Prescribing patterns for paediatric hyperopia among paediatric eye care providers.

PURPOSE: To survey paediatric eye care providers to identify current patterns of prescribing for hyperopia. METHODS: Paediatric eye care providers were invited, via email, to participate in a survey to evaluate current age-based refractive error prescribing practices. Questions were designed to determine which factors may influence the survey participant's prescribing pattern (e.g., patient's age, magnitude of hyperopia, patient's symptoms, heterophoria and stereopsis) and if the providers were to prescribe, how much hyperopic correction would they prescribe (e.g., full or partial prescription). The response distributions by profession (optometry and ophthalmology) were compared using the Kolmogorov-Smirnov cumulative distribution function test. RESULTS: Responses were submitted by 738 participants regarding how they prescribe for their hyperopic patients. Most providers within each profession considered similar clinical factors when prescribing. The percentages of optometrists and ophthalmologists who reported considering the factor often differed significantly. Factors considered similarly by both optometrists and ophthalmologists were the presence of symptoms (98.0%, p = 0.14), presence of astigmatism and/or anisometropia (97.5%, p = 0.06) and the possibility of teasing (8.3%, p = 0.49). A wide range of prescribing was observed within each profession, with some providers reporting that they would prescribe for low levels of hyperopia while others reported that they would never prescribe. When prescribing for bilateral hyperopia in children with age-normal visual acuity and no manifest deviation or symptoms, the threshold for prescribing decreased with age for both professions, with ophthalmologists typically prescribing 1.5-2 D less than optometrists. The threshold for prescribing also decreased for both optometrists and ophthalmologists when children had associated clinical factors (e.g., esophoria or reduced near visual function). Optometrists and ophthalmologists most commonly prescribed based on cycloplegic refraction, although optometrists most commonly prescribed based on both the manifest and cycloplegic refraction for children ≥7 years. CONCLUSION: Prescribing patterns for paediatric hyperopia vary significantly among eye care providers.

Genotypic and Phenotypic Spectrum of Foveal Hypoplasia: A Multicenter Study.

PURPOSE: To characterize the genotypic and phenotypic spectrum of foveal hypoplasia (FH). DESIGN: Multicenter, observational study. PARTICIPANTS: A total of 907 patients with a confirmed molecular diagnosis of albinism, PAX6, SLC38A8, FRMD7, AHR, or achromatopsia from 12 centers in 9 countries (n = 523) or extracted from publicly available datasets from previously reported literature (n = 384). METHODS: Individuals with a confirmed molecular diagnosis and availability of foveal OCT scans were identified from 12 centers or from the literature between January 2011 and March 2021. A genetic diagnosis was confirmed by sequence analysis. Grading of FH was derived from OCT scans. MAIN OUTCOME MEASURES: Grade of FH, presence or absence of photoreceptor specialization (PRS+ vs. PRS-), molecular diagnosis, and visual acuity (VA). RESULTS: The most common genetic etiology for typical FH in our cohort was albinism (67.5%), followed by PAX6 (21.8%), SLC38A8 (6.8%), and FRMD7 (3.5%) variants. AHR variants were rare (0.4%). Atypical FH was seen in 67.4% of achromatopsia cases. Atypical FH in achromatopsia had significantly worse VA than typical FH (P < 0.0001). There was a significant difference in the spectrum of FH grades based on the molecular diagnosis (chi-square = 60.4, P < 0.0001). All SLC38A8 cases were PRS- (P = 0.003), whereas all FRMD7 cases were PRS+ (P < 0.0001). Analysis of albinism subtypes revealed a significant difference in the grade of FH (chi-square = 31.4, P < 0.0001) and VA (P = 0.0003) between oculocutaneous albinism (OCA) compared with ocular albinism (OA) and Hermansky-Pudlak syndrome (HPS). Ocular albinism and HPS demonstrated higher grades of FH and worse VA than OCA. There was a significant difference (P < 0.0001) in VA between FRMD7 variants compared with other diagnoses associated with FH. CONCLUSIONS: We characterized the phenotypic and genotypic spectrum of FH. Atypical FH is associated with a worse prognosis than all other forms of FH. In typical FH, our data suggest that arrested retinal development occurs earlier in SLC38A8, OA, HPS, and AHR variants and later in FRMD7 variants. The defined time period of foveal developmental arrest for OCA and PAX6 variants seems to demonstrate more variability. Our findings provide mechanistic insight into disorders associated with FH and have significant prognostic and diagnostic value.

