Enfermedades fibropoliquísticas hepáticas

Abstract

IntroductionFibropolycistic diseases of the liver comprise a heterogeneous group of different disorders in which fibrosis is associated with segmental dilatations of the bile ducts. Although classified into specific conditions, there is much overlap between them and close association with fibrocystic anomalies in the kidneys is usually seen. ClassificationIn polycystic liver disease associated with renal cysts the impairment in renal function may overshadow the liver disease. In congenital hepatic fibrosis the principal symptoms arise from portal hypertension, as the liver synthetic function is preserved. Simple hepatic cysts and von Meyendeburg complex are the minimal expression of all these fibrocystic diseases. Carolís syndrome and Carolís disease both have large intrahepatic bile duct dilatations; however liver fibrosis and portal hypertension signs occur only in Carolís syndrome. Dilatation of the extrahepatic bile ducts, name commonly as choledocal cysts cause cholangitis. PathogenyThe origin of all these conditions is the malformation of the ductal plate during the embryonic period. On the one hand, the abnormal proliferation of the bile epithelium leads to an excess synthesis of extracellular matrix (fibrosis). On the other hand large cysts arise in the liver and also in the bile tree.