Degree of tectal beaking correlates to the presence of nystagmus in children with Chiari II malformation

Tectal beaking is a common finding in children born with a myelomeningocele. Quantitation of the presence or absence of this finding correlated with normal or abnormal eye movements is lacking in the medical literature. We prospectively evaluated 50 children in our multidisciplinary spina bifida clinic. Each child was observed for the presence or absence of nystagmus at rest or with extraocular movement. Grades of nystagmus and severity of tectal beaking (Types I-III) on MRI were then correlated. Overwhelmingly, children with greater dysmorphology of the mesencephalic tectum were most likely to have nystagmus. Higher grades of nystagmus were associated with Type III tectal beaking. We have demonstrated that there is a correlation between the presence and severity of nystagmus and the severity of tectal beaking and have quantified this association in patients with the Chiari II malformation. This information may act as a guide in predicting which children with the Chiari II malformation will have significant nystagmus. These data may aid the clinician in the interpretation of oculomotor abnormalities in this cohort of patients.