Lissencephaly, cerebellar hypoplasia, and extrahepatic biliary atresia: an unusual association

Congenital Extrahepatic Biliary Atresia

Case 1. The elder of two sibs, this boy was born on May 21, 1965 to unrelated healthy parents aged 29 (mother) and 30 years (father). Birthweight was 2820 g.; delivery was by forceps. His mother had 'influenza' between the fifth and sixth months of the pregnancy which had been otherwise normal. There was no previous foetal loss. Four generations of the family, comprising, in addition to the par...

Extrahepatic Biliary Atresia

Prognosis of extrahepatic biliary atresia.

We carried out a retrospective investigation of the 89 patients with extrahepatic biliary atresia born in The Netherlands during a 10 year period. Of these 89 patients 10 had a diagnostic laparotomy only. Eight patients had an anastomosis between the proximal bile duct and the intestine, and the remaining 71 had hepatic portoenterostomies. Bile drainage was re-established in 46 (65%). After suc...

[Extrahepatic biliary atresia: diagnostic methods].

OBJECTIVE To emphasize the importance of precocious diagnosis of extrahepatic biliary atresia and its direct relationship with the surgical re-establishment of the biliary flow before the second month of life. To discuss several complementary methods with the aim of selecting the ones that present better evidence, and avoiding delays in diagnosis and worse prognostic. SOURCES OF DATA Bibliogr...

Hereditary lissencephaly and cerebellar hypoplasia in Churra lambs

BACKGROUND Lissencephaly is a rare developmental brain disorder in veterinary and human medicine associated with defects in neuronal migration leading to a characteristic marked reduction or absence of the convolutional pattern of the cerebral hemispheres. In many human cases the disease has a genetic basis. In sheep, brain malformations, mainly cerebellar hypoplasia and forms of hydrocephalus,...