Multifocal infantile hemangioma with extracutenous involvement

Multifocal infantile hemangioma with extracutaneous involvement, also known as diffuse neonatal hemangiomatosis or disseminated infantile hemangiomatosis, is a rare vascular disorder characterized by the presence of multiple (typically five or more) infantile hemangiomas affecting both the skin and internal organs. This condition is distinct from benign neonatal hemangiomatosis, in which multiple cutaneous hemangiomas occur without visceral involvement. The most commonly affected internal organs include the liver, gastrointestinal tract, lungs, brain, and eyes. Hepatic hemangiomas are particularly frequent and can lead to serious complications including high-output cardiac failure, hepatomegaly, consumptive hypothyroidism, and Kasabach-Merritt phenomenon (a life-threatening coagulopathy with thrombocytopenia). Clinically, affected infants typically present at birth or within the first weeks of life with numerous small, red or bluish cutaneous hemangiomas. The severity of the condition depends largely on the extent and location of visceral involvement. Hepatic lesions may cause abdominal distension, anemia, and heart failure due to arteriovenous shunting. Gastrointestinal hemangiomas can lead to bleeding, while central nervous system involvement may cause neurological symptoms. Without treatment, the condition can be life-threatening, with mortality historically reported as high in cases with significant visceral disease. Treatment has evolved significantly with the introduction of oral propranolol (a beta-blocker), which is now considered first-line therapy and has dramatically improved outcomes. Propranolol accelerates involution of both cutaneous and visceral hemangiomas. Systemic corticosteroids may also be used, particularly in refractory cases. Additional supportive measures include management of cardiac failure, thyroid hormone replacement for consumptive hypothyroidism, and in rare cases, surgical intervention or embolization for life-threatening hepatic lesions. Early diagnosis through imaging (ultrasound, MRI) and prompt initiation of treatment are critical for improving survival and reducing complications.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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