Diffuse neonatal haemangiomatosis: new views on diagnostic criteria and prognosis.

Diffuse neonatal haemangiomatosis (DNH) is a rare and life-threatening congenital disorder. An extensive retrospective analysis of the literature was performed to evaluate the clinical features, therapies and prognostic factors of DNH. Reports on 68 patients with DNH were obtained. The skin, liver, lungs, brain and intestine were the organs most commonly involved. Congestive heart failure (CHF) was the primary cause of death. The mortality rate was 77.4% in untreated patients and 27% in treated patients. CHF, Kasabach-Merritt syndrome (KMS) and the involvement of five or more organs were important risk factors in DNH. The measurement of cardiac output might give more insight into the potential prognostic value of total blood-volume loss through shunting in the haemangiomas. Reports on 64 patients with neonatal haemangiomatosis limited to only the skin and liver were also obtained. The clinical features and outcome of patients with only cutaneous and hepatic haemangiomas were similar to those of patients with DNH. The inclusion criteria for DNH should be expanded to include similar patients with only cutaneous and hepatic haemangiomas.