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DIFFUSE NEONATAL HEMANGIOMATOSIS AND HEART FAILURE

B.Tchana, N. Carano, A. Agnetti, C. Cicero, V. Allegri, A. Saracino, A. Ndongko, C. Cavalli, L.Zavota, U. Squarcia.
Cardiologia Pediatrica - Dip. dell'Età Evolutiva - Univ. di Parma


Diffuse neonatal hemangiomatosis is a rare and life-threatening congenital disorder characterized by the presence of multiple hemangiomas on the skin, the internal organs, the central nervous system and high mortality rate. We report of two patients with diffuse neonatal hemangiomatosis with cutaneous hemangiomas and hepatic arteriovenous fistulas. The first case is an infant girl, with some superficial red little hemangiomas scattered on the trunk, at birth, who unexpectedly presented at the age of two months with dyspnea, tachypnea, feeding difficulties, poor growth and cardiac failure. Laboratory exams showed cardiomegaly, hepatomegaly and pulmonary hypertension. The second case is a premature newborn with a prenatal diagnosis of hepatic arteriovenous fistula that presented at birth few superficial hemangiomas and heart failure with hepatomegaly, cardiomegaly and pulmonary hypertension. Both patients underwent extended ultrasound and computed tomographic scanning to determine the extension of visceral and neural involvement. Patients with hemangiomas need an adequate diagnosis principally to differentiate benign from diffuse neonatal hemangiomatosis in which early recognition is crucial. The most common causes of death are high-output congestive heart failure as a result of arteriovenous shunting in the liver and lungs. Treatment should be supportive and based on the type of complications that are evident, most authors agree that prednisone at doses of at least 2 to 5 mg/ kg/day should be used when visceral involvement is present. Our second patient after three weeks of treatment with prednisone, 3 mg/kg/day, showes a stabilization of the clinical conditions and the cardiac failure with a persisting pulmonary hypertension. The first patient underwent a two months cycle of therapy with 4mg/kg/day of prednisone and showed a progressive reduction of superficial hemangiomas and hepatic hemangiomatosis and resolution of the cardiac failure.

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