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Purpose Children with high ametropia who have difficulties with spectacle or contact lens wear benefit from gains in visual function after intraocular collamer lens (ICL) surgery. Here we assess safety by reporting rates and case specifics of peri-operative and longer-term adverse events (AE).[LINE SEPARATOR] SETTING:: St. Louis Children's Hospital at Washington University Medical Center. Design Retrospective Case Series. Methods Clinical data were collated retrospectively for 95 special-needs children (160 eyes) implanted with a Visian ICL over the last 5 years. All surgeries were performed at St Louis Children's Hospital under brief general anesthesia. Follow-up was a mean 2.0 ±1.4 years (range 0.5-5.2). Results Average age at implantation was 9.3 ± 5.2 years (range 1.8-25) and mean pre-operative SEQRE was -11.2 ± 3.9 D (range - 4-22). Sixty-two children (65%) had a neurodevelopmental disorder. The minor AE rate was 2% (3 eyes), consisting of steroid-response ocular hypertension (OHT), which resolved with cessation of topical steroid drops. Endothelial cell loss averaged 8.1 % over 2 years, comparable to that reported in ICL implanted adults. The most common major AE (4%, 7 eyes) was post-operative pupillary block, requiring revision of the peripheral iridotomy. One child (0.6%, 1 eye) with self-injurious behavior required repair of a wound leak. One child (0.6%, 1 eye) with Down syndrome developed a cataract 2.8 years after ICL surgery. And one child (0.6%, 1 eye) with severe Autism Spectrum Disorder experienced traumatic retinal detachment 1.2 years after implantation. Conclusion The most common major AE among our Visian ICL cohort was pupillary block due to closure of the iridotomy. Overall, the AE rate was low in this higher risk, difficult-to-manage population of special-needs children.

The Artisan-Ophtec aphakic iris-enclaved anterior chamber intraocular lens (IOL) has been used to advantage in children and adults who lack capsular support for implantation of a standard posterior chamber IOL. However, even the most skilled anterior segment surgeon may encounter difficulties enclaving the IOL to the iris using the enclavation needle. We describe a simple, secure, rapid alternative method for enclaving the IOL that requires only two eye incisions: one at the corneal limbus and the other through the pars plana.

This review assesses the risk of a photic-induced seizure in a child during viewing of 3D (binocular 3 dimensional, stereoscopic) movies or games, either on standard video displays or when wearing a virtual reality (VR) headset. Studies published by pediatric epilepsy experts emphasize the low risk of 3D viewing even for children with known photosensitive epilepsy (PSE). The low incidence of PSE is noteworthy because the number of hours devoted to 2D or 3D screen viewing and/or VR headset use by children worldwide has increased markedly over the last decade. The medical literature does not support the notion that VR headset use poses a risk for PSE.

BACKGROUNDWolfram syndrome is a rare ER disorder characterized by insulin-dependent diabetes mellitus, optic nerve atrophy, and progressive neurodegeneration. Although there is no treatment for Wolfram syndrome, preclinical studies in cell and rodent models suggest that therapeutic strategies targeting ER calcium homeostasis, including dantrolene sodium, may be beneficial.METHODSBased on results from preclinical studies on dantrolene sodium and ongoing longitudinal studies, we assembled what we believe is the first-ever clinical trial in pediatric and adult Wolfram syndrome patients with an open-label phase Ib/IIa trial design. The primary objective was to assess the safety and tolerability of dantrolene sodium in adult and pediatric Wolfram syndrome patients. Secondary objectives were to evaluate the efficacy of dantrolene sodium on residual pancreatic β cell functions, visual acuity, quality-of-life measures related to vision, and neurological functions.RESULTSDantrolene sodium was well tolerated by Wolfram syndrome patients. Overall, β cell functions were not significantly improved, but there was a significant correlation between baseline β cell functions and change in β cell responsiveness (R2, P = 0.004) after 6-month dantrolene therapy. Visual acuity and neurological functions were not improved by 6-month dantrolene sodium. Markers of inflammatory cytokines and oxidative stress, such as IFN-γ, IL-1β, TNF-α, and isoprostane, were elevated in subjects.CONCLUSIONThis study justifies further investigation into using dantrolene sodium and other small molecules targeting the ER for treatment of Wolfram syndrome.TRIAL REGISTRATIONClinicalTrials.gov identifier NCT02829268FUNDINGNIH/National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) (DK112921, DK113487, DK020579), NIH/National Center for Advancing Translational Sciences (NCATS) (TR002065, TR000448), NIH training grant (F30DK111070), Silberman Fund, Ellie White Foundation, Snow Foundation, Unravel Wolfram Syndrome Fund, Stowe Fund, Eye Hope Foundation, Feiock Fund, Washington University Institute of Clinical and Translational Sciences grant UL1TR002345 from NIH/NCATS, Bursky Center for Human Immunology & Immunotherapy Programs.

