A rare presentation of chronic Budd Chiari syndrome in a 13 years old female

Abstract

These days pathologies related to liver are increasing due to one or other factors. Here, we review a case of a girl with Budd Chiari syndrome (BCS), which is due to obstruction of hepatic veins, in turn causing portal hypertension. The pathology of the syndrome includes hepatic venous outflow tract obstruction due to thrombosis along the vein. This in turn leads to portal hypertension and increased pressure in the inferior vena cava (IVC). Clinically, it affects the heart, liver, esophagus, rectum, superficial skin. Early diagnosis can be done by Doppler and liver biopsy to prevent chronic complications of hepatic fibrosis and cirrhosis. The patient was a 13-year-old girl who had abdominal distension, pedal edema, discoloured and decreased urine and fever. On examining the patient, we found out stunted linear and intellectual growth as per her age, low IQ and communication deficits. On ultrasonography, a nodular liver surface with rounded edges and hypoechoic nodules within the parenchyma were seen. Biochemical investigations showed increase in liver enzymes. Also, there was free fluid in abdomen, few anechoic channels in periumbilical and perisplenic regions showing color flow on Doppler. Ascitic tap showed a high total cell count with a lymphocytic predominance. Based on history by the patient, radiological findings, Ascitic tap and biochemical investigations we have come to the conclusion that this is a case of BCS. After being admitted, we gave antibiotics, diuretic, folic acid, albumin, lactulose. Patient was discharged on propanolol as prophylaxis for portal hypertension.