NEONATAL HEMANGIOMATOSIS AND HEART FAILURE
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.
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