The Pediatric Optic Neuritis Prospective Outcomes Study: Two-Year Results

Pediatric Eye Disease Investigator Group

doi:10.1016/j.ophtha.2022.03.021

The Pediatric Optic Neuritis Prospective Outcomes Study: Two-Year Results

Pediatric Eye Disease Investigator Group

Ophthalmology & Visual Neurosciences

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

5 Scopus citations

Abstract

Purpose: Pediatric optic neuritis (ON) is a rare disease that has not been well characterized. The Pediatric ON Prospective Outcomes Study (PON1) was the first prospective study to our knowledge aiming to evaluate visual acuity (VA) outcomes, including VA, recurrence risk, and final diagnosis 2 years after enrollment. Design: Nonrandomized observational study at 23 pediatric ophthalmology or neuro-ophthalmology clinics in the United States and Canada. Participants: A total of 28 (64%) of 44 children initially enrolled in PON1 (age 3–<16 years) who completed their 2-year study visit. Methods: Participants were treated at the investigator's discretion. Main Outcomes Measures: Age-normal monocular high-contrast VA (HCVA). Secondary outcomes included low-contrast VA (LCVA), neuroimaging findings, and final diagnoses. Results: A total of 28 participants completed the 2-year outcome with a median enrollment age of 10.3 years (range, 5–15); 46% were female, and 68% had unilateral ON at presentation. Final 2-year diagnoses included isolated ON (n = 11, 39%), myelin oligodendrocyte glycoprotein–associated demyelination (n = 8, 29%), multiple sclerosis (MS) (n = 4,14%), neuromyelitis optica spectrum disease (NMOSD) (n = 3, 11%), and acute disseminated encephalomyelitis (n = 2, 7%). Two participants (7%; 95% confidence interval [CI], 1–24) had subsequent recurrent ON (plus 1 participant who did not complete the 2-year visit); all had MS. Two other participants (7%) had a new episode in their unaffected eye. Mean presenting HCVA was 0.81 logarithm of the minimum angle of resolution (logMAR) (∼20/125), improving to 0.14 logMAR (∼20/25^-2) at 6 months, 0.12 logMAR (∼20/25^-2) at 1 year, and 0.11 logMAR (20/25^-1) at 2 years (95% CI, −0.08 to 0.3 [20/20⁺¹–20/40^-1]). Twenty-four participants (79%) had age-normal VA at 2 years (95% CI, 60–90); 21 participants (66%) had 20/20 vision or better. The 6 participants without age-normal VA had 2-year diagnoses of NMOSD (n = 2 participants, 3 eyes), MS (n = 2 participants, 2 eyes), and isolated ON (n = 2 participants, 3 eyes). Mean presenting LCVA was 1.45 logMAR (∼20/500^-2), improving to 0.78 logMAR (∼20/125⁺²) at 6 months, 0.69 logMAR (∼20/100⁺¹) at 1 year, and 0.68 logMAR (∼20/100⁺²) at 2 years (95% CI, 0.48–0.88 [20/50⁺¹–20/150^-1]). Conclusions: Despite poor VA at presentation, most children had marked improvement in VA by 6 months that was maintained over 2 years. Associated neurologic autoimmune diagnoses were common. Additional episodes of ON occurred in 5 (18%) of the participants (3 relapses and 2 new episodes).

Original language	English (US)
Pages (from-to)	856-864
Number of pages	9
Journal	Ophthalmology
Volume	129
Issue number	8
DOIs	https://doi.org/10.1016/j.ophtha.2022.03.021
State	Published - Aug 2022

Bibliographical note

Funding Information:
Research reported in this publication was supported by the National Eye Institute of the National Institutes of Health, under Award Numbers EY011751, EY018810, and EY023198. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. The funding organization had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Funding Information:
Research reported in this publication was supported by the National Eye Institute of the National Institutes of Health , under Award Numbers EY011751, EY018810, and EY023198. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. The funding organization had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Publisher Copyright:
© 2022 American Academy of Ophthalmology