BackgroundWolfram syndrome is a rare endoplasmic reticulum disorder characterized by insulin-dependent diabetes mellitus, optic nerve atrophy, and progressive neurodegeneration. Although there is currently no treatment to delay, halt, or reverse the progression of Wolfram syndrome, preclinical studies in cell and rodent models suggest that therapeutic strategies targeting endoplasmic reticulum calcium homeostasis, including dantrolene sodium, may be beneficial.MethodsBased on the results from preclinical studies on dantrolene sodium and ongoing longitudinal studies, our group put together the first-ever clinical trial in pediatric and adult patients with Wolfram syndrome. An open-label phase 1b/2a trial design was chosen. The primary objective of the study was to assess the safety and tolerability of dantrolene sodium in adult and pediatric patients with Wolfram syndrome. Secondary objectives were to evaluate the efficacy of dantrolene sodium on residual pancreatic β-cell functions, visual acuity, quality of life measures related to vision, and neurological functions.ResultsThe results indicate that dantrolene sodium is well tolerated by patients with Wolfram syndrome. Although the study was small, a select few patients seemed to have improvements in β-cell function, which might correlate with a positive trend in other outcome measures, including visual acuity and neurological functions.ConclusionThis study justifies further investigation into using dantrolene sodium and other small molecules targeting the endoplasmic reticulum for the treatment of Wolfram syndrome.Trial registrationClinicalTrials.gov Identifier NCT02829268Key PointsQuestionIs dantrolene sodium safe and effective for the treatment of adult and pediatric patients with Wolfram syndrome?FindingsThe results of this open-label clinical trial show that dantrolene sodium is well tolerated by patients with Wolfram syndrome. Although the study was small, a select few patients seemed to have improvements in β-cell function, which might correlate with a positive trend in other outcome measures, including visual acuity and neurological functions.MeaningDantrolene sodium is well tolerated by patients with Wolfram syndrome. Some patients may experience an increase in β cell function when taking dantrolene.ImportanceWolfram syndrome is a rare endoplasmic reticulum disorder characterized by insulin-dependent diabetes mellitus, optic nerve atrophy, and progressive neurodegeneration. Although there is currently no treatment to delay, halt, or reverse the progression of Wolfram syndrome, preclinical studies in cell and rodent models suggest that targeting endoplasmic reticulum calcium homeostasis, including dantrolene sodium, is an emerging therapeutic strategy.ObjectiveThe primary objective of the study was to assess the safety and tolerability of dantrolene sodium in adult and pediatric subjects with Wolfram syndrome. Secondary objectives were to evaluate the efficacy of dantrolene sodium on residual pancreatic β-cell functions, visual acuity, quality of life measures related to vision, and neurological functions.DesignOpen-label phase 1b/2a trial of dantrolene sodium over a 6-month treatment period.SettingSingle site, academic medical center.ParticipantsAdult and pediatric subjects with a genetically confirmed diagnosis of Wolfram syndrome.InterventionsAll subjects received increasing doses of dantrolene sodium.Main Outcomes and MeasuresThe safety and tolerability of dantrolene sodium administered orally at the upper end of therapeutic dose range for 6 months, and the efficacy of dantrolene sodium on residual pancreatic β-cell functions using a mixed-meal tolerance test, visual acuity using LogMar scores, quality of life measures related to vision using Visual Functioning Questionnaire – 25, and neurological functions using the Wolfram Unified Rating Scale (WURS) and standard neurological assessments.ResultsThe results indicate that dantrolene sodium is well tolerated by subjects with Wolfram syndrome. Although the study was small, a select few subjects seemed to have improvements in β-cell function, which might be correlated with a positive trend in visual acuity.Conclusions and RelevanceThis study justifies further investigation into using dantrolene sodium and other small molecules targeting the endoplasmic reticulum for the treatment of Wolfram syndrome.Trial RegistrationRegistered with clinicaltrials.gov, NCT02829268, (https://clinicaltrials.gov/ct2/show/NCT02829268?term=NCT02829268&draw=2&rank=1)

Purpose To analyze outcomes in a large cohort of spectacle-aversive children with high myopia who were treated by implantation of the Ophtec-Artisan or Visian phakic intraocular lens (pIOL). Methods Outcome data were collated retrospectively in 78 children (115 eyes) implanted with the Ophtec-Artisan iris-enclaved anterior chamber pIOL and 91 children (154 eyes) implanted with the Visian ICL (intraocular collamer lens) sulcus pIOL. All children had difficulties with spectacle or contact lens wear. Mean age at surgery was 9.9 years; mean follow-up was 3.9 years (range, 0.6-14.1 years). Results A total of 248 of 269 eyes (92%) were corrected to within ± 0.5 D of their target value. Spherical correction averaged 12.3 ± 1.0 D. Refractive spherical regression was −0.04 D/year at last follow-up. Uncorrected distance visual acuity improved from an average logMAR 1.8 to 0.4; corrected distance visual acuity improved an average 0.3 logMAR. Of the treated children, 68% had a gain in binocular fusion. Neurobehavioral and/or visuomotor comorbidities were present in 87% of children. Five eyes (2%) developed retinal detachment an average 6 years after implantation. Nine eyes (3%) implanted with the Ophtec-Artisan pIOL required repositioning after trauma. Conclusions Implantation of pIOLs in children is an effective method for correcting high myopia in spectacle noncompliant children. Rates of myopic regression after pIOL surgery are substantially lower than those reported for children treated by excimer laser photorefractive keratectomy (PRK). The prevalence of major complications was relatively low in this high-risk population.

Purpose A subset of children with high anisometropia or isoametropia and neurobehavioral disorders have chronic difficulties with spectacle or contact lens wear. We report the results of refractive surgery in a series of these children treated using bilateral or unilateral intraocular collamer lens (Visian ICL) implantation for moderate to high myopia. Design Prospective nonrandomized cohort study. Methods Clinical course and outcome data were collated prospectively for 40 implanted eyes in 23 children (mean age 10.2 ± 5.3 years, range, 1.8–17 years). Myopia ranged from −3.0 to −14.5 diopters (D), mean −9.2 ± 3.5 D. Goal refraction was plano to +1 D. Correction was achieved by sulcus implantation of a Visian ICL (STAAR Surgical, Monrovia, California, USA) under general anesthesia. Mean follow-up was 15.1 months (range, 6–22 months). Results Thirty-five eyes (88%) were corrected to within ±1.0 D of goal refraction; the other 5 (12%) were corrected to within 1.5 D. Uncorrected distance visual acuity improved substantially in all eyes (from mean 20/1050 [logMAR 1.72] to mean 20/42 [logMAR 0.48]). Spherical regression at last follow-up was an average of +0.59 D. Visuomotor comorbidities (eg, amblyopia, nystagmus, foveopathy, optic neuropathy) accounted for residual postoperative subnormal visual acuity. Thirteen of the 23 children (57%) had a neurobehavioral disorder (eg, developmental delay/intellectual disability/mental retardation, Down syndrome, cerebral palsy, autism spectrum disorder). Eighty-five percent (11/13) of those children were reported to have enhanced visual awareness, attentiveness, or social interactions. Endothelial cell density was measureable in 6 cooperative children (10 eyes), showing an average 1% decline. Central corneal thickness, measured in all children, increased an average of 8 μm. Two children (8%) required unplanned return to the operating room on the first postoperative day to alleviate pupillary block caused by a nonpatent iridotomy. No other complications were encounterd. Conclusion Visian ICL implantation improves visual function in special needs children who have moderate to high myopia and difficulties wearing glasses or contact lenses.