Effectiveness of vergence/accommodative therapy for accommodative dysfunction in children with convergence insufficiency.

PURPOSE: To determine the effectiveness of office-based vergence/accommodative therapy for improving accommodative amplitude and accommodative facility in children with symptomatic convergence insufficiency and accommodative dysfunction. METHODS: We report changes in accommodative function following therapy among participants in the Convergence Insufficiency Treatment Trial - Attention and Reading Trial with decreased accommodative amplitude (115 participants in vergence/accommodative therapy; 65 in placebo therapy) or decreased accommodative facility (71 participants in vergence/accommodative therapy; 37 in placebo therapy) at baseline. The primary analysis compared mean change in amplitude and facility between the vergence/accommodative and placebo therapy groups using analyses of variance models after 4, 8, 12 and 16 weeks of treatment. The proportions of participants with normal amplitude and facility at each time point were calculated. The average rate of change in amplitude and facility from baseline to week 4, and from weeks 4 to 16, were determined in the vergence/accommodative therapy group. RESULTS: From baseline to 16 weeks, the mean improvement in amplitude was 8.6 dioptres (D) and 5.2 D in the vergence/accommodative and placebo therapy groups, respectively (mean difference = 3.5 D, 95% confidence interval (CI): 1.5 to 5.5 D; p = 0.01). The mean improvement in facility was 13.5 cycles per minute (cpm) and 7.6 cpm in the vergence/accommodative and placebo therapy groups, respectively (mean difference = 5.8 cpm, 95% CI: 3.8 to 7.9 cpm; p < 0.0001). Significantly greater proportions of participants treated with vergence/accommodative therapy achieved a normal amplitude (69% vs. 32%, difference = 37%, 95% CI: 22 to 51%; p < 0.0001) and facility (85% vs. 49%, difference = 36%, 95% CI: 18 to 55%; p < 0.0001) than those who received placebo therapy. In the vergence/accommodative therapy group, amplitude increased at an average rate of 1.5 D per week during the first 4 weeks (p < 0.0001), then slowed to 0.2 D per week (p = 0.002) from weeks 4 to 16. Similarly, facility increased at an average rate of 1.5 cpm per week during the first 4 weeks (p < 0.0001), then slowed to 0.6 cpm per week from weeks 4 to 16 (p < 0.0001). CONCLUSION: Office-based vergence/accommodative therapy is effective for improving accommodative function in children with symptomatic convergence insufficiency and coexisting accommodative dysfunction.

Effect of artificial scotomas on open-loop disparity vergence eye movements.

PURPOSE: To investigate the effect of an artificial scotoma on open-loop disparity vergence responses (DVRs) and vergence control mechanisms, we examined open-loop DVRs to disparity stimuli using monocular artificial scotomas in normal subjects. METHODS: Using a mirror haploscope with two computer monitors, we delivered disparity stimuli on a pair of random dot patterns subtending 40 by 30 degrees at 47 cm from each eye. The scotomas were black circles located in the center of a random dot pattern for the left eye. Eye movements of both eyes were recorded with a magnetic search coil system. RESULTS: We first found that the amplitudes of DVRs were gradually decreased and the latency of DVRs was moderately increased as the size of the scotomas was increased. Second, monocular responses from each eye were symmetrical although the stimuli to each eye were asymmetrical. CONCLUSIONS: The results suggest that the monocular eye movements in disparity vergence are controlled by a binocular central mechanism, not driven separately by monocular inputs in the open-loop window.

Mutations in FRMD7, a newly identified member of the FERM family, cause X-linked idiopathic congenital nystagmus.

