Peripapillary retinoschisis: the expanded spectrum and new insights from multimodal imaging

Lucy Yi Yang; Andrew W. Kam; Fred K. Chen; Rachael C. Heath Jeffrey; Andrew Farag; Ananda Kalevar; J. A. Y. Chhablani; Marco Lupidi; Michael Chilov; Michael Branley; Jenny Ip; David Kalatzis; Shanil Dhanji; Devin Bestch; R. Rishi Gupta; Netan Choudhry; Diogo Cabral; Caroline R. Baumal; K. Bailey Freund; Adrian T. Fung

doi:10.1016/j.ajo.2025.10.017

Peripapillary retinoschisis: the expanded spectrum and new insights from multimodal imaging

Lucy Yi Yang, Andrew W. Kam, Fred K. Chen, Rachael C. Heath Jeffrey, Andrew Farag, Ananda Kalevar, J. A. Y. Chhablani, Marco Lupidi, Michael Chilov, Michael Branley, Jenny Ip, David Kalatzis, Shanil Dhanji, Devin Bestch, R. Rishi Gupta, Netan Choudhry, Diogo Cabral, Caroline R. Baumal, K. Bailey Freund, Adrian T. Fung^*

^*Corresponding author for this work

Macquarie Medical School

Research output: Contribution to journal › Article › peer-review

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Abstract

Purpose: To characterize and classify different forms of peripapillary retinoschisis (PPRS), and to clarify the nomenclature of this condition. Methods: A retrospective, multicenter, multinational case series of PPRS was performed from August 2021 to September 2024. Cases were included if they demonstrated retinoschisis contiguous with and thought to be originating from the optic disc. Demographic and clinical data collected included age, gender, visual acuity, intraocular pressure, axial length, refraction and referring symptoms. Mandatory investigations included optical coherence tomography of the optic disc and macula with radial scans, color fundus photography, and fundus autofluorescence. Select cases underwent fundus fluorescein and/or indocyanine green angiography. Retinoschisis was characterized by the meridian in relation to the optic disc, layer(s) of the retina affected and associated conditions. A literature review was performed to identify all causes of PPRS. Results: A total of 47 eyes from 41 patients with PPRS were identified, comprising of 22 (54%) females and a mean age of 56 years (range 14-92 years). These were classified into 9 aetiologies: Congenital Disc Abnormalities (CDA, n = 16 eyes), peripapillary chorioretinal coloboma (n = 1), peripapillary atrophy (n = 3), glaucoma (n = 7), Peripapillary Pachychoroid Syndrome (PPS, n = 4), peripapillary choroidal neovascularization (PP-CNV, n = 3), high/ pathological myopia (n = 5), vitreopapillary traction (VPT, n = 4) and idiopathic (n = 4). Conclusion: The 9 aetiologies of peripapillary retinoschisis can be classified into 5 groups: non-glaucomatous optic disc abnormalities (CDA, PP-coloboma, PP-atrophy), glaucomatous optic disc abnormalities, peripapillary choroidal diseases (PPS and PP-CNV), vitreous optic disc interface abnormalities and idiopathic. A new entity, “Focal Optic Disc Dome” (FODD) was identified. Understanding the full spectrum of PPRS can assist in correct diagnosis and management.

Original language	English
Pages (from-to)	26-40
Number of pages	15
Journal	American Journal of Ophthalmology
Volume	282
Early online date	17 Oct 2025
DOIs	https://doi.org/10.1016/j.ajo.2025.10.017
Publication status	Published - Feb 2026

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Copyright the Author(s) 2025. Version archived for private and non-commercial use with the permission of the author/s and according to publisher conditions. For further rights please contact the publisher.

A corrigendum exists for this article and can be found at doi: 10.1016/j.ajo.2026.04.012

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Yang, L. Y., Kam, A. W., Chen, F. K., Jeffrey, R. C. H., Farag, A., Kalevar, A., Chhablani, J. A. Y., Lupidi, M., Chilov, M., Branley, M., Ip, J., Kalatzis, D., Dhanji, S., Bestch, D., Gupta, R. R., Choudhry, N., Cabral, D., Baumal, C. R., Freund, K. B., & Fung, A. T. (2026). Peripapillary retinoschisis: the expanded spectrum and new insights from multimodal imaging. American Journal of Ophthalmology, 282, 26-40. https://doi.org/10.1016/j.ajo.2025.10.017