Keywords

Neuro-ophthalmology
Pediatric ophthalmology
Pediatric optic neuritis

PubMed: MeSH publication types

Journal Article
Observational Study
Research Support, N.I.H., Extramural

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@article{7337eb5b0e60429981d754648c76c17c,

title = "The Pediatric Optic Neuritis Prospective Outcomes Study: Two-Year Results",

abstract = "Purpose: Pediatric optic neuritis (ON) is a rare disease that has not been well characterized. The Pediatric ON Prospective Outcomes Study (PON1) was the first prospective study to our knowledge aiming to evaluate visual acuity (VA) outcomes, including VA, recurrence risk, and final diagnosis 2 years after enrollment. Design: Nonrandomized observational study at 23 pediatric ophthalmology or neuro-ophthalmology clinics in the United States and Canada. Participants: A total of 28 (64%) of 44 children initially enrolled in PON1 (age 3–<16 years) who completed their 2-year study visit. Methods: Participants were treated at the investigator's discretion. Main Outcomes Measures: Age-normal monocular high-contrast VA (HCVA). Secondary outcomes included low-contrast VA (LCVA), neuroimaging findings, and final diagnoses. Results: A total of 28 participants completed the 2-year outcome with a median enrollment age of 10.3 years (range, 5–15); 46% were female, and 68% had unilateral ON at presentation. Final 2-year diagnoses included isolated ON (n = 11, 39%), myelin oligodendrocyte glycoprotein–associated demyelination (n = 8, 29%), multiple sclerosis (MS) (n = 4,14%), neuromyelitis optica spectrum disease (NMOSD) (n = 3, 11%), and acute disseminated encephalomyelitis (n = 2, 7%). Two participants (7%; 95% confidence interval [CI], 1–24) had subsequent recurrent ON (plus 1 participant who did not complete the 2-year visit); all had MS. Two other participants (7%) had a new episode in their unaffected eye. Mean presenting HCVA was 0.81 logarithm of the minimum angle of resolution (logMAR) (∼20/125), improving to 0.14 logMAR (∼20/25-2) at 6 months, 0.12 logMAR (∼20/25-2) at 1 year, and 0.11 logMAR (20/25-1) at 2 years (95% CI, −0.08 to 0.3 [20/20+1–20/40-1]). Twenty-four participants (79%) had age-normal VA at 2 years (95% CI, 60–90); 21 participants (66%) had 20/20 vision or better. The 6 participants without age-normal VA had 2-year diagnoses of NMOSD (n = 2 participants, 3 eyes), MS (n = 2 participants, 2 eyes), and isolated ON (n = 2 participants, 3 eyes). Mean presenting LCVA was 1.45 logMAR (∼20/500-2), improving to 0.78 logMAR (∼20/125+2) at 6 months, 0.69 logMAR (∼20/100+1) at 1 year, and 0.68 logMAR (∼20/100+2) at 2 years (95% CI, 0.48–0.88 [20/50+1–20/150-1]). Conclusions: Despite poor VA at presentation, most children had marked improvement in VA by 6 months that was maintained over 2 years. Associated neurologic autoimmune diagnoses were common. Additional episodes of ON occurred in 5 (18%) of the participants (3 relapses and 2 new episodes).",

keywords = "Neuro-ophthalmology, Pediatric ophthalmology, Pediatric optic neuritis",

author = "{Pediatric Eye Disease Investigator Group} and Pineles, {Stacy L.} and Henderson, {Robert J.} and Repka, {Michael X.} and Gena Heidary and Liu, {Grant T.} and Waldman, {Amy T.} and Borchert, {Mark S.} and Sangeeta Khanna and Graves, {Jennifer S.} and Collinge, {Janine E.} and Conley, {Julie A.} and Davis, {Patricia L.} and Kraker, {Raymond T.} and Cotter, {Susan A.} and Holmes, {Jonathan M.} and Chang, {Melinda Y.} and Dilshad Contractor and Zolfaghari, {Emily J.} and Aarti Vyas and Tiffany Yuen and Shah, {Veeral S.} and Paysse, {Evelyn A.} and Gihan Romany and Peragallo, {Jason H.} and Brower, {Judy L.} and Aparna Raghuram and {Al Wattar}, Bilal and Ryan Chinn and Srishti Kothari and Siatkowski, {R. Michael} and Lim, {Maria E.} and Brewer, {Alisha N.} and Doughty, {Annette M.} and Icks, {Sonny W.} and Shannon Almeida and {de Alba Campomanes}, Alejandra and Premilla Banwait and Leila Hajkazemshirazi and Yizhuo Bastea-Forte and Arjona, {Jennifer K.} and Jeremy Chen and Karen Cooper and Rafif Ghadban and Chung, {Sophia M.} and Cruz, {Oscar A.} and Christenson, {Traci A.} and Breeding, {Lisa L.} and McClelland, {Collin M.} and Areaux, {Raymond G.} and Holleschau, {Ann M.}",