Idiopathic congenital nystagmus is characterized by involuntary, periodic, predominantly horizontal oscillations of both eyes. We identified 22 mutations in FRMD7 in 26 families with X-linked idiopathic congenital nystagmus. Screening of 42 singleton cases of idiopathic congenital nystagmus (28 male, 14 females) yielded three mutations (7%). We found restricted expression of FRMD7 in human embryonic brain and developing neural retina, suggesting a specific role in the control of eye movement and gaze stability.

An interesting case of bilateral bifid insertion of superior rectus muscle as an intra-operative finding in a patient with oculocutaneous albinism.

We report a case of bilateral bifid insertion of superior rectus muscles, in a patient with oculocutaneous albinism as an incidental intraoperative finding during eye muscle surgery. The muscle was successfully operated on and the patient did well postoperatively. To our knowledge, this is the first report of this anomaly.

Demographic and Clinical Characteristics of 600 Children With Nystagmus.

PURPOSE: To characterize a large, international cohort of children with nystagmus. METHODS: Data were collected from a single-center, prospectively developed database on patients with nystagmus. Outcome variables for this study included: (1) demographic characteristics, (2) nystagmus type, (3) clinical characteristics, (4) associated ophthalmic conditions, (5) associated non-ophthalmic conditions, (6) special testing findings, and (7) treatments. RESULTS: Six hundred patients from 38 states and 30 countries were included. Age ranged from birth to younger than 18 years (mean: 15.2 years), 58% were female, 55% were race other than White, 75% had infantile nystagmus syndrome, 17% had neurologically significant nystagmus, 7% had fusion maldevelopment nystagmus syndrome, 64% had strabismus, 56% had an anomalous head posture, 94% had a significant refractive error, 64% had an associated ophthalmic abnormality (excluding ametropia), and 45% had an associated systemic condition. Special testing showed abnormalities in 67% and 95% had treatment directed at their nystagmus. CONCLUSIONS: The most common diagnosis in this cohort is neurologically non-significant infantile nystagmus syndrome. Eye movement recordings provide a path toward accurate diagnosis and classification. There was a high prevalence of underlying ocular and/or systemic conditions requiring ophthalmic special testing as part of a diagnostic routine. Clinical treatments are available to most patients. [J Pediatr Ophthalmol Strabismus. 2023;60(6):406-410.].

The clinical and molecular genetic features of idiopathic infantile periodic alternating nystagmus.

Periodic alternating nystagmus consists of involuntary oscillations of the eyes with cyclical changes of nystagmus direction. It can occur during infancy (e.g. idiopathic infantile periodic alternating nystagmus) or later in life. Acquired forms are often associated with cerebellar dysfunction arising due to instability of the optokinetic-vestibular systems. Idiopathic infantile periodic alternating nystagmus can be familial or occur in isolation; however, very little is known about the clinical characteristics, genetic aetiology and neural substrates involved. Five loci (NYS1-5) have been identified for idiopathic infantile nystagmus; three are autosomal (NYS2, NYS3 and NYS4) and two are X-chromosomal (NYS1 and NYS5). We previously identified the FRMD7 gene on chromosome Xq26 (NYS1 locus); mutations of FRMD7 are causative of idiopathic infantile nystagmus influencing neuronal outgrowth and development. It is unclear whether the periodic alternating nystagmus phenotype is linked to NYS1, NYS5 (Xp11.4-p11.3) or a separate locus. From a cohort of 31 X-linked families and 14 singletons (70 patients) with idiopathic infantile nystagmus we identified 10 families and one singleton (21 patients) with periodic alternating nystagmus of which we describe clinical phenotype, genetic aetiology and neural substrates involved. Periodic alternating nystagmus was not detected clinically but only on eye movement recordings. The cycle duration varied from 90 to 280 s. Optokinetic reflex was not detectable horizontally. Mutations of the FRMD7 gene were found in all 10 families and the singleton (including three novel mutations). Periodic alternating nystagmus was predominantly associated with missense mutations within the FERM domain. There was significant sibship clustering of the phenotype although in some families not all affected members had periodic alternating nystagmus. In situ hybridization studies during mid-late human embryonic stages in normal tissue showed restricted FRMD7 expression in neuronal tissue with strong hybridization signals within the afferent arms of the vestibulo-ocular reflex consisting of the otic vesicle, cranial nerve VIII and vestibular ganglia. Similarly within the afferent arm of the optokinetic reflex we showed expression in the developing neural retina and ventricular zone of the optic stalk. Strong FRMD7 expression was seen in rhombomeres 1 to 4, which give rise to the cerebellum and the common integrator site for both these reflexes (vestibular nuclei). Based on the expression and phenotypic data, we hypothesize that periodic alternating nystagmus arises from instability of the optokinetic-vestibular systems. This study shows for the first time that mutations in FRMD7 can cause idiopathic infantile periodic alternating nystagmus and may affect neuronal circuits that have been implicated in acquired forms.