Wolfram syndrome is a rare, progressive neurodegenerative disorder characterized by juvenile-onset diabetes, optic nerve atrophy and mutation of the WFS1 gene. Calcium dyshomeostasis is a key mechanism underlying neuronal and pancreatic β-cell death and dysfunction in this endoplasmic reticulum (ER) disorder. Dantrolene sodium is an FDA-approved muscle relaxant that preserves ER calcium through inhibition of ryanodine receptor (RyR) activity. We therefore hypothesized that dantrolene could have therapeutic potential for Wolfram syndrome. Our preclinical studies showed that dantrolene improves β-cell and neuronal cell survival in mouse and induced pluripotent stem (iPS) cell models of Wolfram syndrome. To translate these findings to humans, we conducted the first clinical trial in 19 Wolfram syndrome patients through a 6-month phase 1b/2 study of dantrolene sodium with an optional extension phase up to 24-months (NCT02829268). The endpoints of this study were to assess: (1) dantrolene safety and tolerability in pediatric and adult Wolfram syndrome patients and (2) its effects on remaining β-cell, visual and neurologic functions and quality of life measures. Among pediatric subjects, dantrolene was well-tolerated at a daily dose of 0.5mg/kg in those < 50kg and 2mg/kg in those > 50kg. Adults tolerated dantrolene well at 50mg - 100mg daily. Common adverse events included mild fatigue, diarrhea, headache and hypoglycemia. A mild increase in 30-minute mixed-meal-stimulated C-peptide was observed in subjects after 6- and 12-months of dantrolene treatment. No significant changes were observed in visual or neurological functions or quality of life measures, although mild improvement in disease severity was noted by the Wolfram Unified Rating Scale. These findings suggest that dantrolene is safe for use in Wolfram syndrome at the specified doses and support further study of dantrolene in a randomized, placebo-controlled trial. Disclosure D. Abreu: None. T.S. Pearson: None. R.C. Bucelli: None. A. Simpson: None. C.M. Brown: None. K. Kries: None. H. Gu: None. S.I. Stone: Research Support; Self; National Institute of Diabetes and Digestive and Kidney Diseases. Speaker’s Bureau; Self; AbbVie Inc. L. Tychsen: None. G. Van Stavern: None. N.H. White: None. B.A. Marshall: None. T. Hershey: Research Support; Spouse/Partner; Sage Pharmaceuticals. F. Urano: Board Member; Self; Healthbeat. Research Support; Self; Aetas, Amylyx, JDRF, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health. Stock/Shareholder; Self; CytRx. Other Relationship; Self; Novus Biologicals. Funding National Institutes of Health (R21DK113487-02)

Purpose To assess the safety of VR 3D headset (virtual reality 3-dimensional binocular-stereoscopic near-eye display) use in young children. Product safety warnings that accompany VR headsets ban their use in children under age 13 years. Design Prospective, interventional, before-and-after study. Methods Recordings were obtained in 50 children (29 boys) aged 4-10 years (mean 7.2 ± 1.8 years). Minimum binocular corrected distance visual acuity (CDVA) was 20/50 (logarithm of the minimum angle of resolution [logMAR] 0.4) and stereoacuity 800 seconds of an arc or better. A Sony PlayStation VR headset was worn for 2 sequential play sessions (of 30 minutes each) of a first-person 3D flying game (Eagle Flight) requiring head movement to control flight direction (pitch, yaw, and roll axes). Baseline testing preceded VR exposure, and each VR session was followed by post-VR testing of binocular CDVA, refractive error, binocular eye alignment (strabismus), stereoacuity, and postural stability (imbalance). Visually induced motion sickness was probed using the Simulator Sickness Questionnaire modified for pediatric use (Peds SSQ). Visual-vestibulo-ocular reflex (V-VOR) adaptation was also tested pre- vs post-trial in 5 of the children. Safety was gauged as a decline or change from baseline in any visuomotor measure. Results Forty-six of 50 children (94%) completed both VR play sessions with no significant change from baseline in measures of binocular CDVA (P = .89), refractive error (P = .36), binocular eye alignment (P = .90), or stereoacuity (P = .45). Postural stability degraded an average 9% from baseline after 60 minutes of VR exposure (P = .06). Peds SSQ scores increased a mean 4.7%—comparing pretrial to post-trial—for each of 4 symptom categories: eye discomfort (P = .02), head/neck discomfort (P = .03), fatigue (P = .03), and motion sickness (P = .01). None of the children who finished both trial sessions (94%) asked to end the play, and the majority were disappointed when play was halted. V-VOR gain remained unaltered in the 5 children tested. Three children (6% of participants) discontinued the trial during the first 10 minutes of the first session of VR play, 2 girls (aged 5 and 6 years) and 1 boy (aged 7 years). The girls reported discomfort consistent with mild motion sickness; the boy said he was bored and the headset was uncomfortable. No child manifested aftereffects (“flashbacks”) in the days following the VR exposure. Conclusion Young children tolerate fully immersive 3D virtual reality game play without noteworthy effects on visuomotor functions. VR play did not induce significant post-VR postural instability or maladaption of the vestibulo-ocular reflex. The prevalence of discomfort and aftereffects may be less than that reported for adults.