note = "Funding Information: Research reported in this publication was supported by the National Eye Institute of the National Institutes of Health, under Award Numbers EY011751, EY018810, and EY023198. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. The funding organization had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. Funding Information: Research reported in this publication was supported by the National Eye Institute of the National Institutes of Health , under Award Numbers EY011751, EY018810, and EY023198. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. The funding organization had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. Publisher Copyright: {\textcopyright} 2022 American Academy of Ophthalmology",

year = "2022",

month = aug,

doi = "10.1016/j.ophtha.2022.03.021",

language = "English (US)",

volume = "129",

pages = "856--864",

journal = "Ophthalmology",

issn = "0161-6420",

publisher = "Elsevier Inc.",

number = "8",

}

TY - JOUR

T1 - The Pediatric Optic Neuritis Prospective Outcomes Study

T2 - Two-Year Results

AU - Pediatric Eye Disease Investigator Group

AU - Pineles, Stacy L.

AU - Henderson, Robert J.

AU - Repka, Michael X.

AU - Heidary, Gena

AU - Liu, Grant T.

AU - Waldman, Amy T.

AU - Borchert, Mark S.

AU - Khanna, Sangeeta

AU - Graves, Jennifer S.

AU - Collinge, Janine E.

AU - Conley, Julie A.

AU - Davis, Patricia L.

AU - Kraker, Raymond T.

AU - Cotter, Susan A.

AU - Holmes, Jonathan M.

AU - Chang, Melinda Y.

AU - Contractor, Dilshad

AU - Zolfaghari, Emily J.

AU - Vyas, Aarti

AU - Yuen, Tiffany

AU - Shah, Veeral S.

AU - Paysse, Evelyn A.

AU - Romany, Gihan

AU - Peragallo, Jason H.

AU - Brower, Judy L.

AU - Raghuram, Aparna

AU - Al Wattar, Bilal

AU - Chinn, Ryan

AU - Kothari, Srishti

AU - Siatkowski, R. Michael

AU - Lim, Maria E.

AU - Brewer, Alisha N.

AU - Doughty, Annette M.

AU - Icks, Sonny W.

AU - Almeida, Shannon

AU - de Alba Campomanes, Alejandra

AU - Banwait, Premilla

AU - Hajkazemshirazi, Leila

AU - Bastea-Forte, Yizhuo

AU - Arjona, Jennifer K.

AU - Chen, Jeremy

AU - Cooper, Karen

AU - Ghadban, Rafif

AU - Chung, Sophia M.

AU - Cruz, Oscar A.

AU - Christenson, Traci A.

AU - Breeding, Lisa L.

AU - McClelland, Collin M.

AU - Areaux, Raymond G.

AU - Holleschau, Ann M.

N1 - Funding Information: Research reported in this publication was supported by the National Eye Institute of the National Institutes of Health, under Award Numbers EY011751, EY018810, and EY023198. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. The funding organization had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. Funding Information: Research reported in this publication was supported by the National Eye Institute of the National Institutes of Health , under Award Numbers EY011751, EY018810, and EY023198. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. The funding organization had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. Publisher Copyright: © 2022 American Academy of Ophthalmology

PY - 2022/8

Y1 - 2022/8

N2 - Purpose: Pediatric optic neuritis (ON) is a rare disease that has not been well characterized. The Pediatric ON Prospective Outcomes Study (PON1) was the first prospective study to our knowledge aiming to evaluate visual acuity (VA) outcomes, including VA, recurrence risk, and final diagnosis 2 years after enrollment. Design: Nonrandomized observational study at 23 pediatric ophthalmology or neuro-ophthalmology clinics in the United States and Canada. Participants: A total of 28 (64%) of 44 children initially enrolled in PON1 (age 3–<16 years) who completed their 2-year study visit. Methods: Participants were treated at the investigator's discretion. Main Outcomes Measures: Age-normal monocular high-contrast VA (HCVA). Secondary outcomes included low-contrast VA (LCVA), neuroimaging findings, and final diagnoses. Results: A total of 28 participants completed the 2-year outcome with a median enrollment age of 10.3 years (range, 5–15); 46% were female, and 68% had unilateral ON at presentation. Final 2-year diagnoses included isolated ON (n = 11, 39%), myelin oligodendrocyte glycoprotein–associated demyelination (n = 8, 29%), multiple sclerosis (MS) (n = 4,14%), neuromyelitis optica spectrum disease (NMOSD) (n = 3, 11%), and acute disseminated encephalomyelitis (n = 2, 7%). Two participants (7%; 95% confidence interval [CI], 1–24) had subsequent recurrent ON (plus 1 participant who did not complete the 2-year visit); all had MS. Two other participants (7%) had a new episode in their unaffected eye. Mean presenting HCVA was 0.81 logarithm of the minimum angle of resolution (logMAR) (∼20/125), improving to 0.14 logMAR (∼20/25-2) at 6 months, 0.12 logMAR (∼20/25-2) at 1 year, and 0.11 logMAR (20/25-1) at 2 years (95% CI, −0.08 to 0.3 [20/20+1–20/40-1]). Twenty-four participants (79%) had age-normal VA at 2 years (95% CI, 60–90); 21 participants (66%) had 20/20 vision or better. The 6 participants without age-normal VA had 2-year diagnoses of NMOSD (n = 2 participants, 3 eyes), MS (n = 2 participants, 2 eyes), and isolated ON (n = 2 participants, 3 eyes). Mean presenting LCVA was 1.45 logMAR (∼20/500-2), improving to 0.78 logMAR (∼20/125+2) at 6 months, 0.69 logMAR (∼20/100+1) at 1 year, and 0.68 logMAR (∼20/100+2) at 2 years (95% CI, 0.48–0.88 [20/50+1–20/150-1]). Conclusions: Despite poor VA at presentation, most children had marked improvement in VA by 6 months that was maintained over 2 years. Associated neurologic autoimmune diagnoses were common. Additional episodes of ON occurred in 5 (18%) of the participants (3 relapses and 2 new episodes).

