Positive angle kappa: a sign of albinism in patients with congenital nystagmus

Brodsky, Michael C.; Fray, Katherine J.

doi:10.1016/j.ajo.2003.11.066

Cited by 14 publications

(12 citation statements)

References 12 publications

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“…Albinism occurs in approximately 1 in 17,000 in the United States [1,2]. Ocular manifestations typically include reduced visual acuity, nystagmus, iris transillumination, foveal hypoplasia, and misrouting of the retinostriate fibers, which refers to excessive decussation of some of the temporal nerve fibers to the contralateral central visual pathway [3][4][5]. A wide spectrum of visual acuity has been reported in albinism.…”

Section: Introductionmentioning

confidence: 99%

The optic nerve and peripapillary vasculature in albinism

Conley¹,

Mancera²,

Holleschau³

et al. 2016

New Front Ophthalmol

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Albinism, an inherited disorder resulting in reduced ocular melanin, is usually associated with reduced best-corrected visual acuity, nystagmus, foveal hypoplasia, and iris transillumination. However, a spectrum of findings has been reported, with foveal hypoplasia, nystagmus, and misrouting of the retinostriate fibers having been purported to cause reduced visual acuity. The purpose of this case-controlled study is to describe the optic nerve anatomy and peripapillary retinal vasculature in albinism and to determine if there are changes related to visual acuity. We evaluated digital fundus photographs using computer analysis software in 34 patients with albinism and 51 controls. Our data indicate that the optic disc diameter and optic nerve area are significantly smaller in patients with albinism than controls (p <0.001; p=0.0008, respectively). Significantly more patients with albinism also had a double ring sign, situs inversus, and a nasally-directed artery (p ≤ 0.0148). There were no significant differences between groups for optic nerve color, vessel branching pattern, cilioretinal artery, or number of vessels crossing the optic nerve margin. Correlation of best-corrected visual acuity to disc diameter and optic nerve area was poor (r=0.0542 r=0.1527, respectively). In this study, patients with albinism had mild optic nerve hypoplasia when compared to controls. Reduced best-corrected visual acuity is not explained by the optic nerve findings. Further studies are required to elucidate the causes of reduced best-corrected visual acuity in albinism.

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Section: Introductionmentioning

confidence: 99%

The optic nerve and peripapillary vasculature in albinism

Conley¹,

Mancera²,

Holleschau³

et al. 2016

New Front Ophthalmol

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show abstract

“…3 They have also been reported in patients with congenital nystagmus associated with albinism. 4 The line of sight can also deviate from the pupillary axis along the vertical axis, creating a vertical angle kappa, but this has been a relatively rare finding. 5 In the modern era of macular surgery, however, vertical macular dislocation has become more common.…”

Section: Discussionmentioning

confidence: 98%

Abnormal vertical angle kappa caused by ectopic fovea

Fabian

Spierer

Berger

et al. 2012

Journal of American Association for Pediatric Ophthalmology and

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“…Both disorders are characterized by a significant reduction in central visual acuity, hypopigmentation of the retinal pigmented epithelium, foveal hypoplasia, and misrouting of the retinal ganglion cell axons through the chiasm. 7 The detection of iris transillumination defects is the most diagnostically important finding to identify these disorders. The recognition of a positive angle k may also suggest these diagnoses.…”

Section: Early Onset Nystagmusmentioning

confidence: 99%