Surgical Treatments to Improve Visual Acuity in Infantile Nystagmus Syndrome OTA

Abstract

A report by the American Academy of Ophthalmology Ophthalmic Technology Assessment Committee Pediatric Ophthalmology/Strabismus Panel.

Melinda Y. Chang, MD;¹ Gil Binenbaum, MD, MSCE;² Gena Heidary, MD, PhD;³ Kara M. Cavuoto, MD;⁴ David G. Morrison, MD;⁵ Rupal H. Trivedi, MD, MSCR;⁶ Stephen J. Kim, MD;⁷ Stacy L. Pineles, MD⁸

Ophthalmology, Vol. 130, 331-344, © 2023 by the American Academy of Ophthalmology. Click here for free access to the OTA.

Purpose: To review the literature on the efficacy of surgical procedures to improve visual acuity (VA) in patients with infantile nystagmus syndrome (INS).

Methods: Literature searches were last conducted in January 2022 in the PubMed database for English-language studies with no date restrictions. The combined searches yielded 354 abstracts, of which 46 were reviewed in full text. Twenty-three of these were considered appropriate for inclusion in this assessment and were assigned a level of evidence rating by the panel methodologist.

Results: One included study was a randomized trial; the remaining 22 were case series. The 23 studies included children and adults with INS and a variable proportion with anomalous head position (AHP), strabismus, and sensory diagnoses. The surgical interventions evaluated included large recessions, tenotomy and reattachment (TAR), myectomy with or without pulley fixation, and anterior extirpation of the 4 horizontal rectus muscles, as well as various procedures to correct an AHP in which VA was reported as a secondary outcome. The data were mixed, with improvements in binocular best corrected visual acuity (BCVA) ranging from no improvement to 0.3 logMAR, or 3 lines. (Most studies were in the range of 0.05 to 0.2 logMAR.) Statistically significant improvement in VA was noted in 12 of 16 (75%) studies that performed statistical analyses, with no clear advantage of any single procedure. Complications and reoperations were lowest in patients who underwent TAR and highest in those who underwent myectomy or anterior extirpation.

Conclusions: The best available evidence suggests that eye muscle surgery in patients with INS results in a modest improvement in VA.

¹Children's Hospital of Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, California 
²Division of Ophthalmology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
³Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts 
⁴Bascom Palmer Eye Institute, Department of Ophthalmology, University of Miami Miller School of Medicine, Miami, Florida
⁵Hess Pediatric Ophthalmology, Saint Petersburg, Florida
⁶Storm Eye Institute, Department of Ophthalmology, Medical University of South Carolina, Charleston, South Carolina 
⁷Department of Ophthalmology, Vanderbilt University School of Medicine, Nashville, TN 
⁸Jules Stein Eye Institute, Los Angeles, California