Improvement in academic behaviors after successful treatment of convergence insufficiency.

PURPOSE: To determine whether treatment of symptomatic convergence insufficiency (CI) has an effect on Academic Behavior Survey (ABS) scores. METHODS: The ABS is a six-item survey developed by the Convergence Insufficiency Treatment Trial Group that quantifies the frequency of adverse school behaviors and parental concern about school performance on an ordinal scale from 0 (never) to 4 (always) with total scores ranging from 0 to 24. The ABS was administered at baseline and after 12 weeks of treatment to the parents of 218 children aged 9 to 17 years with symptomatic CI, who were enrolled in the Convergence Insufficiency Treatment Trial and randomized into (1) home-based pencil push-ups; (2) home-based computer vergence/accommodative therapy and pencil push-ups; (3) office-based vergence/accommodative therapy with home reinforcement; and (4) office-based placebo therapy with home reinforcement. Participants were classified as successful (n = 42), improved (n = 60), or non-responder (n = 116) at the completion of 12 weeks of treatment using a composite measure of the symptom score, nearpoint of convergence, and positive fusional vergence. Analysis of covariance methods were used to compare the mean change in ABS between response to treatment groups while controlling for the ABS score at baseline. RESULTS: The mean ABS score for the entire group at baseline was 12.85 (SD = 6.3). The mean ABS score decreased (improved) in those categorized as successful, improved, and non-responder by 4.0, 2.9, and 1.3 points, respectively. The improvement in the ABS score was significantly related to treatment outcome (p < 0.0001), with the ABS score being significantly lower (better) for children who were successful or improved after treatment as compared to children who were non-responders (p = 0.002 and 0.043, respectively). CONCLUSIONS: A successful or improved outcome after CI treatment was associated with a reduction in the frequency of adverse academic behaviors and parental concern associated with reading and school work as reported by parents.

A Story of Discovery and Change: What We Learned from Studying Nystagmus in Infancy and Childhood.

The last 40 years of clinical experience and investigation of nystagmus in infancy and childhood have resulted in a clearer understanding and characterization of the disorder and provided guidelines for the beneficial effects of increased medical, optical, and surgical treatments. Our modern understanding is due to the results of contribution by scientists and clinicians representing vision, neurology, bioengineering, genetics, and pediatrics. From this group, a new classification system was developed. The routine use of eye-movement recordings (EMR) and its characteristics have made the largest contribution to this increased knowledge. The value of EMR is due to the observation of specific waveforms, which are the basis for classification, neuro-pathophysiology, effect on vision, and a measure of nystagmus response to treatment. Successful treatment of the visual system is now directed toward associated afferent system abnormalities (decreased acuity, amblyopia, ametropia, retinal and optic pathway disease, and photophobia), centrally at the neuronal level responsible for the oscillations (oral and topical medications) and peripherally to reduce the underlying oscillation (eye-muscle surgery and botulinum toxin). Evidence over the last 40 years has shown that these treatments result in increased spatial acuity, contrast sensitivity, visual recognition time, motion processing, gaze-dependent visual acuity, and anomalous head posturing. The hope of this review is that clinicians now provide hope for these patients and families and can be counseled that nystagmus treatment is available.

Vision and Concussion: Symptoms, Signs, Evaluation, and Treatment.

