A Newborn With Neurocutaneous Melanocytosis and Dandy-Walker Malformation

作者：De Cock Jens; Snauwaert Julie; Van Rompaey Walter; Morren Marie Anne; Demaerel Philippe^*

来源：Pediatric Neurology, 2014, 50(3): 276-278.

DOI：10.1016/j.pediatrneurol.2013.04.006

• neurocutaneous syndromes
• melanocytosis
• congenital nevi
• Dandy-Walker malformation

BACKGROUND: Neurocutaneous melanocytosis is a rare congenital dysplasia of the neuroectodermal melanocyte precursor cells that leads to proliferation of melanin-producing cells in the skin and leptomeninges. PATIENT: We describe a newborn with a giant congenital melanocytic nevus on his back, buttocks, and thighs. His brain magnetic resonance imaging study revealed bilateral T1 hyperintense lesions in the cerebellum and in the amygdala, hydrocephalus, and a Blake's pouch cyst, consistent with neurocutaneous melanocytosis and Dandy-Walker malformation. CONCLUSION: Neurocutaneous melanocytosis has a wide clinical spectrum that includes hydrocephalus, epilepsy, cranial nerve palsy, increased intracranial pressure, and sensorimotor deficits.

• 出版日期2014-3