open access

Vol 4 (2019): Continuous Publishing
Case report
Published online: 2019-07-31
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Refractory macular edema in X-linked juvenile retinoschisis

Rashed Mustafa Nazzal1, Robert Rejdak1
·
Ophthalmol J 2019;4:46-48.
Affiliations
  1. Department of Surgery/Ophthalmology, King Hussein Cancer Center (KHCC), Amman, Jordan

open access

Vol 4 (2019): Continuous Publishing
CASE REPORTS
Published online: 2019-07-31

Abstract

X-linked juvenile retinoschisis (XLRS) is one of the most common X-linked inherited, bilateral vitreoretinal dystrophies
affecting males in the first two decades of life. In this article, we present a case report of a young man who
presented with gradual decrease in visual acuity in both eyes that was attributed to macular edema and foveal schisis
in the inner retinal layers. He was investigated by appropriate ocular images which were consistent with a diagnosis
of XLRS. He was treated by anti-vascular endothelial growth factor (anti-VEGF) intravitreal injection, dorzolamide
eye drops and intravitreal steroids injection without improvement.

Abstract

X-linked juvenile retinoschisis (XLRS) is one of the most common X-linked inherited, bilateral vitreoretinal dystrophies
affecting males in the first two decades of life. In this article, we present a case report of a young man who
presented with gradual decrease in visual acuity in both eyes that was attributed to macular edema and foveal schisis
in the inner retinal layers. He was investigated by appropriate ocular images which were consistent with a diagnosis
of XLRS. He was treated by anti-vascular endothelial growth factor (anti-VEGF) intravitreal injection, dorzolamide
eye drops and intravitreal steroids injection without improvement.

Get Citation

Keywords

retinoschisis; macular edema; juvenile; carbonic anhydrase inhibitors; vascular endothelial growth factor

About this article
Title

Refractory macular edema in X-linked juvenile retinoschisis

Journal

Ophthalmology Journal

Issue

Vol 4 (2019): Continuous Publishing

Article type

Case report

Pages

46-48

Published online

2019-07-31

Page views

594

Article views/downloads

806

DOI

10.5603/OJ.2019.0005

Bibliographic record

Ophthalmol J 2019;4:46-48.

Keywords

retinoschisis
macular edema
juvenile
carbonic anhydrase inhibitors
vascular endothelial growth factor

Authors

Rashed Mustafa Nazzal
Robert Rejdak

References (13)
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  2. Tantri A, Vrabec TR, Cu-Unjieng A, et al. X-linked retinoschisis: a clinical and molecular genetic review. Surv Ophthalmol. 2004; 49(2): 214–230.
  3. Ansari WH, Browne AW, Singh RP. Juvenile X-linked retinoschisis responsive to intravitreal corticosteroids. Am J Ophthalmol Case Rep. 2017; 5: 48–51.
  4. Forsius H, Krause U, Helve J, et al. Visual acuity in 183 cases of X-chromosomal retinoschisis. Can J Ophthalmol. 1973; 8(3): 385–393.
  5. Sikkink SK, Biswas S, Parry NRA, et al. X-linked retinoschisis: an update. J Med Genet. 2007; 44(4): 225–232.
  6. Hu QR, Huang LZ, Chen XL, et al. Genetic analysis and clinical features of X-linked retinoschisis in Chinese patients. Sci Rep. 2017; 7: 44060.
  7. Hu QR, Huang LZ, Chen XL, et al. X-Linked Retinoschisis in Juveniles: Follow-Up by Optical Coherence Tomography. Biomed Res Int. 2017; 2017: 1704623.
  8. Witkin A.J., Duker J.S. Young woman presents with decreasing vision. Ocul Surg News US Ed. 2008. https://www.healio.com/ophthalmology/retina-vitreous/news/print/ocular-surgery-news/%7B120df068-796b-4ef7-9ea5-0ad4335772e8%7D/young-woman-presents-with-decreasing-vision.
  9. Wang JK, Lin WC. Posterior subtenon injection of triamcinolone acetonide after phacoemulsification in a patient with X-linked retinoschisis. Optom Vis Sci. 2013; 90(10): e274–e277.
  10. Deshmukh S, Gupta K, Das D, et al. Juvenile X-Linked retinoschisis: Response to topical dorzolamide therapy. TNOA J Ophthal Sci Res. 2018; 56(1): 35.
  11. Apushkin MA, Fishman GA. Use of dorzolamide for patients with X-linked retinoschisis. Retina. 2006; 26(7): 741–745.
  12. Ghajarnia M, Gorin MB. Acetazolamide in the treatment of X-linked retinoschisis maculopathy. Arch Ophthalmol. 2007; 125(4): 571–573.
  13. Coussa RG, Kapusta MA. Treatment of cystic cavities in X-linked juvenile retinoschisis: The first sequential cross-over treatment regimen with dorzolamide. Am J Ophthalmol Case Rep. 2017; 8: 1–3.

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