title: Optic nerve hypoplasia, encephalopathy, and neurodevelopmental handicap.
abstract: Abnormalities of the central nervous system are frequently described in optic nerve hypoplasia. In a longitudinal study of 46 consecutive children (32 term, 14 preterm) with bilateral optic nerve hypoplasia 32 (69.5%) had associated neurodevelopmental handicap. Of these, 90% had structural central nervous system abnormalities on computed tomographic brain scans. Neurodevelopmental handicap occurred in 62.5% of the term and 86% of the preterm infants respectively. Term infants had a greater incidence of ventral developmental midline defects and proportionately fewer maternal and/or neonatal complications throughout pregnancy, while encephaloclastic lesions were commoner among the premature infants. An association of optic nerve hypoplasia with the twin transfusion syndrome and prenatal vascular encephalopathies is described.
authors.affiliation: Department of Paediatric Ophthalmology, Children's Hospital, Dublin 1, Ireland.
authors.forename: J P
authors.initials: JP
authors.lastname: Burke
authors.forename: M
authors.initials: M
authors.lastname: O'Keefe
authors.forename: R
authors.initials: R
authors.lastname: Bowell
mesh_terms: D000015:Abnormalities, Multiple; D001921:Brain / Q000002:abnormalities*; D002547:Cerebral Palsy / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D004827:Epilepsy / Q000209:etiology*; D005074:Evoked Potentials, Visual / Q000502:physiology; D005260:Female; D005330:Fetofetal Transfusion; D005865:Gestational Age; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000209:etiology*; D008137:Longitudinal Studies; D008297:Male; D009900:Optic Nerve / Q000002:abnormalities*; D011247:Pregnancy; D014792:Visual Acuity / Q000502:physiology