Hepatic Vascular Shunts (HVSs) are rare anomalies that consist of abnormal communications between the hepatic arteries, portal veins, and the hepatic or systemic veins and can be associated with severe morbidity and high mortality rate. We report a newborn patient with hepatic vascular shunts and discuss their etiology and management. We review the presentation, course of disease and outcome in a male newborn who developed hepatomegaly, gastrointestinal bleeding, severe anemia, signs of disseminated intravascular coagulopathy, hypoproteinemia, hypoglycemia and jaundice two hours after birth. Multiple intra- and extrahepatic portosystemic shunts and intrahepatic arteriovenous malformation were diagnosed using Doppler ultrasound and contrast-enhanced CT scan. Echocardiography showed enlarged hepatic veins and subsystemic pressure in the right ventricular. Despite vigorous intensive treatment for progressing liver failure the patient developed cerebral edema, pulmonary hemorrhage renal and heart failure, and died 11 days after birth. Autopsy was not preformed due to the religious beliefs of the parents. Multiple HVSs in the newborn can lead to fulminant hepatic failure and fatal outcome. When practically the entire hepatic circulation is disturbed the only possible treatment option is allogeneic liver transplantation which is further limited due to the lack of size appropriate organ donors in that age group.
 

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