Visual symptoms are common after concussion in children and adolescents, making it essential for clinicians to understand how to screen, identify, and initiate clinical management of visual symptoms in pediatric patients after this common childhood injury. Although most children and adolescents with visual symptoms after concussion will recover on their own by 4 weeks, for a subset who do not have spontaneous recovery, referral to a specialist with experience in comprehensive concussion management (eg, sports medicine, neurology, neuropsychology, physiatry, ophthalmology, otorhinolaryngology) for additional assessment and treatment may be necessary. A vision-specific history and a thorough visual system examination are warranted, including an assessment of visual acuity, ocular alignment in all positions of gaze, smooth pursuit (visual tracking of a moving object), saccades (visual fixation shifting between stationary targets), vestibulo-ocular reflex (maintaining image focus during movement), near point of convergence (focusing with both eyes at near and accommodation (focusing with one eye at near because any of these functions may be disturbed after concussion. These deficits may contribute to difficulty with returning to both play and the learning setting at school, making the identification of these problems early after injury important for the clinician to provide relevant learning accommodations, such as larger font, preprinted notes, and temporary use of audio books. Early identification and appropriate management of visual symptoms, such as convergence insufficiency or accommodative insufficiency, may mitigate the negative effects of concussion on children and adolescents and their quality of life.

Evaluation of the Visual System by the Primary Care Provider Following Concussion.

Concussion is a common injury in childhood and has the potential for substantial impact on quality of life. Visual issues have been increasingly recognized as a common problem after concussion. Many children initially seek care for concussion with their pediatrician, making it even more important for pediatricians to recognize, evaluate, and refer children with visual issues after concussion. This clinical report is intended to support the recommendations in the companion policy statement on vision and concussion and provides definitions of some of the physiologic aspects of the visual system as they relate to concussion. A description of clinically feasible testing methodologies is provided in more detail to aid the clinician in assessing the visual system in a focused fashion after concussion. This guidance helps direct clinical management, including support for return to school, sports, and other activities, as well as potential referral for subspecialty care for the subset of those with persistent symptoms.

Safety and efficacy of gene transfer for Leber's congenital amaurosis.

Leber's congenital amaurosis (LCA) is a group of inherited blinding diseases with onset during childhood. One form of the disease, LCA2, is caused by mutations in the retinal pigment epithelium-specific 65-kDa protein gene (RPE65). We investigated the safety of subretinal delivery of a recombinant adeno-associated virus (AAV) carrying RPE65 complementary DNA (cDNA) (ClinicalTrials.gov number, NCT00516477 [ClinicalTrials.gov]). Three patients with LCA2 had an acceptable local and systemic adverse-event profile after delivery of AAV2.hRPE65v2. Each patient had a modest improvement in measures of retinal function on subjective tests of visual acuity. In one patient, an asymptomatic macular hole developed, and although the occurrence was considered to be an adverse event, the patient had some return of retinal function. Although the follow-up was very short and normal vision was not achieved, this study provides the basis for further gene therapy studies in patients with LCA.

Effects of topical brinzolamide on infantile nystagmus syndrome waveforms: eyedrops for nystagmus.

BACKGROUND: Recent advances in infantile nystagmus syndrome (INS) surgery have uncovered the therapeutic importance of proprioception. In this report, we test the hypothesis that the topical carbonic anhydrase inhibitor (CAI) brinzolamide (Azopt) has beneficial effects on measures of nystagmus foveation quality in a subject with INS. METHODS: Eye movement data were taken, using a high-speed digital video recording system, before and after 3 days of the application of topical brinzolamide 3 times daily in each eye. Nystagmus waveforms were analyzed by applying the eXpanded Nystagmus Acuity Function (NAFX) at different gaze angles and determining the longest foveation domain (LFD) and compared to previously published data from the same subject after the use of a systemic CAI, contact lenses, and convergence and to other subjects before and after eye muscle surgery for INS. RESULTS: Topical brinzolamide improved foveation by both a 51.9% increase in the peak value of the NAFX function (from 0.395 to 0.600) and a 50% broadening of the NAFX vs Gaze Angle curve (the LFD increased from 20° to 30°). The improvements in NAFX after topical brinzolamide were equivalent to systemic acetazolamide or eye muscle surgery and were intermediate between those of soft contact lenses or convergence. Topical brinzolamide and contact lenses had equivalent LFD improvements and were less effective than convergence. CONCLUSIONS: In this subject with INS, topical brinzolamide resulted in improved-foveation INS waveforms over a broadened range of gaze angles. Its therapeutic effects were equivalent to systemic CAI. Although a prospective clinical trial is needed to prove efficacy or effectiveness in other subjects, an eyedrops-based therapy for INS may emerge as a viable addition to optical, surgical, behavioral, and systemic drug therapies.

