Neonatal Med.  2016 May;23(2):116-120. 10.5385/nm.2016.23.2.116.

Diffuse Neonatal Hemangiomatosis with Unilateral Cranial Nerve Palsy Improved by Propranolol

• Kim SW
• Park JK
• Jeon GW
• Sin JB

• KMID: 2164926
• DOI: http://doi.org/10.5385/nm.2016.23.2.116

Abstract

Diffuse neonatal hemangiomatosis (DNH) is characterized by multiple capillary or cavernous hemangiomas on the skin and internal organs occurring during the neonatal period. It is a life-threatening condition due to high-output heart failure with a mortality rate of 60-85% without proper treatment. The areas that are most commonly involved include the skin (100%), liver (64-100%), and central nervous system (52%). Corticosteroids are the drugs of choice as an initial treatment and have a response rate of 30-60%. We present here a case of a newborn baby with multiple hemangiomas on her skin (scalp, lips, neck, back, shoulder, arm, buttock, and leg), brain (right cerebellum, pons, and medulla oblongata), lungs, liver, kidney, and bones. She suffered from 6th, 7th, 9th, 10th, and 12th cranial nerve palsy resulting from hemorrhage of the hemangiomas in the brain. The first-line treatment of prednisolone (4 mg/kg/day) was not effective and propranolol (2 mg/kg/day) was administered as a second-line treatment. After 2 weeks of treatment, the hemangiomas had decreased in size with no associated acute hemorrhage. The infant is now 10 months old and both the multiple hemangiomas and cranial nerve palsy have improved. Propranolol was effective without significant adverse effects in treating DNH resistant to corticosteroids.

Keyword

Abducens nerve palsy; Facial palsy; Hemangioma; Propranolol

MeSH Terms

Abducens Nerve Diseases
Adrenal Cortex Hormones
Arm
Brain
Buttocks
Capillaries
Central Nervous System
Cerebellum
Cranial Nerve Diseases*
Cranial Nerves*
Facial Paralysis
Heart Failure
Hemangioma
Hemangioma, Cavernous
Hemorrhage
Humans
Infant
Infant, Newborn
Kidney
Lip
Liver
Lung
Mortality
Neck
Pons
Prednisolone
Propranolol*
Shoulder
Skin
Adrenal Cortex Hormones
Prednisolone
Propranolol