[Congenital absence of portal vein in a girl with biliary atresia treated with liver transplant].
The congenital absence of the portal vein is a rare malformation, which has been generally discovered in association with another anomalies like a cardiac, gastrointestinal or genitourinary defects. With portal hypertension and hepatic cirrhosis, this anomaly is similar to spontaneous porto-systemic derivation, and for that not collateral venous drainage is present. In these patients, total interruption of mesentaric venous drainage during procurement of liver transplant produce a very important bowel and mesenteric edema, which can promote an injert fatal evolution. The authors present the first paediatric liver transplant, in a patient with portal venous agenesia, with de piggy-back technique was done and this complication was obviated, and a review of the literature about this issue is done.
Taoube
K A
KA
Hopital Sainte-Justine, Montréal, Quebec, Canadá.
Alonso Calderón
J L
JL
Yandza
T
T
St Vil
D
D
Blanchard
H
H
D001656:Biliary Atresia / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D011169:Portal Vein / Q000002:abnormalities*