Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis and Central Anomalous Retinoschisis with mid-PEripheral Traction

Authors

Alessandro Feo, Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California of Los Angeles, David Geffen School of Medicine at UCLA, Los Angeles, California; Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, 20072. Pieve Emanuele-Milan, Italy.
Andrea Govetto, Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, 20072. Pieve Emanuele-Milan, Italy; Department of Ophthalmology, Eye Unit Humanitas Gavazzeni-Castelli, via Mazzini 11, Bergamo, Italy.
Prithvi Ramtohul, Ophthalmology Department, Hopital Nord, Aix-Marseille University, Marseille, France; Vitreous Retina Macula Consultants of New York, New York, New York.
Néda Abraham, Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California of Los Angeles, David Geffen School of Medicine at UCLA, Los Angeles, California.
Diogo Cabral, Vitreous Retina Macula Consultants of New York, New York, New York; Department of Ophthalmology, Unidade Local de Saúde Almada Seixal, Almada, Portugal; NOVA Medical School, Universidade NOVA de Lisboa, Lisbon, Portugal.
Peter Y. Chang, Massachusetts Eye Research and Surgery Institution, Waltham, Massachusetts.
Nauman Chaudhry, Retina Group of New England, Waterford, Connecticut.
Fred K. Chen, Centre for Ophthalmology and Visual Science (incorporating Lions Eye Institute), The University of Western Australia, Perth, Western Australia, Australia.
Dean Eliott, Department of Ophthalmology, Massachusetts Eye & Ear, Harvard Medical School, Boston, Massachusetts.
Livia Faes, Vitreous Retina Macula Consultants of New York, New York, New York; Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom.
Rachael C. Heath Jeffery, Centre for Ophthalmology and Visual Science (incorporating Lions Eye Institute), The University of Western Australia, Perth, Western Australia, Australia.
Sarah Mrejen, Hôpital National des Quinze-Vingts, Paris, France; Centre Ophtalmologique d'Imagerie et de Laser, CIL, Paris, France.
Marko M. Popovic, Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California of Los Angeles, David Geffen School of Medicine at UCLA, Los Angeles, California; Department of Ophthalmology and Visual Sciences, University of Toronto, Toronto, Ontario, Canada.
Marisa G. Tieger, Department of Ophthalmology, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts.
Luca Zatreanu, Rochester Regional HealthFollow
SriniVas R. Sadda, Department of Ophthalmology, Doheny Eye Institute, University of California Los Angeles, Los Angeles, California.
K Bailey Freund, Vitreous Retina Macula Consultants of New York, New York, New York; Department of Ophthalmology, NYU Grossman School of Medicine, New York, New York.
Mario R. Romano, Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, 20072. Pieve Emanuele-Milan, Italy; Department of Ophthalmology, Eye Unit Humanitas Gavazzeni-Castelli, via Mazzini 11, Bergamo, Italy.
David Sarraf, Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California of Los Angeles, David Geffen School of Medicine at UCLA, Los Angeles, California; Greater Los Angeles VA Healthcare Center, Los Angeles, California.

Department

Ophthalmology

Document Type

Article

Publication Title

Ophthalmology. Retina

Abstract

PURPOSE: To report the clinical and multimodal imaging (MMI) findings and long-term follow-up of stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) contiguous with midperipheral retinoschisis (MPRS) and to describe a severe SNIFR variant termed CARPET (Central Anomalous Retinoschisis with mid-PEripheral Traction).

DESIGN: Retrospective case series.

SUBJECTS: Eleven patients (15 eyes) with SNIFR contiguous with MPRS in at least 1 eye at baseline or final follow-up.

METHODS: Multimodal imaging features, including cross-sectional and en face macular and peripheral spectral-domain OCT and OCT angiography, were reviewed in all cases at baseline and at the final follow-up visit.

MAIN OUTCOME MEASURES: Various courses (including progression, regression, or stability) of MPRS or SNIFR over time were evaluated.

RESULTS: Midperipheral retinoschisis exhibited centripetal progression to SNIFR in 5 eyes of 3 patients with follow-up of 67, 60, and 27 months, respectively, with maintenance of excellent visual acuity (range: 20/25-20/20) in 4 of these 5 eyes. In 2 eyes of 2 patients (including 1 eye with initial centripetal progression of MPRS to SNIFR), MPRS contiguous with SNIFR spontaneously resolved with long-term follow-up (77 and 25 months, respectively). Stellate nonhereditary idiopathic foveomacular retinoschisis contiguous with MPRS partially regressed after 48 months in 1 patient, and was stable after 54 months in another. A distinctive midperipheral microvasculopathy, associated with MPRS that was contiguous with SNIFR, was identified in 7 eyes of 4 patients. Finally, 3 eyes of 3 patients exhibited additional unique features, including central neurosensory detachment and outer lamellar macular hole, which were associated with significant midperipheral traction, representing a severe variant subtype of SNIFR that we refer to as CARPET. Two of these 3 eyes progressed with short-term follow-up of 6 and 2 months, respectively, whereas the schisis resolved and vision improved after pars plana vitrectomy in the third case.

CONCLUSIONS: Midperipheral retinoschisis can progress to SNIFR over multiple years of follow-up. Stellate nonhereditary idiopathic foveomacular retinoschisis with MPRS can also spontaneously resolve or remain stable. Midperipheral retinoschisis can additionally be complicated by a midperipheral inner retinal microvasculopathy. Finally, CARPET may represent a unique and severe variant form of SNIFR driven by midperipheral vitreoretinal traction and associated with significant vision loss.

FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

First Page

747

Last Page

755

DOI

10.1016/j.oret.2025.01.019

Volume

9

Issue

8

Publication Date

8-1-2025

Medical Subject Headings

Humans; Retinoschisis (diagnosis); Retrospective Studies; Male; Tomography, Optical Coherence (methods); Female; Visual Acuity; Follow-Up Studies; Fluorescein Angiography (methods); Middle Aged; Fovea Centralis (pathology); Aged; Multimodal Imaging (methods); Fundus Oculi; Adult; Disease Progression

PubMed ID

39922381

Recommended Citation

Feo, A., Govetto, A., Ramtohul, P., Abraham, N., Cabral, D., Chang, P. Y., Chaudhry, N., Chen, F. K., Eliott, D., Faes, L., Heath Jeffery, R. C., Mrejen, S., Popovic, M. M., Tieger, M. G., Zatreanu, L., Sadda, S. R., Freund, K. B., Romano, M. R., & Sarraf, D. (2025). Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis and Central Anomalous Retinoschisis with mid-PEripheral Traction. Ophthalmology. Retina, 9 (8), 747-755. https://doi.org/10.1016/j.oret.2025.01.019