Clinical and Prothrombotic Profile of Hepatic Vein Outflow Tract Obstruction

Abstract

To study the clinical as well as prothrombotic profile and outcome of hepatic venous outflow tract obstruction in children. This is a prospective study of a cohort of hepatic venous outflow tract obstruction (HVOTO) pediatric cases. All children and adolescents presenting with acute or chronic liver disease were screened for HVOTO with ultrasound and Doppler imaging and confirmed by multidetector computerised tomography (MDCT) with contrast enhancement. Of the 162 cases of chronic liver disease, 13 (7.4%) were diagnosed to have HVOTO. Ascites and edema over the feet were the most prominent features. Anabolic steroids and herbal drugs were being taken by one case each. Six cases were diagnosed on Doppler and for rest 7 cases conclusive diagnosis was made on multidetector computerised tomography. Five out of 13 cases were heterogenous (CT) for mutation of the gene encoding methylene tetrahydrofolate reductase (MTHFR) and one case of these was also heterogenous for Factor Leiden V. One case was known celiac and developed HVOTO and was also found to be having hepatocellular carcinoma. Other causes were drug induced, pressure on inferior vena cava (IVC) and inferior vena cava (IVC) web. Thus the authors could find a prothrombotic cause for 10 out of 13 (76.9%) cases. Three cases did not need any intervention. In one patient with infective thrombus of the IVC intervention was not planned. Six underwent angioplasty and 3 underwent transjugular intrahepatic portosystemic shunt. All were asymptomatic with improving growth parameters at follow up. Ascites, pedal edema, prominent abdominal veins and hepatomegaly should raise the suspicion of HVOTO in childhood liver disease. Majority of the cases would be harbouring a prothrombotic cause. MTHFR mutation was the commonest cause of HVOTO in the present study. Angioplasty and/or transjugular intrahepatic portosystemic shunt (TIPSS) can successfully treat HVOTO.