Optic nerve morphology as marker for disease severity in cerebral palsy of perinatal origin.

Background: It is difficult to predict the neurologic outcome and ambulatory status in children with perinatal neurologic insult until 2-5years age. This study aims to correlate clinical optic nerve head (ONH) findings-cupping, pallor and hypoplasia, with gestational period and neurologic (motor) outcomes in patients with cerebral palsy (CP) from perinatal insults.

Methods: 54 consecutive patients with CP from perinatal insults were enrolled. Patients with intraocular disease, retinopathy of prematurity and hydrocephalus were excluded. ONH was labeled as pale, hypoplastic or large cup (cup/disc ratio≥0.5) if 2 ophthalmologists independently agreed after an ophthalmoscopic examination. Inter-rater reliability was excellent.

Results: Mean age at examination was 10.98±6.49years; mean gestational period was 33.26±4.78weeks. Abnormal ONH (pallor, cupping or hypoplasia) was seen in 38/54 (70%) patients. Of patients with pallor (n=17), 88% were quadriplegic and 82% non-ambulatory. Mean cup/disc ratio was 0.45±0.22; 50% patients had large cup. Multivariate logistic regression models showed that disc pallor was associated with non-ambulatory status (OR: 21.7; p=0.003) and quadriplegia (OR: 12.8; p=0.03). Large cup was associated with age at examination (OR 1.15; p=0.03). Cup/disc ratio showed positive correlation with age at examination (Pearson's r=0.39; p=0.003). There was no significant association of ONH parameters with gestational age.

Conclusion: Clinically observed ONH changes (pallor, cupping and hypoplasia) are common in CP. Presence of ONH pallor serves as an indicator for poor motor outcome in patients who develop CP from perinatal causes and should prompt early referral for rehabilitation.