Optic nerve hypoplasia

Lambert, Scott R.; Hoyt, Creig S.; Narahara, Michael

doi:10.1016/0039-6257(87)90069-5

Cited by 128 publications

(62 citation statements)

References 79 publications

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“…DD/DM ratios of 0.3 or greater are associated with normal vision and were classed as reference eyes; those between 0.3 and 0.15 were classed as moderate ONH; and a DD/DM of 0.15 or less was severe ONH. 6 The observer also judged the retinal vessels for tortuosity (ranked within normal limits, tortuous veins only or tortuous veins and arteries) and assessed the presence or absence of double ring sign. Optic nerve colour was classified as normal, temporal pallor, or overall pallor.…”

Section: Ocular Biometry and Clinical Signsmentioning

confidence: 99%

“…1 ONH may occur as an isolated unilateral or bilateral condition but it is also frequently associated with other congenital anomalies, often as part of the triad of septo-optic dysplasia, De Moisiers Syndrome (ONH with pituitary hormone deficiencies and midline brain lesions). [2][3][4][5][6] Although a number of risk factors have been identified, the pathogenesis of ONH remains unclear and is likely multifactorial.…”

Section: Introductionmentioning

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%

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

McCulloch

Garcia-Filion

Boemel

et al. 2006

Eye

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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.

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Section: Ocular Biometry and Clinical Signsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

McCulloch

Garcia-Filion

Boemel

et al. 2006

Eye

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“…Supranormal regression of axons in the optic nerve (rather than a primary failure of differentiation) is thought to be the principal mechanism of development of ONH. 46 Histopathologically, the lack of degenerated neurons in ONH is indicative of axonal loss due to apoptosis during development. 47 Risk factors for ONH include young maternal age, first parity, maternal smoking, and preterm birth and its complications.…”

Section: Aetiologymentioning

confidence: 99%

Congenital disorders of the optic nerve: excavations and hypoplasia

Dutton

2004

Eye

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“…134 Probably most frequent Growth hormone (GHD) 19 May not be clinically apparent for 3 years þ 216 Thyroid hormone (THD) 206 Suggested by neonatal jaundice? 206,217 Corticotrophin…”

Section: Endocrine Deficiency Commentmentioning

confidence: 99%