Binocular fusion is blending of corresponding images from each eye to form a single percept. Maldevelopment of fusion (binocular non-correspondence) in infancy causes a specific type of lifelong ocular instability: fusion maldevelopment nystagmus (FMN). Because fusion maldevelopment – in the form of strabismus and amblyopia – is common, FMN is the most prevalent pathologic nystagmus encountered in clinical practice. Experiments on nonhuman primates (NHP) with strabismus and amblyopia have revealed that loss of binocular connections within area V1 (striate cortex) in the first months of life is the necessary and sufficient cause of FMN. The severity of FMN increases with greater losses of V1 connections. No manipulation of brainstem motor pathways is required. The binocular maldevelopment originating in area V1 is passed on to downstream, extrastriate regions of cerebral cortex that drive conjugate gaze (notably MSTd). Conjugate gaze is stable when MSTd neurons of the right vs. left cerebral hemisphere have balanced, binocular activity. Fusion maldevelopment causes unbalanced, monocular activity. If input from one eye dominates and the other is suppressed, MSTd in one hemisphere becomes more active. Downstream projections to the ipsilateral nucleus of the optic tract (NOT) drive the eyes conjugately to that side. The unbalanced MSTd drive is evident as nasalward slow-phase nystagmus when viewing with either eye.

A 10-year-old boy was referred for a circumscribed choroidal hemangioma with underlying exudative detachment of the left eye. To avoid general anesthetics required for laser-based therapy in a child, we began a trial of oral propranolol. The patient's exudative detachment resolved, with resulting improvement in visual acuity, and remained quiescent for 3 years.

Background/aimsTo report alterations in visual acuity and visual pathway structure over an interval of 1–3 years in a cohort of children, adolescents and young adults who have Wolfram syndrome (WFS) and to describe the range of disease severity evident in patients with WFS whose ages differed by as much as 20 years at first examination.MethodsAnnual, prospective ophthalmological examinations were performed in conjunction with retinal nerve fibre layer (RNFL) analysis. Diffusion tensor MRI-derived fractional anisotropy was used to assess the microstructural integrity of the optic radiations (OR FA).ResultsMean age of the 23 patients with WFS in the study was 13.8 years (range 5–25 years). Mean log minimum angle resolution visual acuity was 0.66 (20/91). RNFL thickness was subnormal in even the youngest patients with WFS. Average RNFL thickness in patients with WFS was 57±8 µ or ~40% thinner than that measured in normal (94±10 µ) children and adolescents (PConclusionThe genetic mutation of WFS causes damage to both pregeniculate and postgeniculate regions of the visual pathway. The damage is progressive. The decline in visual pathway structure is accompanied by declines of visual function. Disease severity differs widely in individual patients and cannot be predicted from their age.

Diagnosing idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, can be challenging in children. Diagnosis is based on lumbar puncture, opening pressures, and appearance of the optic disk. Misdiagnosis of papilledema, a typical finding, may lead to unnecessary treatments and procedures. We report 52 children over a 6-year period to better identify the true incidence of pseudopapilledema and other factors that may confound the diagnosis of IIH. A retrospective chart review approved by the Institutional Review Board was performed. Fifty-two children under the age of 21 referred to us based on suspected IIH or papilledema from 2007 to 2013 are included in this study. Patients were assessed by a pediatric ophthalmologist and a neurosurgeon. Fifty-two children were initially diagnosed with IIH and/or papilledema; 26 diagnoses were revised to pseudopapilledema after pediatric ophthalmological review. Out of those 26 patients with pseudopapilledema, 14 had undergone lumbar punctures, 19 had MRIs, 9 had CTs, and 12 were taking medications—these medications were discontinued upon revision of the diagnoses. The difference in the CSF opening pressure between children diagnosed with true IIH (32.7 cm H2O) and children diagnosed with pseudopapilledema (24.7 cm H2O) was statistically significant. IIH diagnosis is heavily reliant on the appearance of the optic disk. Pediatric ophthalmological assessment is essential to carefully examine the optic disk and prevent further unnecessary investigation and treatments. Close communication between pediatricians, ophthalmologists, and neurosurgeons can avoid invasive procedures for children who do have pseudopapilledema, and not IIH or associated papilledema.

Lymphocytic choriomeningitis virus is a rodent-borne arenavirus that can cause congenital infection affecting the developing central nervous system. When the infection occurs during pregnancy, the virus targets the fetal brain and retina, potentially causing ventriculomegaly, hydrocephalus, chorioretinitis, and neurodevelopmental abnormalities. It has been previously suggested that lymphocytic choriomeningitis virus be added to the list of congenital infections currently included in the TORCH acronym (toxoplasmosis, rubella, cytomegalovirus, herpes, and syphilis). We present 2 neonates with antenatally known ventriculomegaly that were diagnosed with congenital lymphocytic choriomeningitis virus infection after birth. In addition to ventriculomegaly, one had nonimmune hydrops fetalis and the other had intracranial hemorrhage. In view of the seroprevalence of lymphocytic choriomeningitis virus (4.7%-10%), our findings suggest that screening for congenital lymphocytic choriomeningitis virus infection should be considered in fetuses and newborns with ventriculomegaly as well as other abnormal neuroimaging findings such as intracranial hemorrhage.