Surgery for the treatment of vertical head posturing associated with infantile nystagmus syndrome: results in 24 patients.

BACKGROUND: The study of the clinical and electrophysiological effects of eye muscle surgery on patients with infantile nystagmus has broadened our knowledge of the disease and its interventions. DESIGN: Prospective, comparative, interventional case series. PARTICIPANTS: Twenty-four patients with a vertical head posture because of electrophysiologically diagnosed infantile nystagmus syndrome. The ages ranged from 2.5 to 38 years and follow up averaged 14.0 months. METHODS: Thirteen patients with a chin-down posture had a bilateral superior rectus recession, inferior oblique myectomy and a horizontal rectus recession or tenotomy. Those 11 with a chin-up posture had a bilateral superior oblique tenectomy, inferior rectus recession and a horizontal rectus recession or tenotomy. MAIN OUTCOME MEASURES: Outcome measures included: demography, eye/systemic conditions and preoperative and postoperative; binocular, best optically corrected, null zone acuity, head posture, null zone foveation time and nystagmus waveform changes. RESULTS: Associated conditions were strabismus in 66%, ametropia in 96%, amblyopia in 46% and optic nerve, foveal dysplasia or albinism in 54%. Null zone acuity increased at least 0.1 logMAR in 20 patients (P < 0.05 group mean change). Patients had significant (P < 0.05) improvements in degrees of head posture, average foveation time in milliseconds and infantile nystagmus syndrome waveform improvements. CONCLUSIONS: This study illustrates a successful surgical approach to treatment and provides expectations of ocular motor and visual results after vertical head posture surgery because of an eccentric gaze null in patients with infantile nystagmus syndrome.

Enough Sight to Fight? The history of military visual system requirements.

Medicine has a six-fold role to play in war, 1) selection of suitable military personnel, 2) to protect against disease, 3) to give medical attention and treatment to those who are ill and injured, 4) to assist in the rehabilitation of the disabled, 5) research to improve etiology and treatments of disease, and 6) the unit surgeon serves as a special staff officer to the military commander at all levels of a battalion and above, along with the attorney and chaplain, and supplies medical input into all plans whether it be training or combat operations. This article focuses on the realization by soldiers, since antiquity, the importance of the visual system in battle, how this was measured, and how modern military visual requirements have evolved along with the science of ophthalmology. Necessity and natural selection were the driving forces for recruitment and assignment in ancient and medieval armies. Since the advent of mechanized warfare, more soldiers can perform more tasks, more uniformly, radically changing the way soldiers are selected. Modern military duty eligibility requirements and assignments are now the result of special tests and documentation procedures resulting from a mixture of medical knowledge, science, and potential legal consequences. Nowhere is this more evident in the stringent visual system requirements for military service.

Analysis of anomalous head posturing in patients with infantile nystagmus syndrome.

