[The syndrome of biliary atresia, typical physiognomy, anomalies of the pulmonary arteries and eventual other malformations (author's transl)].

Among the various types of hepatic ductular atresias, there is a group of patients with a definable syndrome of malformations: typical physiognomy, malformation of pulmonary arteries, mental retardation and disturbed growth of body and genitals. This syndrome has been defined only in the last two years by Watson et al. (1973) and Allagille et al. (1975). A detailed description of a boy with this combination of malformations is given. Additionally he has aplasia of the right kidney. A second patient out of 4, which we found in our cardiologic department, has hypoblasia of one kidney, too. The prognosis of the liver disease in these patients seems to be better than in other children with biliary atresia.