Two groups of visually impaired children now benefit from refractive surgery and stand to further benefit with future advances. These children have difficulties with spectacle-wear and are unsuitable for contact lens-wear. The majority are neurologically normal and have anisometropic amblyopia. They are treated by advanced surface ablation using the excimer laser (photorefractive keratectomy or laser-assisted subepithelial keratomilieusis). Surface ablation is safe in children; the drawback is the high rate of pediatric refractive regression (more pronounced for myopia > 10.0 D, and hyperopia > 4.5 D). Ametropia beyond this range may be treated using phakic intraocular lens implantation or clear lens extraction, with or without implantation of a posterior chamber intraocular lens (refractive lens exchange). The minority are neurobehaviorally impaired children (e.g., cerebral palsy, autism, Angelman syndrome, Down syndrome and mental retardation) with high bilateral ametropia. Spectacle noncompliance releg...

Purpose A subset of children with high ametropia and neurobehavioral disorders have chronic difficulties with spectacle or contact lens wear. We report the results of refractive surgery in a series of these children treated using bilateral or unilateral phakic intraocular lenses (IOLs) for ametropia >10 D. Methods Clinical course and outcome data were collated prospectively in a group of 12 children (mean age, 10.1 years; range, 4-17 years) with neurobehavioral disorders exacerbated by poor compliance with spectacles. Myopia in 18 eyes ranged from −10.0 to −22.75 D (mean, −15.2 D) and in 2 hyperopic eyes from +10.25 to +10.75 (mean, +10.5 D). Goal refraction was approximately 0 to +1 D. Correction was achieved by implantation of an iris-enclaved phakic IOL under general anesthesia. Mean follow-up was 9.1 months (range, 3-15 months). Results Myopia correction averaged 14.5 D and hyperopia correction 9.4 D. Eighty-six percent of eyes (17/20 eyes) were corrected to within ± 1 D of emmetropia and the remaining 14% (3 eyes) to within ± 2 D. Uncorrected visual acuity improved substantially in all 20 eyes (60-fold; from a mean of 20/3400 to a mean of 20/57). Ocular comorbidities in each child accounted for residual postoperative, subnormal visual acuity (eg, amblyopia, nystagmus, albinism, regressed retinopathy of prematurity). Visual functions (measured using a 23-item validated survey) improved. One eye required IOL exchange; no other clinically significant complications have been encountered. Conclusions Phakic IOL implantation improves visual function substantially in neurobehaviorally impaired children who have high ametropia and difficulties wearing glasses.

Can ophthalmologists repair defects of visual cortex circuitry in infants who have esotropia? The answer to this question encompasses both sensory and motor behaviors because the clinical hallmarks of the disorder are stereoblindness and absence of motor fusion, which manifests as convergently deviated eyes. Functional recovery of sensory and motor fusion in infantile esotropia was a consuming interest, if not career-defining passion, of Marshall Parks. The purpose of this work is to pay tribute to Parks' legacy by showing how human and animal studies, conducted largely during the last 25 years, support both his clinical insights and treatment philosophy.

Purpose: We sought to determine whether laser subepithelial keratomileusis (LASEK) and photorefractive keratectomy (PRK) are effective methods for correcting amblyopiogenic refractive errors in children. Methods: Thirty-six eyes in 35 amblyopic children, who ranged in age from 4 to 16 years (mean, 8.4 years), received treatment for large magnitude ametropia. Seventy-two percent (25/35) of the children had a neurobehavioral disorder and/or were noncompliant with spectacle or contact lens wear. Myopia ranged from −3.25 to −24.25 D (mean, −11.48 D); one patient had hyperopia of +5.87 D. Correction was tailored to match the refractive error of the nonamblyopic eye. VISX Star S2/S3 excimer lasers were used in manual or auto-tracking modes, and corneal centration was achieved using brief, general anesthesia. Mean follow-up was 29.2 months (range, 4–42 months). Results: Myopia correction averaged −8.95 ± 2.89 D (range, −3.25 to −15.50). Eighty-nine percent (31 children) were corrected to within ± 1.00 D of goal refraction and the remaining 11% to within 2.0 D of the goal (most were undercorrected). Acuity improved postoperatively in 97%; by 1 optotype line in 37% and by 2 or more in 60%. No child lost acuity. Binocularity improved in 69% (24/35) and remained the same in 31%. Corneal haze measured grade 0–1 in 78%, grade 2 in 14%, and grade 3–4 in 8%. Myopic regression exceeding ≅ 1.0 D/year (0.08 D/month) occurred in 50% (18/36) of eyes treated. No substantial differences were observed in PRK- (n = 18) versus LASEK- (n = 17) treated children. Conclusions: Laser refractive surgery is effective for correcting anisometropic myopia in amblyopic children. Recurrence of myopia is common. Further study is indicated to determine long-term stability and safety of the procedure in this population.

