Abstract
Congenital hepatic fibropolycystic diseases are a combination of diseases characterized by a defect in the growth and maturation process of the cells constituting the bile ducts (ductal plate). The clinical expression of these diseases varies greatly, and depends on the leve on which malformation of the ductal plate occurs. Thus, congenital hepatic fibrosis, hepatic polytheistic disease, Caroli disease and choledoch cysts represent different forms of the alteration in the development of the ductal plate, with very different clinical manifestations. Diagnoses and treatment varies based on the presentation form. Evaluation of the presence of retinal cysts and development of bread of failure is important.
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