Hepatic vena cava syndrome: a common cause of liver cirrhosis in children in Nepal.

BACKGROUND: Disease of hepatic portion of the inferior vena cava (IVC) now renamed hepatic vena cava syndrome (HVCS) is an insidious onset chronic disease characterized by recurrent acute exacerbations and development of cavo-caval collaterals. In adults it is complicated by high incidence of liver cirrhosis. Aim of this study was to assess the incidence of liver cirrhosis in children with HVCS and discuss its pathogenesis.

METHOD: One hundred and seventy eight children with HVCS were followed up with ultrasonography (USG), routine hematology and liver tests. During acute exacerbations, cultures of blood and ascitic fluid for aerobic microorganisms were also done. Diagnosis of liver cirrhosis was based on transformation of the liver parenchymal echo-texture from normal to uniformly coarse with rounded edges in follow-up USG It was confirmed by direct inspection of the liver or by biopsy in six. The duration from disease onset to detection of liver cirrhosis was also assessed.

RESULTS: HVCS was seen in children from poor socio-economic background. Forty nine patients (27.5%) developed liver cirrhosis within a few months to a few years of onset of the disease. Development of cirrhosis was related to frequency or severity of acute exacerbations and not to duration of illness or severity of the caval lesion.

CONCLUSION: The cause of cirrhosis in HVCS was the ischemic injury of the hepatocytes and sinusoids following thrombosis of the IVC and/or intra-hepatic veins during severe acute episodes or exacerbations precipitated by bacterial infection. Since such acute exacerbations are amenable to medical treatment, HVCS may be a cause of preventable liver cirrhosis in developing countries.