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New-Onset Macular Schisis in a Patient With Glaucoma

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 70-year-old woman with an ocular history of primary open-angle glaucoma on latanoprost, bilateral trabeculectomies, and argon laser trabeculoplasties presented with several months of worsening vision in the left eye. On examination, her best-corrected distance visual acuity was 20/40 OD and 20/60 OS (baseline 20/40 OU). Pupils were equally round and reactive, without afferent pupillary defect. Findings of Amsler grid testing were notable for a small area of distortion inferiorly in the left eye. Intraocular pressures (IOPs) were 13 mm Hg in both eyes. Findings of anterior segment examination were unremarkable. Findings of dilated fundus examination were notable for cup-disc ratio of 0.9 in the right eye and 0.85 in the left eye with temporal thinning, stable from prior, and new intraretinal thickening in the left eye. Optical coherence tomography (OCT) imaging of the left eye revealed schiticlike separation of the outer plexiform and outer nuclear layers extending from the temporal edge of the optic nerve head to the fovea (Figure 1A), continuous with the peripapillary nerve sheath.

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Macular retinoschisis in the setting of glaucomatous optic atrophy

C. Observation

The patient’s decreased vision was due to macular retinoschisis, an abnormal splitting of the neuroretina associated with glaucomatous optic atrophy. Observation (choice C) is correct because the patient noted only mild distortion, and retinoschisis can remain stable1 or resolve spontaneously2,3 in up to 40% of cases.3 Schisis can resolve after vitrectomy,1 so surgery (choice A) could be pursued if symptoms worsened. Barrier laser (choice B), as described by Prinzi et al,4 has been used to treat retinoschisis in glaucoma; however, peripapillary laser photocoagulation carries risk of vision loss and the patient had only a 2-line decrease in vision. Testing for the RS1 gene (choice D) would be indicated if X-linked retinoschisis was suspected; however, this disease appears almost exclusively in boys.

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Article Information

Corresponding Author: Rajendra S. Apte, MD, PhD, Department of Ophthalmology and Visual Sciences, Washington University, 660 S Euclid Ave, Campus Box 8096, St Louis, MO 63110 (apte@wustl.edu).

Published Online: September 2, 2021. doi:10.1001/jamaophthalmol.2021.0939

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Zumbro  DS , Jampol  LM , Folk  JC , Olivier  MM , Anderson-Nelson  S .  Macular schisis and detachment associated with presumed acquired enlarged optic nerve head cups.   Am J Ophthalmol. 2007;144(1):70-74. doi:10.1016/j.ajo.2007.03.027PubMedGoogle ScholarCrossref
2.
Farjad  H , Besada  E , Frauens  BJ .  Peripapillary schisis with serous detachment in advanced glaucoma.   Optom Vis Sci. 2010;87(3):E205-E217. doi:10.1097/OPX.0b013e3181d1dad5PubMedGoogle ScholarCrossref
3.
Lee  EJ , Kim  TW , Kim  M , Choi  YJ .  Peripapillary retinoschisis in glaucomatous eyes.   PLoS One. 2014;9(2):e90129. doi:10.1371/journal.pone.0090129Google Scholar
4.
Prinzi  RA , Desai  A , Gao  H .  Laser treatment of macular retinoschisis due to acquired optic nerve pit from glaucoma.   BMJ Case Rep. 2015;2015:bcr2015211036. doi:10.1136/bcr-2015-211036PubMedGoogle Scholar
5.
Radius  RL , Maumenee  AE , Green  WR .  Pit-like changes of the optic nerve head in open-angle glaucoma.   Br J Ophthalmol. 1978;62(6):389-393. doi:10.1136/bjo.62.6.389PubMedGoogle ScholarCrossref
6.
Lincoff  H , Lopez  R , Kreissig  I , Yannuzzi  L , Cox  M , Burton  T .  Retinoschisis associated with optic nerve pits.   Arch Ophthalmol. 1988;106(1):61-67. doi:10.1001/archopht.1988.01060130067030PubMedGoogle ScholarCrossref
7.
Zhao  M , Li  X .  Macular retinoschisis associated with normal tension glaucoma.   Graefes Arch Clin Exp Ophthalmol. 2011;249(8):1255-1258. doi:10.1007/s00417-011-1668-yPubMedGoogle ScholarCrossref
8.
Woo  R , Akil  H , Koulisis  N , Olmos de Koo  LC , Tan  JCH .  Sustained resolution of macular retinoschisis after trabeculectomy in a patient with progressive glaucoma.   J Glaucoma. 2017;26(6):e180-e186. doi:10.1097/IJG.0000000000000632PubMedGoogle ScholarCrossref
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