Skip to main content
NCBI home page
NIHPA Author Manuscripts logoLink to NIHPA Author Manuscripts
. Author manuscript; available in PMC: 2023 Jan 1.
Published in final edited form as: Retin Cases Brief Rep. 2022 Jan 1;16(1):73–76. doi: 10.1097/ICB.0000000000000920

Successful surgical treatment of macula-involving degenerative retinoschisis by vitrectomy and drainage of the schisis cavity

Eric B Desjarlais 1, Philip S Garza 1, Mark W Johnson 1, Thiran Jayasundera 1
PMCID: PMC7035175  NIHMSID: NIHMS1535526  PMID: 31453931

Abstract

Purpose:

To demonstrate the potential for visual recovery following surgery for early foveal involvement by progressive degenerative retinoschisis.

Method:

Surgical case report.

Results:

A 79-year-old woman was monitored over 4 years for degenerative retinoschisis in the inferotemporal periphery of both eyes. Over the course of follow-up, visual acuity in the left eye decreased from 20/40 to 20/100, concurrent with extension of the schisis cavity into the macula. Macular involvement was confirmed on OCT imaging and there was no rhegmatogenous retinal detachment. The patient elected to undergo 25-gauge pars plana vitrectomy and drainage of the schisis cavity. Eleven weeks after surgery, the macula remained attached, and the uncorrected visual acuity was 20/30. Fundus examination and SD-OCT confirmed ongoing resolution of the macula-involving retinoschisis.

Conclusion:

Bullous degenerative retinoschisis is thought to result in an absolute scotoma that is not surgically correctable. Our patient’s excellent visual recovery suggests that the synaptic integrity of the macular outer plexiform layer can be preserved and a permanent scotoma avoided if early macular involvement by progressive degenerative retinoschisis is surgically repaired.

Keywords: Retina, degenerative retinoschisis, acquired retinoschisis, macula-splitting retinoschisis, retinoschisis, vitrectomy

Summary Statement:

We present a case of degenerative retinoschisis progressing to involve the macula with deterioration of visual acuity. Visual acuity returned to baseline following surgical drainage of the schisis cavity, suggesting the synaptic integrity of the outer plexiform layer can be preserved before a permanent scotoma develops due to fovea-involving retinoschisis.

Introduction

Posterior encroachment is a rare complication of degenerative retinoschisis for which data and treatment options are limited, even when it progresses to involve the macula and significantly impacts vision.1 Surgical intervention is typically considered only when degenerative retinoschisis is complicated by retinal detachment.2,3,4 Likewise, medical treatment has been described only for the management of hereditary retinoschisis, and this has met with limited success.5 Visual recovery following vitrectomy and schisis cavity drainage has been reported once before in a case where macular encroachment was associated with retinal folds that involved the fovea.6 The authors report that the macula remained flat with improved visual function throughout postoperative follow-up despite recurrent retinoschisis in the inferotemporal periphery. Here, we present a case of foveal involvement by degenerative retinoschisis with sustained visual recovery following vitrectomy and surgical drainage of the retinoschisis cavity.

Report of a Case

A 79-year-old woman was monitored over 4 years for degenerative retinoschisis in the inferotemporal periphery of both eyes. Her history was remarkable for primary open angle glaucoma status post bilateral selective laser trabeculoplasty. Over the course of 3.5 years, visual acuity in the left eye decreased from 20/40 to 20/100, concurrent with extension of the retinoschisis cavity into the macula (Figure 1A and 1B). Fundus photographs taken at annual intervals demonstrated gradual posterior progression of the retinoschisis cavity corresponding with subjectively decreased vision. In the year prior to surgery, the fovea became involved with a measured change in visual acuity. Additional OCT imaging was performed to evaluate a small pocket of foveal subretinal fluid identified in the left eye, which revealed foveal involvement of retinoschisis without evidence of retinal detachment beneath the schisis cavity (Figure 2A-D).

Figure 1.

Figure 1.

Open in a new tab

Wide-field fundus photographs (left column), near-infrared fundus images (middle column), and SD-OCT of the macula (right column) of the left eye at initial presentation (A), before surgery, 3.5 years following initial presentation (B), and 11 weeks after surgery (C). Visual acuity at each stage is overlaid on the SD-OCT image.

Figure 2.

Figure 2.

Open in a new tab

Pre-operative near-infrared fundus images (left column) and wide-field SD-OCT images (right column) taken on the same day as the images presented in Figure 1B, demonstrating foveal involvement by retinoschisis and the presence of subfoveal fluid (row A), without evidence of rhegmatogenous retinal detachment on horizontal (row B), diagonal (row C), and vertical (row D) cross-sections through the schisis cavity.

The patient elected to undergo 25-gauge pars plana vitrectomy and drainage of the schisis cavity. After core vitrectomy, during which the posterior hyaloid was found to be detached to the retinal periphery, a retinotomy was created in the inner layer of the retina and the retinoschisis cavity was drained by active aspiration. Laser retinopexy was performed around the retinotomy site. The vitreous cavity was filled with 12% octafluoropropane.

The immediate post-operative course was uncomplicated. At one month of follow-up, the uncorrected visual acuity was 20/50 in the operated eye, but a localized retinal detachment was seen in the inferior periphery, with no causative break identified on ophthalmoscopy. The area of detachment was demarcated with laser retinopexy to prevent further progression. Eleven weeks after surgery, the macula remained attached, and the uncorrected visual acuity was 20/30. Fundus examination and SD-OCT imaging confirmed ongoing resolution of macula-splitting retinoschisis and there was no appreciable retinoschisis cavity inferotemporally (Figure 1C). At six months following surgery, the clinical findings were unchanged.

