April 2010
Volume 51, Issue 13
ARVO Annual Meeting Abstract  |   April 2010
X-Linked Retinoschisis: Long-Term Follow-Up Data
Author Affiliations & Notes
  • J. M. Rosenthal
    Wills Eye Institute, Philadelphia, Pennsylvania
  • W. S. Tasman
    Retina Service,
    Wills Eye Institute, Philadelphia, Pennsylvania
  • Footnotes
    Commercial Relationships  J.M. Rosenthal, None; W.S. Tasman, None.
  • Footnotes
    Support  None.
Investigative Ophthalmology & Visual Science April 2010, Vol.51, 1380. doi:
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      J. M. Rosenthal, W. S. Tasman; X-Linked Retinoschisis: Long-Term Follow-Up Data. Invest. Ophthalmol. Vis. Sci. 2010;51(13):1380.

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      © ARVO (1962-2015); The Authors (2016-present)

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Introduction: : Background: X-linked Retinoschisis (XLR) is a rare but disabling disorder wherein layers of the inner retina split. The macula takes on a petaloid appearance, and the peripheral retina is often involved. Patients with this disorder experience many complications, including vitreous hemorrhage, retinal detachment, and even complete visual loss to no light perception.

Purpose: : To obtain long-term follow-up of patients with XLR.

Methods: : Six males (12 eyes) with XLR, presenting between the years 1982 and 1994, continue to be followed. Those with less than 15 years of follow-up were excluded. Photographs, as well as OCT and fluorescein angiogram findings, were included in the study.

Results: : Twelve eyes of six patients diagnosed between ages 7 months to 10 years were followed for a range of 15 to 24 years, with a median length of follow-up of twenty years. Two of twelve eyes of these six patients became blind and painful, with a vision of no light perception. Six eyes of four patients experienced vitreous hemorrhages, some patients with multiple episodes of hemorrhage in the same eye. One eye underwent pars plana vitrectomy for non-clearing vitreous hemorrhage. Two eyes underwent laser treatment, one for a full thickness retinal break and one for exudation. The ages at last follow-up exam ranged from 18 to 34 and visual acuities ranged from 20/30 to no light perception, with three patients having at least one eye with an acuity of 20/70 or better, and five patients with an acuity of at least 20/400. The maculae of two patients lost the classic petaloid appearance during the period of follow-up. However their OCT findings still showed cystic changes in the macula.

Conclusions: : All patients in this study had useful vision (20/400), and half had at least one eye with an acuity of 20/70 or better.

Keywords: clinical (human) or epidemiologic studies: natural history • inner retina dysfunction: hereditary 

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