Neuro-ophthalmological findings in relation to CNS lesions in patients with myelomeningocele.

Twenty-eight patients (14 male, 14 female; aged four to 34 years) with myelomeningocele were studied. Magnetic resonance imaging was used to determine the correlation between disturbances of ocular motility and the degree of hydrocephalus, tectal plate deformity and dislocation of the cerebellum and medulla oblongata. All patients had Chiari malformations. Strabismus and spontaneous nystagmus were strongly related mainly to the degree of hydrocephalus and to some extent to the amount of lower brainstem deformities. Ocular motility defects with regard to oblique muscle functions and horizontal and vertical gaze and saccadic control often correlated with lower brainstem lesions. Convergence defects correlated with deformities of the upper brainstem. However, these correlations were not valid in the individual patient; some had no strabismus and normal ocular motility, in spite of advanced hydrocephalus and Chiari malformations.