Symptomatic Infantile Hepatic Hemangioendothelioma Successfully Treated with Steroid
One day old male infant, after an uncomplicated pregnancy and delivery, was referred from the periphery with a presenting feature of poor feeding and respiratory distress. There was no antenatal ultrasonography report available. Examination revealed an irritable child, weighing 2.7 kg, in respiratory distress with relative ratio about 120/min, hazard ratio 180/min, and a high-volume pulse. There was cardiomegaly, a grade 3/6 ejection systolic murmur in the tricuspid area and a left ventricular S3. There were fine crackles over both lungs at the basal region. Liver was soft, 4 cm below the costal margin at the right mid clavicular line. A large firm mass, with irregular surface was palpable over left hypochondrium, epigastric and left lumbar region measuring about 8 × 5 cm, not bimanually palpable.\nThe complete hemogram were normal. There was evidence of prerenal azotemia (blood urea 47 mg/dL and creatinine 0.94 mg/dL). Serum bilirubin, liver enzymes, and serum alpha fetoprotein (AFP) (1.5 mcg/mL) were essentially normal.\nThe chest X-ray showed cardiomegaly and pulmonary venous congestion. The electrocardiography was normal except sinus tachycardia. Echocardiography showed left and right ventricular dilatation, with normal valves and intact septae. There was no patent ductus arteriosus or coarctation. Ejection fraction (EF) was 58% along with mild tricuspid regurgitation and trivial mitral regurgitation.\nUltrasonography of the abdomen showed a well-defined lump measuring 8.5 × 5.2 cm, having marked internal vascularity in the upper abdomen, separated from spleen and kidney. Liver was enlarged with hugely dilated left hepatic vein (LHV). Intrahepatic biliary radicals and common bile duct were also dilated [].\nThen computed tomography (CT) scan of the abdomen showed features suggestive of hemangioendothelioma of liver arising from the left lobe exophytically. Focal calcifications were present; LHV was dilated and there was narrowing of aorta below the level of the celiac artery [].\nCardiac failure was managed with fluid restriction, oxygen, diuretics, and inotropic support for 2 days. But there was no clinical improvement noted. Then after studying different literature, oral prednisolone was started in a dose of 4 mg/kg/day in two divided doses. There was a dramatic improvement in general physical condition of the child. Prednisolone was tapered to 4 mg/kg/day on alternate days after 2 weeks, 2 mg/kg on alternate days by 6 weeks and 1 mg/kg on alternate days at 3 months. At 3 months follow-up, the child was gaining weight steadily, size of the mass had decreased considerably, and echocardiography showed normal EF (65%). Follow-up USG showed a decrease in vascularity and size of the hemangioendothelioma (3.2 × 2.1), but LHV was still dilated.