@article{328f27e40b0440d6942d3e0985a5b75c,

title = "Peripapillary retinoschisis: the expanded spectrum and new insights from multimodal imaging",

abstract = "Purpose: To characterize and classify different forms of peripapillary retinoschisis (PPRS), and to clarify the nomenclature of this condition. Methods: A retrospective, multicenter, multinational case series of PPRS was performed from August 2021 to September 2024. Cases were included if they demonstrated retinoschisis contiguous with and thought to be originating from the optic disc. Demographic and clinical data collected included age, gender, visual acuity, intraocular pressure, axial length, refraction and referring symptoms. Mandatory investigations included optical coherence tomography of the optic disc and macula with radial scans, color fundus photography, and fundus autofluorescence. Select cases underwent fundus fluorescein and/or indocyanine green angiography. Retinoschisis was characterized by the meridian in relation to the optic disc, layer(s) of the retina affected and associated conditions. A literature review was performed to identify all causes of PPRS. Results: A total of 47 eyes from 41 patients with PPRS were identified, comprising of 22 (54\%) females and a mean age of 56 years (range 14-92 years). These were classified into 9 aetiologies: Congenital Disc Abnormalities (CDA, n = 16 eyes), peripapillary chorioretinal coloboma (n = 1), peripapillary atrophy (n = 3), glaucoma (n = 7), Peripapillary Pachychoroid Syndrome (PPS, n = 4), peripapillary choroidal neovascularization (PP-CNV, n = 3), high/ pathological myopia (n = 5), vitreopapillary traction (VPT, n = 4) and idiopathic (n = 4). Conclusion: The 9 aetiologies of peripapillary retinoschisis can be classified into 5 groups: non-glaucomatous optic disc abnormalities (CDA, PP-coloboma, PP-atrophy), glaucomatous optic disc abnormalities, peripapillary choroidal diseases (PPS and PP-CNV), vitreous optic disc interface abnormalities and idiopathic. A new entity, “Focal Optic Disc Dome” (FODD) was identified. Understanding the full spectrum of PPRS can assist in correct diagnosis and management.",

author = "Yang, \{Lucy Yi\} and Kam, \{Andrew W.\} and Chen, \{Fred K.\} and Jeffrey, \{Rachael C. Heath\} and Andrew Farag and Ananda Kalevar and Chhablani, \{J. A. Y.\} and Marco Lupidi and Michael Chilov and Michael Branley and Jenny Ip and David Kalatzis and Shanil Dhanji and Devin Bestch and Gupta, \{R. Rishi\} and Netan Choudhry and Diogo Cabral and Baumal, \{Caroline R.\} and Freund, \{K. Bailey\} and Fung, \{Adrian T.\}",

note = "Copyright the Author(s) 2025. Version archived for private and non-commercial use with the permission of the author/s and according to publisher conditions. For further rights please contact the publisher. A corrigendum exists for this article and can be found at doi: 10.1016/j.ajo.2026.04.012",

year = "2026",

month = feb,

doi = "10.1016/j.ajo.2025.10.017",

language = "English",

volume = "282",

pages = "26--40",

journal = "American Journal of Ophthalmology",

issn = "0002-9394",

publisher = "Elsevier",

}

Yang, LY, Kam, AW, Chen, FK, Jeffrey, RCH, Farag, A, Kalevar, A, Chhablani, JAY, Lupidi, M, Chilov, M, Branley, M, Ip, J, Kalatzis, D, Dhanji, S, Bestch, D, Gupta, RR, Choudhry, N, Cabral, D, Baumal, CR, Freund, KB & Fung, AT 2026, 'Peripapillary retinoschisis: the expanded spectrum and new insights from multimodal imaging', American Journal of Ophthalmology, vol. 282, pp. 26-40. https://doi.org/10.1016/j.ajo.2025.10.017

TY - JOUR

T1 - Peripapillary retinoschisis

T2 - the expanded spectrum and new insights from multimodal imaging

AU - Yang, Lucy Yi

AU - Kam, Andrew W.

AU - Chen, Fred K.

AU - Jeffrey, Rachael C. Heath

AU - Farag, Andrew

AU - Kalevar, Ananda

AU - Chhablani, J. A. Y.

AU - Lupidi, Marco

AU - Chilov, Michael

AU - Branley, Michael

AU - Ip, Jenny

AU - Kalatzis, David

AU - Dhanji, Shanil

AU - Bestch, Devin

AU - Gupta, R. Rishi

AU - Choudhry, Netan

AU - Cabral, Diogo

AU - Baumal, Caroline R.