AB - Purpose: Pediatric optic neuritis (ON) is a rare disease that has not been well characterized. The Pediatric ON Prospective Outcomes Study (PON1) was the first prospective study to our knowledge aiming to evaluate visual acuity (VA) outcomes, including VA, recurrence risk, and final diagnosis 2 years after enrollment. Design: Nonrandomized observational study at 23 pediatric ophthalmology or neuro-ophthalmology clinics in the United States and Canada. Participants: A total of 28 (64%) of 44 children initially enrolled in PON1 (age 3–<16 years) who completed their 2-year study visit. Methods: Participants were treated at the investigator's discretion. Main Outcomes Measures: Age-normal monocular high-contrast VA (HCVA). Secondary outcomes included low-contrast VA (LCVA), neuroimaging findings, and final diagnoses. Results: A total of 28 participants completed the 2-year outcome with a median enrollment age of 10.3 years (range, 5–15); 46% were female, and 68% had unilateral ON at presentation. Final 2-year diagnoses included isolated ON (n = 11, 39%), myelin oligodendrocyte glycoprotein–associated demyelination (n = 8, 29%), multiple sclerosis (MS) (n = 4,14%), neuromyelitis optica spectrum disease (NMOSD) (n = 3, 11%), and acute disseminated encephalomyelitis (n = 2, 7%). Two participants (7%; 95% confidence interval [CI], 1–24) had subsequent recurrent ON (plus 1 participant who did not complete the 2-year visit); all had MS. Two other participants (7%) had a new episode in their unaffected eye. Mean presenting HCVA was 0.81 logarithm of the minimum angle of resolution (logMAR) (∼20/125), improving to 0.14 logMAR (∼20/25-2) at 6 months, 0.12 logMAR (∼20/25-2) at 1 year, and 0.11 logMAR (20/25-1) at 2 years (95% CI, −0.08 to 0.3 [20/20+1–20/40-1]). Twenty-four participants (79%) had age-normal VA at 2 years (95% CI, 60–90); 21 participants (66%) had 20/20 vision or better. The 6 participants without age-normal VA had 2-year diagnoses of NMOSD (n = 2 participants, 3 eyes), MS (n = 2 participants, 2 eyes), and isolated ON (n = 2 participants, 3 eyes). Mean presenting LCVA was 1.45 logMAR (∼20/500-2), improving to 0.78 logMAR (∼20/125+2) at 6 months, 0.69 logMAR (∼20/100+1) at 1 year, and 0.68 logMAR (∼20/100+2) at 2 years (95% CI, 0.48–0.88 [20/50+1–20/150-1]). Conclusions: Despite poor VA at presentation, most children had marked improvement in VA by 6 months that was maintained over 2 years. Associated neurologic autoimmune diagnoses were common. Additional episodes of ON occurred in 5 (18%) of the participants (3 relapses and 2 new episodes).

KW - Neuro-ophthalmology

KW - Pediatric ophthalmology

KW - Pediatric optic neuritis

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DO - 10.1016/j.ophtha.2022.03.021

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SN - 0161-6420

VL - 129

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JO - Ophthalmology

JF - Ophthalmology

IS - 8

ER -

The Pediatric Optic Neuritis Prospective Outcomes Study: Two-Year Results

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