Successful propranolol treatment for diffuse neonatal hemangiomatosis

Dear Editor, Diffuse neonatal hemangiomatosis (DNH) is a rare disorder characterized by multiple cutaneous infantile hemangiomas (CIH) and visceral hemangiomas such as infantile hepatic hemangiomas (IHH). Although patients with DNH may be asymptomatic, they could be in a critical situation when complicated by congestive heart failure due to vascular shunting, bleedings and disseminated intravascular coagulation. Recently, propranolol has been proposed as a novel treatment modality for infantile hemangiomas and has also been used for DNH. Herein, we report a case in which DNH was successfully treated with propranolol. The patient was delivered at 36 weeks and 6 days of gestation. Her birthweight was 2474 g. She presented with hemangiomas on her abdomen, back and right index finger at birth. At 4 days of age, she had a total of seven cutaneous hemangiomas with new lesions appearing on her forehead, calvaria, auricle and left middle finger (Fig. 1a). At 6 days of age, complete blood count and liver function tests were within the normal values. Magnetic resonance imaging revealed multiple IHH (Fig. 1b). Histopathology of the cutaneous hemangioma showed numerous small vessels in the dermis and subcutaneous tissue (Fig. 1c). Immunohistochemically, these vessels were positive for CD31, CD34 and glucose transporter1. The MIB-1 index of the CD-31 positive endothelial cells was 13.0% (Fig. 1c). She was treated with propranolol at the initial dose of 0.5 mg/kg per day. The dose was then increased to 3.0 mg/kg per day, with no notable adverse events. One month later, CIH reduced and IHH partially regressed. At 1 year of age, CIH markedly regressed and IHH showed complete resolution (Fig. 1b,d). The patient was maintained on oral treatment with propranolol 3.0 mg/kg per day until 1 year age, after which we are planning to taper and discontinue the propranolol treatment. In the present case, hemangiomas were limited to skin and liver. Although the diagnostic criteria for DNH was the involvement of three or more organ systems. The clinical features and outcome of patients with only cutaneous and hepatic hemangiomas were similar to those with three or more organ systems. Therefore, the presence of hemangiomas of the skin and liver is sufficient to be regarded as DNH. Recently, propranolol has been tried for the treatment of DNH. In the past cases, propranolol was started at the rate of