Case on patient with a stenosis of the cavocavostomy after liver transplantation

A 21-year-old girl suffering from Crigler-Najjar syndrome type I was successfully treated with light therapy until her 18th life year. Due to therapy-resistant progressive hyperbulirubinaemia patient was evaluated for a liver transplantation. There were no signs of liver cirrhosis and the MELD score was 19. Her height was 157 cm and her weight 72 kg. CT scanning of the liver and surrounding vascular structures including the vena cava showed no abnormalities. After one year on the waiting list, transplantation could be performed. During transplantation the side-to-side cavocavostomy was difficult to perform because of the size difference. At one week after transplantation the flow in the hepatic vein was monophasic and one week later the flow was absent and patient developed an acute Budd Chiari syndrome.

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