Discussion

Degenerative retinoschisis is generally characterized by splitting of the outer plexiform layer of the retina, disrupting the synapses between the photoreceptor cells of the outer nuclear layer and the neurons of the inner nuclear layer.7 This process results in an absolute scotoma that is thought not to be surgically correctable.8 However, our case demonstrates the potential reversibility of early foveal involvement by retinoschisis, with restoration of the patient’s visual acuity to baseline following surgical drainage of the schisis cavity.

Our patient’s decrease in visual acuity is attributable to both extension of the schisis fluid into the fovea and accumulation of subfoveal fluid (Figure 1B). The subfoveal fluid may have accumulated due to traction by the adjacent bullous retinoschisis cavity or simple diffusion of schisis fluid through the outer leaflet, as no outer break or retinal detachment was seen on examination or OCT imaging (Figure 2B-D). Although a faint epiretinal membrane was noted on OCT imaging, we considered its severity insufficient to produce visual symptoms or modify the progression or morphology of the retinoschisis cavity. Surgery was undertaken in our patient with the hope of resolving the subfoveal fluid and preventing irreversible disruption of the synapses in the outer plexiform layer as retinoschisis progressed through the macula. Our patient’s excellent postoperative visual outcome suggests that the synaptic integrity of the macula can be preserved before a permanent scotoma develops due to progressive retinoschisis. We cannot explain why peripheral retinoschisis recurred following surgical drainage in a similar case of posterior encroachment,6 but did not in our patient.

In their 1988 study of retinoschisis associated with optic nerve pits, Lincoff et al observed that the vision loss that evolves from the schisis-like macular thickening in this condition often improves with flattening of the retina. They suggested that visual potential may be preserved by Henle’s fibers, which assume a vertical orientation and span the schisis cavity.9 In 2010, Imamura et al disputed the use of “schisis” to describe intraretinal fluid accumulation in optic pit maculopathy, stating that retinoschisis describes a clean split between the retinal layers which evokes an absolute scotoma. 10 Our case describes a phenomenon similar to that observed by Lincoff et al, wherein diminished visual acuity returned to baseline following the resolution of degenerative macular schisis.

Conclusion

Historically, vitrectomy has been indicated for degenerative retinoschisis only in the context of progressive secondary retinal detachment. Numerous reports exist of visual recovery following vitrectomy for the management of rhegmatogenous detachment complicating degenerative retinoschisis, including those which involve the fovea.2,3,4 Our patient’s visual outcome demonstrates the utility of vitrectomy for the management of fovea-involving degenerative retinoschisis that is not complicated by rhegmatogenous detachment, indicating the potential reversibility of vision loss associated with early macula-involving retinoschisis.

Acknowledgments

Disclosure statement: Dr. Johnson serves on Data and Safety Monitoring Committees for Pfizer and Chengdu Kanghong Biotechnology, and as site Principal Investigator for a clinical trial sponsored by Apellis Pharmaceuticals. Dr. Jayasundera’s research is funded by National Institute of Health grant K23EY026985.

References

  • 1.Byer NE. Long-Term Natural History Study of Senile Retinoschisis with Implications for Management. Ophthalmology. 1986;93:1127–1137 [DOI] [PubMed] [Google Scholar]
  • 2.Stem MS, Garretson BR, Todorich B, et al. Anatomic and visual outcomes following surgical repair of retinoschisis-related retinal detachments. J Vitreoretin Dis. 2019;3(1):6–9 [Google Scholar]
  • 3.Robert CW, Folk JC, Andreas KL. Foveal Involvement by Acquired Retinoschisis, Long-Term Visual Outcomes. Retina. 2013;33:606–612 [DOI] [PubMed] [Google Scholar]
  • 4.Sneed SR, Blodi CF, Folk JC, Weingeist TA, Pulido JS. Pars Plana Vitrectomy in the Management of Retinal Detachments Associated with Degenerative Retinoschisis. Ophthalmology. 1990;97:470–474 [DOI] [PubMed] [Google Scholar]
  • 5.Khandhadia S, Trump D, Menon G, Lotery AJ. X-linked retinoschisis maculopathy treated with topical dorzolamide, and relationship to genotype. Eye (Lond). 2011;25(7):922–928. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 6.Yu S, Gao Y, Liang X, Huang Y. Acquired retinoschisis resolved after 23Gage pars plana vitrectomy in posterior microphthalmos. BMC Ophthalmology. 2014;14:65. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 7.Diseases of the retina Duke-Elder S (Ed.), System of Ophthalmology, vol. 10, Mosby, St Louis: (1967), pp. 559–568 [Google Scholar]
  • 8.Shea M, Schepens CL, Pirquet Von SR. Retinoschisis: I. Senile type: A clinical report of one hundred seven cases. AMA Arch Ophthalmol. 1960;63:25–33. [DOI] [PubMed] [Google Scholar]
  • 9.Lincoff H, Lopez R, Kreissig, et al. Retinoschisis associated with optic nerve pits. AMA Arch Ophthalmol. 1988;106(1):61–67 [DOI] [PubMed] [Google Scholar]
  • 10.Imamura Y, Zweifel SA, Fujiwara T. High-resolution optical coherence tomography findings in optic pit maculopathy. Retina. 2010;30(7):1104–1112 [DOI] [PubMed] [Google Scholar]

RESOURCES