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S. kedar, D. Ghate, V. Vedanarayanan, N. Mungan, J. J. Corbett, C. J. Chen; Perinatal Onset Static Encephalopathy: An Underrecognized Cause of Optic Nerve Head Cupping in Children. Invest. Ophthalmol. Vis. Sci. 2009;50(13):4031.
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42 consecutive children with POSE (without lasered retinas or intraocular disease) were included. ONH was evaluated independently by 2 ophthalmologists by dilated indirect ophthalmoscopy. A child’s ONH were labeled as large cup (cup/disc ratio ≥ 0.5) and pale only if both observers agreed. ONH imaging was offered to all children but was physically difficult for most. The pediatric neurologist (VV) determined age of onset of POSE clinically and reviewed the available neuroimaging.
43% of the children with POSE had large cups and 33% had pallor. Large cups were seen in 55% of children with neurologic insult (POSE onset) ≤ 28 weeks of gestation (n=11) and 39% with onset > 28 weeks (n=31) (p=0.4). In children with hemiplegic (n=11), diplegic (n=7), quadriplegic (n=21) and miscellaneous (n=3) CP, large cups were found in 18%, 57%, 52% and 33% respectively (p=0.2) and ONH pallor in 9%, 14%, 52% and 33% respectively (p=0.06). In children who were ambulatory (n=24), and non-ambulatory (n=18), large cups were found in 29% and 61% respectively (p=0.04) and ONH pallor in 13% and 61% respectively (p=0.001). Brain CT/MRI was reviewed in 20/42 children. All children with large cups and/or pallor had white matter changes in the posterior periventricular region at the level of the atrium.
ONH cupping and pallor is common in POSE. ONH cupping and pallor in POSE is not associated with age of onset as previously hypothesized, but is associated with more severe forms of CP (diplegic and quadriplegic), more severe motor disability (non-ambulatory status) and changes on neuroimaging in the posterior periventricular region.ONH changes in POSE may reflect global CNS pathology manifested by major motor disability and seen as white matter changes on neuroimaging. Diagnosis of CP and determination of the severity of disability is difficult in infancy; children with large cups and/or disc pallor without glaucoma should be referred to pediatric neurologists for evaluation and neuro-imaging.
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