Introduction: Suppression is a major sensorial abnormality in humans and monkeys with infantile strabismus. We previously reported evidence of metabolic suppression in the visual cortex of strabismic macaques, using the mitochondrial enzyme cytochrome oxidase as an anatomic label. The purpose of this study was to further elucidate alterations in cortical metabolic activity, with or without amblyopia. Materials and methods: Six macaque monkeys were used in the experiments (four strabismic and two control). Three of the strabismic monkeys had naturally occurring, infantile strabismus (two esotropic, one exotropic). The fourth strabismic monkey had infantile microesotropia induced by alternating monocular occlusion in the first months of life. Ocular motor behaviors and visual acuity were tested after infancy in each animal, and development of stereopsis was recorded during infancy in one strabismic and one control monkey. Ocular dominance columns (ODCs) of the striate visual cortex (area V1) were labeled using cytochrome oxidase (CO) histochemistry alone, or CO in conjunction with an anterograde tracer ([H 3 ]proline or WGA-HRP) injected into one eye. Results: Each of the strabismic monkeys showed inequalities of metabolic activity in ODCs of opposite ocularity, visible as rows of lighter CO staining, corresponding to ODCs of lower metabolic activity, alternating with rows of darker CO staining, corresponding to ODCs of higher metabolic activity. In monkeys who had infantile strabismus and unilateral amblyopia, lower metabolic activity was found in (suppressed) ODCs driven by the nondominant eye in each hemisphere. In monkeys who had infantile esotropia and alternating fixation (no amblyopia), metabolic activity was lower in ODCs driven by the ipsilateral eye in each hemisphere. The suppression included a monocular core zone at the center of ODCs and binocular border zones at the boundaries of ODCs. This suppression was not evident in the monocular lamina of the LGN, indicating an intracortical rather than subcortical mechanism. Conclusion: Suppression of metabolic activity in ODCs of V1 differs depending upon whether infantile strabismus is alternating or occurs in conjunction with unilateral amblyopia. Our findings reinforce the principle that unrepaired strabismus promotes abnormal competition in V1, observable as interocular suppression of ODCs.

Extraocular muscle tenotomy has been reported to damp congenital nystagmus in an achiasmatic sheepdog. We performed extraocular muscle tenotomy to evaluate its effects on congenital nystagmus in primates.Magnetic search coil eye movement recordings were used to document the presence of horizontal congenital nystagmus in 2 adult macaque monkeys that also had naturally occurring infantile strabismus. Extraocular muscle tenotomy was performed by operating on all 4 horizontal recti, surgically detaching the muscles from the globe and suturing them back to their original insertions without resection or recession. Eye movement recordings were repeated 4 months after the procedure, comparing the waveform, amplitude, retinal slip velocity, and intensity (frequency x amplitude) of the nystagmus before and after tenotomy. Visual acuity was also measured before and after surgery in 1 animal.Preoperatively, a disconjugate, pendular nystagmus was evident in 1 monkey, and the other had a conjugate pendular-jerk nystagmus damped by convergence. After tenotomy, nystagmus mean amplitude decreased 18% to 52% in 1 monkey but increased 14% in the other (t test, P.002). Retinal slip velocity and nystagmus intensity increased in both monkeys. After tenotomy, mean velocity increased 22% to 218%, while mean intensity increased 40% to 208% (t test, P.002). Visual acuity measured after tenotomy decreased an average of 20% ( approximately 2.0 cycles per degree) in each eye. Tenotomy had no noteworthy effects on eye alignment or other aspects of visual behavior other than the congenital nystagmus.Nystagmus velocity and intensity increased after extraocular muscle tenotomy in 2 monkeys. Further studies are required to establish the clinical value of this procedure as a treatment for various subtypes of congenital nystagmus in humans.

Purpose To describe an ophthalmic phenotype in children at relatively early stages of Wolfram syndrome. Methods Quantitative ophthalmic testing of visual acuity, color vision, automated visual field sensitivity, optic nerve pallor and cupping, and retinal nerve fiber layer (RNFL) thickness assessed by optical coherence tomography (OCT) was performed in 18 subjects 5-25 years of age. Subjects were also examined for presence or absence of afferent pupillary defects, cataracts, nystagmus, and strabismus. Results Subnormal visual acuity was detected in 89% of subjects, color vision deficits in 94%, visual field defects in 100%, optic disk pallor in 94%, abnormally large optic nerve cup:disk ratio in 33%, thinned RNFL in 100%, afferent pupillary defects in 61%, cataracts in 22%, nystagmus in 39%, and strabismus in 39% of subjects. RNFL thinning ( P P = 0.01), strabismus ( P = 0.04), and nystagmus ( P = 0.04) were associated with more severe disease using the Wolfram United Rating Scale. Conclusions Children and adolescents with Wolfram syndrome have multiple ophthalmic markers that correlate with overall disease severity. RNFL thickness measured by OCT may be the most reliable early marker.

Background: Anomalous retinal correspondence (ARC) is a neural adaptation to eye misalignment in which non-corresponding retinal points are linked in the visual cortex to provide binocular fusion. ARC within the striate cortex would require that horizontal neurons link right-eye and left-eye ocular dominance columns (ODCs) separated by a distance in the cortex proportional to the angle of strabismus. Two hypothetical mechanisms are possible: (1) The ODCs can be linked by axons of horizontal neurons that project monosynaptically from a right-eye to a left-eye ODC. The further apart the ODCs, the longer the axons; hence, axon length should be greater in subjects with strabismus than in healthy subjects (elongated axon, monosynaptic hypothesis). In this case, the clinical probability of developing ARC should be independent of the angle of strabismus, until an upper-limit angle of strabismus is reached equally to the maximal length of axons available to link nonadjacent ODCs, at which point an abrupt decline of ARC probability should be evident. (2) Alternatively, ODCs can be linked by a chain of horizontal neurons, the number of which increases as the distance among ODCs increases; axon length in subjects with strabismus would be expected to be the same as in healthy subjects (normal axon, polysynaptic hypothesis). In this case, the greater the angle of strabismus, the more horizontal neurons and synapses required for linkage, and the greater the probability of signal degradation. Thus, the clinical probability of developing ARC through a polysynaptic mechanism should be inversely proportional to the angle of strabismus. The purpose of this study was to test these 2 hypotheses neuroanatomically in primates and clinically in children. Methods: For the neuroanatomic portion of the study, biotinylated dextran amine was injected into ODCs of area V1 to label individual neurons. The length of the horizontal axons from these neurons was then compared in strabismic and normal monkeys. In the clinical portion of the study, the medical records of 192 children with strabismus were reviewed retrospectively. The angle of strabismus (prism cover test) and the presence of ARC (Bagolini striated lenses, Worth/Polaroid 4-dot) were recorded. Plots of the presence of ARC as a function of the angle of strabismus were obtained. Results: There was no significant difference in axon length between healthy (7. 02 ± 0. 83 mm) and strabismic monkeys (6. 60 ± 1. 07 mm) ( P = .16). In children with strabismus, ARC decreased as the angle of strabismus increased ( P Conclusions: The visual cortex adapts to strabismus by combining information from paired ODCs of opposite ocularity that, because of the eye misalignment, are nonadjacent and separated by abnormally long distances across the striate cortex. The cortex appears to achieve the linkage, not by elongating neuronal axons, but by using chains of neurons that have normal-length axons. The visual cortex is most successful stochastically at achieving this linkage (ie, developing ARC) when the gap that must be bridged is no greater than 4° to 5° (8–10 PD), or the retinotopic distance in the foveal visual field is spanned by 2 normal V1 neurons. (J AAPOS 2000;4:168–74)

