Optic Nerve Hypoplasia With Isolated Tortuosity of the Retinal Veins

Hellström, Ann; Wiklund, Lars‐Martin; Svensson, Elisabeth; Albertsson‐Wikland, Kerstin; Strömland, Kerstin

doi:10.1001/archopht.117.7.880

Cited by 32 publications

(19 citation statements)

References 30 publications

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“…50,51 In the present study, we report a clear association between vascular tortuosity and retinal dyfunction, as indicated by absent pattern ERGs. This supports a link between vascular morphology and retinal dysfunction in a subset of our cases.…”

Section: Discussionsupporting

confidence: 61%

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: Discussionsupporting

confidence: 61%

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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“…69 ONH may be associated with tortuosity of the retinal vasculature. 70 This feature has in one study also been shown to be associated with the development of endocrinopathy. 70 A persistent grey appearance has also been described.…”

Section: Clinical Featuresmentioning

confidence: 87%

“…70 This feature has in one study also been shown to be associated with the development of endocrinopathy. 70 A persistent grey appearance has also been described. 71 The ratio of the distance between the centre of the optic disc to the centre of the macula (DM) and the mean optic disc diameter (DD) (ie the DM to DD ratio) provides an index of the size of the optic disc on clinical examination and on photography 72,73 (Figure 4).…”

Section: Clinical Featuresmentioning

confidence: 87%

Congenital disorders of the optic nerve: excavations and hypoplasia

Dutton

2004

Eye

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“…Bilateral or unilateral optic nerve hypoplasia is often associated with hypopituitarism [11] . When it is also associated with absence of the septum pellucidum, the condition is known as septo-optic dysplasia [19] . But macular ectopia associated with growth hormone deficiency has not been described.…”

Section: Discussionmentioning

confidence: 99%