Nasal glial heterotopia in a newborn infant

Abstract

A newborn female infant presented at birth with a congenital, 33 · 25 · 25-mm mass located on the nasal bridge and protruding along the left nasopalpebral region (Fig. 1). The lesion had never bled and there were no problems associated with feeding or breathing. Physical examination revealed a round, solid, nonpulsating, painless tumor covered by erythematous skin with superficial telangiectasias. This mass showed no growth or change in size during crying or jugular vein compression (Furstenberg sign). There were no signs of visual or airway obstruction. The remainder of the physical examination was unremarkable. Magnetic resonance imaging (MRI) was requested, and sagittal MRI reconstruction images showed that the lesion did not exhibit intracranial extension (Fig. 2). Based on the clinical appearance of the lesion and lack of intracranial extension, a presumptive diagnosis of lymphangioma of the nasal bridge was established. Serial ophthalmologic examinations were recommended to assess any visual impairment. During the following months, neither rapid growth nor regression of the lesion was observed, which raised the first clinical suspicion of a misdiagnosis. The infant was referred to the Department of Plastic Surgery, and surgical excision was performed at the age of 18 months (Fig. 3) to prevent further secondary distortion of the nasal bridge and visual developmental sequelae. Pathologic evaluation of the excised mass showed skin overlying glial tissue positive for glial fibrillary acid protein (GFAP) and enlarged neurons positive for synaptophysin (Fig. 4), consistent with neuroglial heterotopia. At the age of 2.5 years, the child is doing well with no evidence of local recurrence (Fig. 5). Discussion