@article{1242a01c267a4bc1ba4a8358a3be5012,
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 Govreau, {Dawn M.} and Wallis, {Beth A.} and Geddie, {Brooke E.} and Wolinski, {Elisabeth T.} and Rudaitis, {Indre M.} and Jacqueline Twite and Bloomquist, {Carrie S.} and Laboy, {Sarah R.} and Twite, {Jackie M.} and Doan, {Michelle V.} and Bernardo, {Marianne J.} and Brodsky, {Michael C.} and Chen, {John J.} and Wernimont, {Suzanne M.} and Czaplewski, {Lindsay L.} and Eastman, {Stacy L.} and Keehn, {Moriah A.} and Priebe, {Debbie M.} and Bremer, {Don L.} and Golden, {Richard P.} and Jordan, {Catherine O.} and McGregor, {Mary Lou} and Reem, {Rachel E.} and Rogers, {David L.} and Schreckengost, {Amanda N.} and Maletic, {Sara A.} and Dairi, {Mays A.} and Enyedi, {Laura B.} and Jones, {Sarah K.} and Barman, {Navajyoti R.} and House, {Robert J.} and Nasrazadani, {David A.} and Gratton, {Sean M.} and Marsh, {Justin D.} and Dent, {Rebecca J.} and Bond, {Lezlie L.} and Soske, {Lori L.} and Padmaja Sudhakar and Willen, {Christi M.} and Deborah Taylor and Moliterno, {Nathaniel Q.} and Michael Nsoesie and Shaista Vally and Phillips, {Paul H.} and Lowery, {Robert S.} and Beth Colon and Stotts, {Nancy L.} and To, {Kelly D.} and McClelland, {Collin M.} and Areaux, {Raymond G.} and Holleschau, {Ann M.} and Merrill, {Kim S.} and Ospina, {Luis H.} and Rosanne Superstein and Maryse Thibeault and Helene Gagnon and Donahue, {Sean P.} and Ruark, {Scott T.} and Fraine, {Lisa A.} and Sprouse, {Petrice A.} and Biernacki, {Ronald J.} and Avery, {Robert A.} and Forbes, {Brian J.} and Imran Jivraj and Kohli, {Anita A.} and Richter, {Meg M.} and Agnieshka Baumritter and Mitchell, {Ellen B.} and Nischal, {Ken K.} and Runkel, {Lauren M.} and Bianca Blaha and Whitney Churchfield and Christina Fulwylie and Ko, {Melissa W.} and Mejico, {Luis J.} and Muhammad Iqbal and Attanasio, {Catherine E.} and Deb, {Lena F.} and Goodrich, {Courtney B.} and Hartwell, {Alisha M.} and Moore, {Jennifer A.} and Lisa Bohra and Apkarian, {Alexandra O.} and Gianfermi, {Elena M.} and Roarty, {John D.} and Rotberg, {Leemor B.} and Perzyk, {Susan N.} and Beck, {Roy W.} and Austin, {Darrell S.} and Boyle, {Nicole M.} and Chandler, {Danielle L.} and Connelly, {Patricia L.} and Conner, {Courtney L.} and Dean, {Trevano W.} and Quayleen Donahue and Fimbel, {Brooke P.} and Amra Hercinovic and Hoepner, {James E.} and Kaplon, {Joseph D.} and Zhuokai Li and Gillaine Ortiz and Robinson, {Julianne L.} and Stutz, {Kathleen M.} and Toro, {David O.} and Woodard, {Victoria C.} and Rui Wu and Laura Balcer and Mark Kupersmith and Lazar, {Elizabeth L.} and Amy Waldman and Wallace, {David K.} and Birch, {Eileen E.} and Chen, {Angela M.} and Christiansen, {Stephen P.} and Erzurum, {S. Ayse} and Everett, {Donald F.} and Freedman, {Sharon F.} and Good, {William V.} and Lee, {Katherine A.} and Richard London and Manh, {Vivian M.} and Manny, {Ruth E.} and Morrison, {David G.} and Schweinler, {Bonita R.} and Silver, {Jayne L.} and Verderber, {Lisa C.} and Weise, {Katherine K.} and Ari Green and Marie Diener-West and Baker, {John D.} and Barry Davis and Phelps, {Dale L.} and Poff, {Stephen W.} and Saunders, {Richard A.} and Lawrence Tychsen",
note = "Publisher Copyright: {\textcopyright} 2022 American Academy of Ophthalmology",
year = "2022",
month = aug,
doi = "10.1016/j.ophtha.2022.03.021",
language = "English",
volume = "129",
pages = "856--864",
journal = "Ophthalmology",
issn = "0161-6420",
number = "8",
}