Congenital absence of the portal vein associated with congenital hepatic fibrosis.
The radiological features of a 7-year-old boy with congenital absence of the portal vein, pathologically proven congenital hepatic fibrosis, double inferior vena cava, ventricular septal defect, vertebral anomalies, crossed fused renal ectopia, and facial anomalies with pathological correlation are reported. This association between congenital absence of the portal vein and congenital hepatic fibrosis is unique.
Gocmen
Rahsan
R
Radiology Department, Hacettepe University Faculty of Medicine, Sihhiye, Ankara, 06100, Turkey. gocmentr@yahoo.com
Akhan
Okan
O
Talim
Beril
B
D002648:Child; D006801:Humans; D008103:Liver Cirrhosis / Q000151:congenital* / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging; D008297:Male; D011169:Portal Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011859:Radiography; D014463:Ultrasonography