An experience with dystocia due to fetal ascites is described. It was necessary to perform intrauterine fetal abdominal paracentesis to complete the delivery. The ascites was associated with a diffuse hepatic developmental anomaly interpreted as a form of polycystic disease without cysts. Associated anomalies included cystic lung and a very rare variety of pulmonary arteriovenous fistula. The structural abnormality in the liver, though in an exceptionally undifferentiated stage, belongs to the category of so-called congenital hepatic fibrosis, which, in turn, is a noncystic variant of congenital polycystic disease. Theoretical aspects of the pathogenesis of the ascites are discussed. A new term, portal dysplasia of the liver (polycystic disease without cysts), descriptively adequate to embrace all of the structural variations of this developmental hepatic abnormality, is suggested.
Unexplained cyanosis revealing hepatopulmonary syndrome in a child with asymptomatic congenital hepatic fibrosis: a case report