PURPOSE: To investigate anomalous head posturing in patients with INS. METHODS: This was a prospective, cohort analysis of clinical and anomalous head posture (AHP) data in 34 patients with INS and an AHP. Particular outcome measures included measurement of AHP in three dimensions of pitch (anterior posterior flexion/extension), yaw (lateral rotation), and roll (lateral flexion) during best-corrected binocular acuity testing and during their subjective sense of straight. Patients were also queried as to their subjective sense of head posture in forced straight position and in their preferred AHP. The paired t test was used to determine significance in differences between measures. RESULTS: A total of 34 patients (19 males [56%]) 9-56 years of age (mean, 16.5 ± 6) were included. Associated systemic or ocular system deficits were present in 30 patients (88%). AHP during best-corrected visual acuity testing averaged 16.5° ± 8.20° (range, 10°-51°), which was significantly different from the mean voluntary "comfortable" position only in the pitch and roll directions (P < 0.001). There was a significant noncongruous response during subjective response to head posturing with most sensing their head as "crooked" (76.5%) when manually straightened (P = 0.001). CONCLUSIONS: The clinical AHP of patients with INS exists in all three spatial dimensions of pitch, yaw, and roll. Although the visual system may be causally related to the onset, amount, and direction of a compensatory AHP in patients with INS, its persistence over time or after surgical intervention is likely due to a combination of visual system (eg, nystagmus, strabismus) and nonvisual system (egocentric and musculo-skeletal) factors.

Clinical and Electrophysiological Outcomes After Eye Muscle Surgery in 81 Adults With Infantile Nystagmus Syndrome.

PURPOSE: To characterize the effects of eye muscle surgery on patients older than 18 years with infantile nystagmus syndrome (INS) who have had only optical treatment. METHODS: This was a prospective, single-center, interventional case series analysis of clinical and electro-phyisological data before and after surgery. Outcome measures included: clinical characteristics, surgical procedure, and preoperative and postoperative binocular best corrected visual acuity (BCVA) in the null position, anomalous head posture (AHP), contrast sensitivity, strabismic deviation, and nystagmus acuity function (NAFX). Postoperative data used were collected for a minimum of 12 months after surgery. Parametric and non-parametric statistical analysis of the outcome measures was performed. RESULTS: Ages ranged from 18 to 72 years (average: 36 years) and follow-up from 12 to 74 months (average: 26 months). A surgical algorithm of nine separate procedures involving at least two recti muscles on each eye was used for each patient. Most patients had associated systemic and/or ocular diagnoses, including albinism (35%), amblyopia (23%), optic nerve or retinal disorders (48%), refractive error (80%), AHP (44%), aperiodicity (27%), and strabismus (69%). There were no serious surgical complications, with a reoperation rate of 12%. There were significant group mean increases in BVCA, AHP, contrast sensitivity, strabismic deviation, and NAFX after surgery. Sixty percent of patients who were legally ineligible for driving prior to surgery became eligible after eye muscle surgery. CONCLUSIONS: Adult patients with INS showed sustained improvement in many afferent and efferent measures of visual function after eye muscle surgery. [J Pediatr Ophthalmol Strabismus. 2021;58(2):93-104.].

A systematic approach to eye muscle surgery for infantile nystagmus syndrome: results in 100 patients.

PURPOSE: To report a systematic approach to, and the visual and electrophysiological effect of, eye muscle surgery in 100 patients with infantile nyustagmus syndrome (INS). METHODS: Prospective, interventional case cohort analysis of clinical and eye movement data in 100 patients with INS who had virgin extraocular eye muscles operated on for nystagmus with or without combinations of strabismus and an anomalous head posture. All patients were followed at least 9 months after surgery. Outcome measures, part of an IRB approved study, included binocular visual acuity, head position, strabismic deviation, and eye movement recordings, from which waveform types and an Automated Nystagmus Acuityn Function (ANAF) was calculated. Computerized parametric and non-parametric statistical analysis of data were performed using standard software on both individual and group data. RESULTS: There were 9 consistent surgical procedures used with the most common being that for a horizontal head posture alone (22%). Age at surgery averaged 14 years with 11 months followup. Sixty-eight percent had associated eye disease (optic nerve, retinal, amblyopia, cataracts). Group means in binocular acuity, strabismic deviation, head posture, abd ANAF measures from eye improved for all procedures. There were 12 (12%) reoperations without any serious surgical complications. Individual analysis revealed only age and head posture differences in outcome measures between the 9 procedures. CONCLUSIONS: Using this approach, surgery on the extraocular muscles in patients with INS results in improvements in multiple aspects of ocular motor and visual function.