AU - Freund, K. Bailey

AU - Fung, Adrian T.

N1 - Copyright the Author(s) 2025. Version archived for private and non-commercial use with the permission of the author/s and according to publisher conditions. For further rights please contact the publisher. A corrigendum exists for this article and can be found at doi: 10.1016/j.ajo.2026.04.012

PY - 2026/2

Y1 - 2026/2

N2 - Purpose: To characterize and classify different forms of peripapillary retinoschisis (PPRS), and to clarify the nomenclature of this condition. Methods: A retrospective, multicenter, multinational case series of PPRS was performed from August 2021 to September 2024. Cases were included if they demonstrated retinoschisis contiguous with and thought to be originating from the optic disc. Demographic and clinical data collected included age, gender, visual acuity, intraocular pressure, axial length, refraction and referring symptoms. Mandatory investigations included optical coherence tomography of the optic disc and macula with radial scans, color fundus photography, and fundus autofluorescence. Select cases underwent fundus fluorescein and/or indocyanine green angiography. Retinoschisis was characterized by the meridian in relation to the optic disc, layer(s) of the retina affected and associated conditions. A literature review was performed to identify all causes of PPRS. Results: A total of 47 eyes from 41 patients with PPRS were identified, comprising of 22 (54%) females and a mean age of 56 years (range 14-92 years). These were classified into 9 aetiologies: Congenital Disc Abnormalities (CDA, n = 16 eyes), peripapillary chorioretinal coloboma (n = 1), peripapillary atrophy (n = 3), glaucoma (n = 7), Peripapillary Pachychoroid Syndrome (PPS, n = 4), peripapillary choroidal neovascularization (PP-CNV, n = 3), high/ pathological myopia (n = 5), vitreopapillary traction (VPT, n = 4) and idiopathic (n = 4). Conclusion: The 9 aetiologies of peripapillary retinoschisis can be classified into 5 groups: non-glaucomatous optic disc abnormalities (CDA, PP-coloboma, PP-atrophy), glaucomatous optic disc abnormalities, peripapillary choroidal diseases (PPS and PP-CNV), vitreous optic disc interface abnormalities and idiopathic. A new entity, “Focal Optic Disc Dome” (FODD) was identified. Understanding the full spectrum of PPRS can assist in correct diagnosis and management.

AB - Purpose: To characterize and classify different forms of peripapillary retinoschisis (PPRS), and to clarify the nomenclature of this condition. Methods: A retrospective, multicenter, multinational case series of PPRS was performed from August 2021 to September 2024. Cases were included if they demonstrated retinoschisis contiguous with and thought to be originating from the optic disc. Demographic and clinical data collected included age, gender, visual acuity, intraocular pressure, axial length, refraction and referring symptoms. Mandatory investigations included optical coherence tomography of the optic disc and macula with radial scans, color fundus photography, and fundus autofluorescence. Select cases underwent fundus fluorescein and/or indocyanine green angiography. Retinoschisis was characterized by the meridian in relation to the optic disc, layer(s) of the retina affected and associated conditions. A literature review was performed to identify all causes of PPRS. Results: A total of 47 eyes from 41 patients with PPRS were identified, comprising of 22 (54%) females and a mean age of 56 years (range 14-92 years). These were classified into 9 aetiologies: Congenital Disc Abnormalities (CDA, n = 16 eyes), peripapillary chorioretinal coloboma (n = 1), peripapillary atrophy (n = 3), glaucoma (n = 7), Peripapillary Pachychoroid Syndrome (PPS, n = 4), peripapillary choroidal neovascularization (PP-CNV, n = 3), high/ pathological myopia (n = 5), vitreopapillary traction (VPT, n = 4) and idiopathic (n = 4). Conclusion: The 9 aetiologies of peripapillary retinoschisis can be classified into 5 groups: non-glaucomatous optic disc abnormalities (CDA, PP-coloboma, PP-atrophy), glaucomatous optic disc abnormalities, peripapillary choroidal diseases (PPS and PP-CNV), vitreous optic disc interface abnormalities and idiopathic. A new entity, “Focal Optic Disc Dome” (FODD) was identified. Understanding the full spectrum of PPRS can assist in correct diagnosis and management.

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Peripapillary retinoschisis: the expanded spectrum and new insights from multimodal imaging

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