Abstract
Purpose:
It has been hypothesized that a large cup in premature children is associated with period of gestation (POG) > 28 weeks.Early prognostication in children with POSE will result in early rehabilitation with improved functional outcomes. Recognition of the association of POSE and large cups will prevent unnecessary examinations under anethesia for glaucoma.Our study aims to correlate optic nerve head (ONH) pallor and cupping to period of gestation (POG) at birth and severity of neurological damage in children with perinatal onset static encephalopathy (POSE).
Methods:
54 consecutive patients with POSE were enrolled. Exclusion criteria included genetic, metabolic or congenital structural brain abnormalities not related to perinatal complications; intraocular disease (ROP/glaucoma/cataract) and hydrocephalus. ONH morphology (pallor and cup to disc ratio-CDR) was assessed independently by 2 fellowship-trained ophthalmologists by dilated examination using direct and indirect ophthalmoscopy. ONH were labeled as pale or large cup (cup/disc ratio≥0.5) only if the 2 ophthalmologists agreed. Inter-rater reliability was >0.8 for all parameters . A pediatric neurologist determined eligibility, age of onset of POSE, neurological deficit and reviewed available neuroimaging.
Results:
Mean age was 11.88±6.53years; period of gestation at birth: 33.26±4.78weeks. 33/54 (61%) showed ONH pallor or cupping. Of 17 patients with ONH pallor, 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 significantly associated with non-ambulatory status (OR: 12.5; p=0.03) and quadriplegia (OR: 21.7; p=0.0025) and large cup was associated with age at examination (OR 1.15; p=0.025). Cup/disc ratio and age showed positive correlation (r=0.42; p=0.002). ONH parameters were not statistically associated with POG at birth.
Conclusions:
ONH changes are common in POSE and are not associated with POG. Optic disc pallor, a bedside clinical finding is a prognostic indicator for severe neurological insult in high-risk children with perinatal complications that should prompt early referral for rehabilitation. Optic disc cupping is correlated with the age at examination which may indicate that the cupping worsens with age.