Background: Persistent hyperplastic primary vitreous (PHPV) is usually a unilateral ocular abnormality not associated with other systemic findings. We describe 16 patients, 7 with unilateral and 9 with bilateral PHPV, in whom significant neurologic abnormalities were identified.
Methods: The clinical records of 16 children referred to the Visually Impaired Program at a university-affiliated children's hospital in Vancouver were studied from 1970 to 1996. Further investigations, including electroencephalography, computed tomography and pediatric neurologic evaluation, were performed to ascertain neurologic deficits.
Results: Neurologic abnormalities were identified in three of the children with unilateral PHPV (hemiparesis, impaired coordination and homonymous hemianopia) and seven of the children with bilateral PHPV (ataxia, impaired coordination, hypotonia, spastic quadriplegia, microcephaly and deafness).
Interpretation: The possibility of underlying neurologic abnormality should be considered in patients with PHPV, particularly those with bilateral disease. Referral to a pediatric neurologist may be appropriate.