Clinical Assessment, Optic Disk Measurements, and Visual-Evoked Potential in Optic Nerve Hypoplasia

Borchert, Mark; McCulloch, Daphne L.; Rother, C.; Stout, Ann U.

doi:10.1016/s0002-9394(14)72207-x

Cited by 45 publications

(46 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Inclusion criteria are referral before 3 years of age and a small optic nerve in at least one eye defined by a ratio of less than 0.3 between the disk diameter (DD) and the disk macula (DM) distance (DD/DM). 7 Parents gave fully informed consent to participate in the study, which was approved by the hospital ethical committee and conforms to the declaration of Helsinki. A comprehensive protocol of ophthalmology, neuro-radiography, endocrine, and psychometric assessments is carried out annually until 5 years of age.…”

Section: Subjectsmentioning

confidence: 99%

“…[5][6][7] Along the severity continuum, there are some cases with sectoral defects in the optic nerve head, and there is a frequent coincidence of ONH and pallor of these small optic nerve heads. 8 Prognosis for vision can be difficult to establish based on clinical observation.…”

Section: Introductionmentioning

confidence: 99%

“…In ONH, the amplitude of VEPs to flash stimulation and the presence of VEPs to pattern stimulation are correlated with visual function. 7,14,[17][18][19] Typically, ONH is characterized by normal full-field electroretinograms (ERGs) and abnormal VEPs. [16][17][18][19] The a-waves of flash ERGs are consistently reported as normal in ONH, but several authors have reported abnormalities in the b-wave.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Retinal function in infants with optic nerve hypoplasia: electroretinograms to large patterns and photopic flash

McCulloch

Garcia-Filion

Boemel

et al. 2006

Eye

View full text Add to dashboard Cite

Aims Optic nerve hypoplasia (ONH), which is defined as a congenital deficiency of retinal ganglion cells, may also involve more distal layers of the retina. We investigated electrophysiological function of the retina in ONH using electroretinograms (ERGs). Methods ERGs were recorded from 48 subjects (3.5-35 months) with unilateral or bilateral ONH. Pattern reversal (41 checks) was presented under chloral hydrate sedation, using an optical system to correct a cycloplegic refraction. A photopic flash stimulus was also used. Fundus photographs were used to measure the disk diameter/disk macula ratio (DD/DM), and to document other clinical signs. Eyes were classified as moderate (0.15-0.3) or severe (o0.15) ONH, and those with DD/DM greater than 0.3 were used as reference eyes. Results Pattern ERG recording was completed in 89 eyes and was detectable in 80% of eyes with ONH (61/76 tested) and in all 13 reference eyes. Photopic flash ERGs were of good quality in all eyes. The severity of ONH correlates with the amplitude of the photopic flash b-waves and with the amplitude of the N95 component of the pattern ERG (Po0.01). However, the ERGs to large patterns were well preserved (43.5 lV) in 10 of 35 eyes with severe ONH. Tortuous retinal vessels in eyes with either moderate or severe ONH were associated with smaller amplitude photopic b-waves and markedly diminished or undetectable pattern ERGs. Conclusions This study supports the hypothesis that retinal dysfunction distal to the ganglion cells is common in ONH, but is not predictable on the basis of ONH severity alone. Additionally, tortuous retinal vessels in ONH may be a sign associated with retinal dysfunction.

show abstract

Section: Subjectsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Retinal function in infants with optic nerve hypoplasia: electroretinograms to large patterns and photopic flash

McCulloch

Garcia-Filion

Boemel

et al. 2006

Eye

View full text Add to dashboard Cite

show abstract

“…In one study of 19 children with ONH, all eyes with a DM : DD ratio of more than 3 had reduced visual acuities but all those with a ratio of less than 3 had normal acuities. 76 In another study, however, in 75% of bilateral cases, the eye with the relatively smaller optic disc was paradoxically found to have a better visual acuity than the fellow eye. 74 Both the degree of afferent pupillary dysfunction 76 and reduction in brightness sense 77 are also related to the level of acuity.…”

Section: Clinical Featuresmentioning

confidence: 91%

Congenital disorders of the optic nerve: excavations and hypoplasia

Dutton

2004

Eye

View full text Add to dashboard Cite

show abstract

“…[2][3][4] Optic nerve size is used to approximate severity of disease and vision potential in children with ONH. 5 This can be done subjectively through indirect ophthalmoscopy or objectively by measuring the relative optic disk size on fundus photography. A study by De Silva et al, 6 reporting 51% growth in the optic disk relative to other fundus landmarks between birth and adulthood, raises the question of whether such optic disk measurements require adjustment by age.…”

Section: Introductionmentioning

confidence: 99%

Absence of age-related optic disk changes in young children with optic nerve hypoplasia

Garcia-Filion

Fink

et al. 2014

Eye

View full text Add to dashboard Cite

Purpose To determine whether the ratio of optic disk diameter to disk-to-macula distance (DD/DM) in children with optic nerve hypoplasia (ONH) changes over time.Patients and methods Fifteen subjects (29 eyes) enrolled in a prospective registry study on ONH had fundus photography performed under the age of 24 months and again at 60 months. Using the ratio of the DD/DM method, the relative size of the optic disk was assessed twice for each photo by one masked expert examiner to determine whether any change in relative disk size occurred over time.Results There was no change in relative optic disk size between initial and final fundus photographs. The average ( ± SD) age at the time of initial and final fundus photography was, respectively, 11.6±5.2 months and 60.6±1.3 months. Strong concordance was noted between the average DD/DM of the initial and the final photographs (r ¼ 0.939; 95% CI: 0.893, 0.981). There was negligible difference between the individual time point measurements ( À 0.011±0.03) (95% LOA: À 0.07, 0.04). Conclusion There were no clinically significant changes in relative optic disk size over time in children with ONH; thus, DD/ DM measurements need not be adjusted by age beyond 1 year in children with this disorder.

show abstract

Clinical Assessment, Optic Disk Measurements, and Visual-Evoked Potential in Optic Nerve Hypoplasia

Cited by 45 publications

References 18 publications

Retinal function in infants with optic nerve hypoplasia: electroretinograms to large patterns and photopic flash

Retinal function in infants with optic nerve hypoplasia: electroretinograms to large patterns and photopic flash

Congenital disorders of the optic nerve: excavations and hypoplasia

Absence of age-related optic disk changes in young children with optic nerve hypoplasia

Contact Info

Product

Resources

About