ductal plate malformation and congenital hepatic fibrosis. Clinical and histological findings in four patients. Hepatol Res 2006;35:147–50.
78
Editorial / Hepatology Research 35 (2006) 77–78
[5] Jorgensen MJ. The ductal plate malformation. Acta Pathol microbial Scand 1977;257(Suppl):1–87. [6] Poddar U, Thapa BR, Vashishta RK, Girish CS, Singh K. Congenital hepatic fibrosis in Indian children. J Gastroenterol Hepatol 1999;14:1192–6. [7] Summerfield JA, Nagafuchi Y, Sherlock S, Cadafalch J, Scheuer PJ. Hepatobiliary fibropolycystic diseases. A clinical and histological review of 51 patients. J Hepatol 1986;2:141–56.
Tetsuya Mine Department of Internal Medicine, Division of Gastroenterology and Hepatology, University of Tokai School of Medicine, Japan E-mail address: [email protected] 12 April 2006
Editorial
Ductal plate malformation, congenital hepatic fibrosis and Calori’s disease are the same entities or not?
Intrahepatic bile ducts develop from primitive hepatocytes precursor cells which ensheathe the mesenchyme of the portal vein. At 9–10 weeks of gestation, these cells become more condensed forming a sleeve around the portal tract. These sleeves are called as the ductal plate. During the development of the liver, structures of peripheral tubules develop in the ductal plates to form mature tubular bile ducts [1]. If these processes are failed or arrested, embryonic bile ducts exceed in a ductal plate configuration. These abnormalities are termed ductal plate malformation (DPM). Congenital hepatic fibrosis (CHF), an autosomal recessive inherited disease and represents DPM of interlobular bile ducts, along with a superimposed destructive type of cholangiopathy associated with scarring fibrosis. An extensive network of thick, collagenous bands, usually continuously, links to adjacent portal tracts. Numerous structures resembling bile ducts, some slightly irregular and dilated, are situated in the fibrous septa and sometimes contain mucin or bile. Although they resemble bile ducts, they actually represent ductal plate remnants. Portal vein branches often appear reduced in size and number, and the sparsity of venous channels may account in part for portal hypertension. The irregular intervening parenchyma frequently exhibits a “jigsaw” pattern reminiscent of that seen in biliary cirrhosis. Caroli’s disease is called as infantile polycystic disease or adult polycystic disease. In infantile type, expression varies from individual to individual. The liver in childhood polycystic disease is enlarged and firm, but cysts are not usually visible grossly. Microscopic sections show numerous irregular, duct-like structures in the portal areas, with apparent branching and irregular, angulated extensions into the acini and a circular arrangement of the ducts, either complete or interrupted, is characteristic of the ductal plate malformation. In adult types, numerous cysts, varying from less
1386-6346/$ – see front matter © 2006 Published by Elsevier Ireland Ltd. doi:10.1016/j.hepres.2006.04.003
than 1 mm to more than 12 cm in size, contain colorless or straw color fluid, unless infected. Microscopically, the cysts appear to originate in the portal areas. They are lined by low columnar to cuboidal epithelium and have a collagenous supporting stroma that can be infiltrated by a few inflammatory cells. Von Meyenburg complexes (biliary microhamartomas) are frequently present and components of complexes lie adjacent to the cysts, which suggests that the cysts evolve from progressive dilation of the biliary channels in the complexes. DPM is now to be the basic component of these diseases and DPM at different levels of the biliary tree induced the wide spectrum of intrahepatic bile duct diseases [2,3]. Gionleme et al. highlighted and emphasized these entities in this issue of Hepatology Research [4]. It has been proposed that virtually all congenital diseases of IHBDs represent DPM and DPM may affect various levels of the intrahepatic biliary tree [5-7]. The DPMs at the different levels of biliary tree determine the different anatomical–clinical entities.
References [1] Terada T, Nakanuma Y. Expression of tenascin, type IV collagen and laminin during human intrahepatic bile duct development and in intrahepatic cholongiocarcinoma. Histopathology 1994;25:143–50. [2] Awasthi A, Das A, Srinivasan R, Joshi K. Morphological and immunohistochemocal analysis of ductal plate malformation: correlation with fetal liver. Histopathology 2004;45:260–7. [3] Desmet VJ. Congenital diseases of intrahepatic bile ducts: variations on the theme ductal plate malformation. Hepatology 1992;16:1069–83. [4] Gionleme O, Nikolaidis N, Tziomalos K, et al. Ductal plate malformation and congenital hepatic fibrosis. Clinical and histological findings in four patients. Hepatol Res 2006;35:147–50.
78
Editorial / Hepatology Research 35 (2006) 77–78
[5] Jorgensen MJ. The ductal plate malformation. Acta Pathol microbial Scand 1977;257(Suppl):1–87. [6] Poddar U, Thapa BR, Vashishta RK, Girish CS, Singh K. Congenital hepatic fibrosis in Indian children. J Gastroenterol Hepatol 1999;14:1192–6. [7] Summerfield JA, Nagafuchi Y, Sherlock S, Cadafalch J, Scheuer PJ. Hepatobiliary fibropolycystic diseases. A clinical and histological review of 51 patients. J Hepatol 1986;2:141–56.
Tetsuya Mine Department of Internal Medicine, Division of Gastroenterology and Hepatology, University of Tokai School of Medicine, Japan E-mail address: [email protected] 12 April 2006