Diffuse neonatal hemangiomatosis: a new constellation of findings.
ABSTRACT Diffuse neonatal hemangiomatosis is a rare, frequently fatal disease characterized by multiple cutaneous and visceral hemangiomas. The skin, liver, lungs, intestines, and central nervous system are the most commonly affected organs. Death can result from high-output cardiac failure as a result of arteriovenous shunting. In this report we present a new constellation of findings in a patient with diffuse neonatal hemangiomatosis, namely, an absent corpus callosum, ectopia cordis (sternal agenesis) with median abdominal raphe, and tricuspid atresia.
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ABSTRACT: Diffuse Neonatal Hemangiomatosis (DNH) is a rare, life-threatening condition associated with a few to hundreds of small, cutaneous and visceral hemangiomas. The authors reported 5 cases of DNH in which hepatic hemangioma were the most common visceral involvement. Response to prednisolone in these cases was not good, one died and four required second line therapy. Of these four cases, one case with embolisation; one with interferon and two with vinblastine. Response to vinblastine was good, but long-term follow-up of the side effects are needed.Journal of the Medical Association of Thailand = Chotmaihet thangphaet 09/2006; 89(8):1297-303.