Background: The probability of visual recovery in tumor-related optic neuropathy usually correlates with the severity and duration of optic pathway compromise. Recovery of visual acuity to normal levels is unexpected after profound loss of vision extending for a period of weeks and months. Methods: A 9-year-old girl who had neurosurgical resection of a craniopharyngioma compressing the optic chiasm and optic tract was followed up serially with neuroimaging and clinical examinations over a 6-year period. Results: Within 3 months of the diagnosis of craniopharyngioma, the girl's vision was reduced to no-light-perception blindness when she viewed with the more involved eye. The blindness correlated with an amaurotic (ie, >3.6 log unit) relative afferent pupillary defect and an absence of any response when tested with visual field perimetry. After more than a year of total blindness and cessation of all neurosurgical and radiation therapy, visual acuity recovered to a normal level (20/25), the afferent pupillary defect improved, and sensitivity in a portion of the temporal hemivisual field was restored. In the follow-up that has extended for 5 years from the time of recovery, stability of the restored vision has been documented. Conclusion: Children who have tumor-related loss of vision due to damage to the anterior visual pathways may be capable of recovery after intervals of blindness that would be considered irreversible in adults. The mechanism of the recovery in our patient may have been decompression-related restoration of axoplasmic flow, followed by gradual remyelination of visual fibers, which allowed reorganization of connections to the lateral geniculate nucleus to optimize synaptic transmission. (J AAPOS 1999;3:366-71)

Purpose: To determine how the duration of infantile strabismus influences the eventual eye alignment. Methods: Six infant macaques were fitted with prisms, which were removed after 3 weeks (wks.), 3 months (mos.), or 6 mos. Two control monkeys wore plano lenses. Eye alignment was measured using search coils. Results: Longer duration of infantile strabismus is correlated with a more severe misalignment. The strabismus in the 6-mos. group was 96% greater than that in the 3-mos. group, which, in turn, was 25 times greater than that in the 3-wks group (p < 0.05). Conclusions: Longer duration of infantile strabismus causes larger-angle esotropia. Early correction of infantile strabismus is supported.

Patients who have infantile strabismus exhibit a directional asymmetry of motion visually evoked potentials (MVEPs) recorded under conditions of monocular viewing. The majority of these patients also have latent nystagmus, raising the possibility that the MVEP asymmetry is an artifact of the nystagmus. To explore this issue, we correlated MVEPs and eye movements under conditions that eliminated or increased latent nystagmus.MVEPs and eye movements were recorded under conditions of monocular viewing in three adults who had combined infantile-onset strabismus and latent nystagmus. The subjects viewed vertically oriented grating stimuli that oscillated horizontally at temporal frequencies of 6.6 to 11.0 Hz by use of spatial frequencies of 1 to 3 cycles/degree. Quantitative eye movement recordings of latent nystagmus and horizontal pursuit/optokinetic nystagmus were also obtained.Eye movement recordings showed that the latent nystagmus was absent or markedly diminished when the viewing eye was in a 45-degree adducted position, whereas nystagmus velocity increased 10 to 40 times (to 2.2 to 4.5 degrees/second) when the viewing eye was in an abducted position (p0.05). MVEPs were abnormal (asymmetry indices0.40) when the viewing eye was in an adducted or abducted position of gaze. No correlation was found between the MVEP asymmetry index and the velocity of latent nystagmus.MVEP asymmetries in infantile strabismus remain robust under conditions that eliminate or greatly reduce the oscillations of latent nystagmus. MVEP asymmetries and ocular motor abnormalities both characterize infantile strabismus, but the ocular motor defects do not cause the MVEP asymmetries. The nasotemporal asymmetry of MVEPs and the nasotemporal asymmetry of pursuit and latent nystagmus are likely caused by deficits in related but separate binocular visual cortical circuits.

almology ing partial ventricuhite arrow nce of the Lymphocytic choriomeningitis virus (LCMV) dent-borne arenavirus that can cause conge fections similar to cytomegalovirus (CMV) plasmosis in liveborn infants. Congenital LC fection causes hydrocephalus, chorioretini neurodevelopmental abnormalities. For t son, it should be added to the list of co infections subsumed currently under the T acronym (toxoplasmosis, rubella, cytomega and herpes simplex). Only 49 cases of co LCMV infections have been described wo since 1955, but a lack of recognition of the may account for the low prevalence. Th oretinopathy of LCMV is noteworthy for features, which may resemble the choriore cunae of Aicardi syndrome. LCMV has been guishable from Aicardi syndrome by 2 find liveborn infants with LCMV may be boys whereas infants with Aicardi syndrome are g lethal in boys) and (2) infants with LCMV ported to have a normal corpus callosum, infants with Aicardi syndrome have callosa sis. We report here a boy with congenita infection, lacunar chorioretinopathy, and agenesis. Our findings indicate that screen congenital LCMV infection is advisable in with the features of Aicardi syndrome.

