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Published online February 4, 2008
PEDIATRICS Vol. 121 No. 3 March 2008, pp. e653-e659 (doi:10.1542/peds.2007-1825)
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ARTICLE

Neuroradiographic, Endocrinologic, and Ophthalmic Correlates of Adverse Developmental Outcomes in Children With Optic Nerve Hypoplasia: A Prospective Study

Pamela Garcia-Filion, MPHa,b, Karen Epport, PhDc, Marvin Nelson, MDd,e, Colleen Azen, MSc, Mitchell E. Geffner, MDf,g, Cassandra Fink, MPHa, Mark Borchert, MDa,g

a Division of Pediatric Ophthalmology, Department of Ophthalmology
f Division of Pediatric Endocrinology, Diabetes, and Metabolism, Department of Pediatrics, University of Southern California Keck School of Medicine and Childrens Hospital Los Angeles, Los Angeles, California
b Division of Biostatistics, Department of Preventive Medicine
e Department of Radiology, University of Southern California Keck School of Medicine, Los Angeles, California
c General Clinical Research Center
d Department of Imaging Services
g Saban Research Institute, Childrens Hospital Los Angeles, Los Angeles, California

BACKGROUND. Developmental delay has been reported to occur with optic nerve hypoplasia, a leading cause of pediatric blindness, but has not been systematically examined for its prevalence and correlation with associated pathologies of optic nerve hypoplasia.

OBJECTIVE. The purpose of this study was to determine the developmental outcomes of children with optic nerve hypoplasia and the correlation of development with neuroradiographic, endocrinologic, and ophthalmic findings.

METHODS. We conducted a prospective analysis of 73 subjects diagnosed with optic nerve hypoplasia at <36 months of age for developmental outcomes at 5 years of age. Subjects underwent neuroradiographic imaging, endocrinologic testing and examination, and ophthalmologic examination; developmental outcomes were assessed by using the Battelle Developmental Inventory.

RESULTS. At 5 years of age, developmental delay was present in 71% of subjects with optic nerve hypoplasia. Of patients with unilateral (18%) and bilateral optic nerve hypoplasia, 39% and 78%, respectively, experienced developmental delay. Corpus callosum hypoplasia and hypothyroidism were significantly associated with poor outcome in all of the developmental domains and an increased risk of delay. Absence of the septum pellucidum was not associated with adverse development. Six subjects had neither a neuroradiographic nor an endocrinologic abnormality, and of those, 4 were developmentally delayed.

CONCLUSIOONS. These prospective data confirm the significant association of developmental delay with optic nerve hypoplasia and identify corpus callosum hypoplasia and hypothyroidism as strong correlates. A diagnosis of optic nerve hypoplasia warrants neuroradiographic and endocrinologic testing for risk factors of delay and developmental assessments for early intervention planning.

Key Words: Optic nerve hypoplasia • septo-optic dysplasia • de Morsier syndrome • developmental delay • cerebral malformations • corpus callosum • septum pellucidum • hypopituitarism • hypothyroidism

Abbreviations: ONH—optic nerve hypoplasia • CHLA—Childrens Hospital Los Angeles • DD/DM—disc diameter to disc-macula distance • CCH—corpus callosum hypoplasia • CCA—corpus callosum area • GH—growth hormone • BDI—Battelle Developmental Inventory • OR—odds ratio • CI—confidence interval • Pall—smallest P value • Padj—adjusted P value

Accepted Aug 20, 2007.


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