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Optic nerve hypoplasia, encephalopathy, and neurodevelopmental handicap
J P Burke1, M O'Keefe, R Bowell
1Department of Paediatric Ophthalmology, Children's Hospital, Dublin 1, Ireland.
The British Journal of Ophthalmology
|April 1, 1991
Summary
Optic nerve hypoplasia is often linked to central nervous system abnormalities, causing neurodevelopmental handicap in most affected children. This study highlights the prevalence and types of brain abnormalities in these cases.
Area of Science:
- Neuroscience
- Pediatric Neurology
- Developmental Biology
Background:
- Optic nerve hypoplasia (ONH) is frequently associated with central nervous system (CNS) abnormalities.
- Neurodevelopmental outcomes in children with ONH require further elucidation.
Purpose of the Study:
- To investigate the prevalence and characteristics of neurodevelopmental handicap in children with bilateral ONH.
- To identify associated structural CNS abnormalities and their relationship with gestational age and clinical presentation.
Main Methods:
- A longitudinal study of 46 consecutive children diagnosed with bilateral ONH.
- Neurodevelopmental assessment and computed tomographic (CT) brain scans were performed.
- Analysis of clinical data including gestational age, maternal/neonatal complications, and specific CNS findings.
Main Results:
- A significant neurodevelopmental handicap was observed in 69.5% of children with ONH.
- Ninety percent of those with handicap had structural CNS abnormalities identified on CT scans.
- Preterm infants showed a higher incidence of handicap (86%) and encephaloclastic lesions compared to term infants (62.5%), who had more midline defects.
Conclusions:
- Bilateral ONH is strongly associated with significant neurodevelopmental handicap and structural CNS abnormalities.
- Gestational age influences the type of CNS abnormalities and associated complications.
- ONH may be linked to conditions such as twin transfusion syndrome and prenatal vascular encephalopathies.