Liver failure Due to Diffuse Neonatal Hemangiomatosis

Abstract

The term Diffuse Neonatal Hemangiomatosis (DNH) has been used to describe multifocal vascular lesions affecting the skin and organs with the liver being the organ most frequently involved. Although spontaneous regression is the rule, complications such as a high cardiac output caused by portovenous or arteriovenous shunts can occur. Liver lesions can be potentially lethal due to severe complications such as fulminant hepatic failure. Mortality varies between 60-80% in the first months of life without treatment. Propranolol has been reported as an effective and well-tolerated treatment for cutaneous hemangiomas and more recently in hepatic hemangiomas. We report the case of an infant with a diffuse neonatal hemangiomatosis with hepatic hemangioma, which caused liver failure and successfully responded to treatment with propranolol. Avens Publishing Group Inviting Innovations Figure 1: MRI delineates multiple discrete homogeneous lesions, 10-30 mm in diameter with very little liver parenchymal between them. Figure 2: An axial T2 magnetic resonance image with fat saturation demonstrates multiple hyperintense lesions with near total hepatic parenchymal replacement. Citation: Cura-Esquivel I, Montes-Tapia F, Rodriguez-Tamez A, Sanchez-Cortes G, González JE. Liver failure Due to Diffuse Neonatal Hemangiomatosis. J Clin Med Case Reports. 2019;6(1): 3. J Clin Med Case Reports 6(1): 3 (2019) Page 02 ISSN: 2332-4120 gamma-glutamyl transpeptidase 178 U/ml, creatinine 0.4 mg/dL, ammonium 80 mg/dL, and clotting times TP 15.8 sec, TTP 43.4 sec and INR 1.3. A thyroid profile was normal. The chest x-ray showed cardiomegaly with a cardiothoracic index of 0.58 and increased pulmonary vasculature. An echocardiogram showed a high cardiac output with heart failure, elevated pulmonary artery pressure and a patent ductus arteriosus. Abdominal ultrasound showed moderate hepatomegaly (7.5 cm) with a heterogeneous echotexture and countless hypoechoic lesions involving the entire liver parenchyma. A contrasted magnetic resonance image of the abdomen demonstrated an enlarged liver. The main hepatic artery, hepatic veins and inferior vena cava were dilated. Multiple hypointense focal lesions on T1 and hyperintense on T2, 10-30 mm in diameter with very little liver parenchymal between them were seen. MRI findings were consistent with diffuse hepatic hemangioma (Figure 1 and 2). Extension studies of the brain and lung showed no lesions. Treatment for heart failure with digoxin and furosemide was started. Also, prednisolone 2 mg/kg/day and propranolol at an initial dose of 1 mg/kg/day and gradually increased to a dose of 3 mg/kg/day were administered for DNH. The patient remained hospitalized in the Pediatric Intensive Care Unit for 3 weeks. His cardiovascular status stabilized after 14 days of treatment. Liver function also gradually normalized. At 3 weeks of treatment jaundice had disappeared and liver function tests were normal (Albumin 3.9 mg/dL, TP 12.2 sec and TTP 18 sec). Treatment with prednisolone was stopped after four weeks and propranolol was continued for 10 months. At 12 months of age, the skin lesions had disappeared. A follow-up ultrasound showed normal liver size with a reduction of hypoechoic lesions (less than 2 mm). At 24 months of follow-up the patient had no evidence of heart failure and normal liver function. The liver hemangiomas were almost imperceptible on abdomen ultrasound and MRI (Figure 3).