Diffuse neonatal hemangiomatosis : a case report

1990 
Diffuse neonatal hemangiomatosis (DNH) is a rare condition characterized by the presence of numerous cutaneous and visceral hemangiomas that become manifest at birth or within the neonatal period . The cutaneous lesions are generalized, vary from 0.5 to 1.5 em in diameter, and often range from 50 to 500 in number. Visceral lesions are most commonly found in the liver, CNS, intestine, and lungs . Skeletal involvement also has been reported. Approximately 60% of infants with DNH die during the first few months of life due to high-output cardiac failure, hemorrhage, or CNS involvement [1 ] . Steroid therapy is thought to accelerate involution of these lesions and has raised prospects for long-term survival [2] . This report describes the usefulness of MR imaging in demonstrating the numerous intracranial hemorrhages seen in DNH . MR imaging was much more sensitive than CT in demonstrating these lesions and also was more specific in showing the characteristic sedimentation levels seen with subacute hemorrhages. Other imaging examinations con­ firmed the presence of additional hemangiomas in the long bones and liver. Despite a stormy course over the first 2 weeks of life , this infant responded successfully to steroid therapy and is doing well at 16 months of age.
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