Background: Dacryocystitis in infants and older children is a serious complication of congenital or acquired nasolacrimal duct obstruction. To define the modes of presentation and treatment strategies of this disorder better, we reviewed the clinical courses of 54 children treated for dacryocystitis at St Louis Children's Hospital. Methods: Clinical, neuroradiologic, and laboratory data were collated for all cases of dacryocystitis treated from 1990 to 1995. Average follow up of the children in this consecutive series was 1.75 years (range, 4 months to 5 years). Results: Of the 54 patients, 36 (67%) had chronic lowgrade dacryocystitis, which was treated with nasolacrimal duct probing on an outpatient basis. The remaining 18 patients (33%) had acute dacryocystitis, which was treated with a combined medical/surgical strategy. Medical treatment consisted of hospital admission for administration of intravenous antibiotics followed by inpatient surgery, which varied according to the age of the patient and the clinical history: 1) Acute dacryocystitis in neonates was treated surgically by nasolacrimal duct probing and nasal endoscopy for excision of intranasal duct cyst; 2) Acute dacryocystitis with periorbital cellulitis was treated surgically by nasolacrimal duct probing; 3) Acute dacryocystitis due to facial trauma was treated surgically by dacryocystorhinostomy and stent placement; and 4) Acute dacryocystitis complicated by orbital abscess was treated by inferior orbitotomy for orbital abscess drainage, simultaneous nasolacrimal duct probing, and stent placement. Conclusion: Dacryocystitis in the pediatrie population may present in either chronic or acute forms. An effective and safe treatment for acute dacryocystitis is hospital admission, both for administering intravenous antibiotics and monitoring to rule out orbital cellulitis or abscess formation. Intravenous antibiotic therapy is followed within a day or two by surgery tailored to the clinical history. In the majority of both chronic and acute cases, nasolacrimal duct probing appears to be an effective treatment strategy.

PURPOSE Optokinetic eye movements stabilize gaze by tracking motion of the visual scene during sustained movement of a creature's body. The purpose of this study was to describe vertical and horizontal optokinetic nystagmus (OKN) in nonhuman primates (NHPs) with normal binocular vision, and to compare their responses to NHPs with binocular maldevelopment induced by prism-rearing. METHODS Optical strabismus was created in infant macaques (n = 6) by fitting them with prism goggles. The goggles were removed after 3, 6, 9, or 12 weeks to determine the effects of increasing durations of binocular noncorrespondence. Infant NHPs (n = 2) reared wearing plano goggles served as controls. OKN was evoked by horizontal or vertical stripe motion. Eye movements were recorded by using binocular search coils. RESULTS NHPs reared in early infancy under conditions of binocular noncorrespondence for durations of 6 weeks or longer had horizontal OKN responses biased directionally in favor of nasalward motion. NHPs reared with prisms for any duration had vertical OKN responses more biased than normal NHPs in favor of upward motion. Diagonal "crosstalk" during horizontal or vertical OKN (vertical slow phases during horizontal stimulus motion, and vice versa) was present to some degree in all NHPs. However, crosstalk-upward during horizontal OKN and nasalward during vertical OKN-was most pronounced in NHPs reared with prism for durations long enough to induce a permanent esotropic strabismus (longer than 3 weeks). CONCLUSIONS With fusion maldevelopment, the OKN pathways retain a nasalward and upward bias. During forward locomotion, optic flow excites temporalward and downward visual motion in each eye. The OKN biases would act in counterbalance. The biases attenuate with emergence of fusion, but may persist and crosstalk when fusion is impeded.

Purpose: Among the options for surgical management of pediatric glaucoma, destruction of the ciliary body reduces aqueous production and, consequently, intraocular pressure (IOP). Compared to more invasive filtering and shunt procedures, cyclodestruction is an attractive option for control of IOP in pediatric glaucomas. Methods: The relative reduction in IOP, duration of effect, and comparable safety and efficacy of transscleral cyclophotocoagulation (TSCP) and endoscopic cyclophotocoagulation (ECP) in pediatric patients with glaucoma was studied in this retrospective chart review. Results: A reduction in IOP of 28.6% and 33.2% with TSCP and ECP, respectively, was found. Eyes treated with ECP underwent an average of 3.24 cyclodestructive procedures; eyes treated with TSCP underwent an average of 2.29 cyclodestructive treatments. These differences were not statistically significant. A final success rate of 67.6% after TSCP and 62% after ECP failed to significantly differ between the two groups. Consequently, two-thirds of the patients were able to avoid penetrating surgery and the associated risks after one or more cyclodestructive procedures. Conclusions: TSCP and ECP are safe, effective, and comparable treatments for pediatric glaucomas. The results suggest that TSCP and ECP may be considered first-line therapy to achieve control of IOP in all forms of pediatric glaucoma. [J Pediatr Ophthalmol Strabismus 2014;51(2):120–127.]

To determine whether demographic or clinical factors are associated with the outcome of office-based nasolacrimal duct probing for the treatment of congenital nasolacrimal duct obstruction (NLDO).In two multicenter prospective studies, 384 eyes of 304 children aged 6 to15 months with NLDO underwent a nasolacrimal duct probing performed in the office using topical anesthesia. Treatment success, defined as no clinical signs of NLDO (epiphora, increased tear lake, or mucous discharge) and no reoperation, was assessed 1 month after probing in one study and 6 months after probing in the other study. Data from both studies were pooled to evaluate associations between baseline characteristics and treatment success.Office probing was successful in 75% of eyes overall (95% CI, 70%-80%). The procedure was less successful in eyes of children with bilateral NLDO compared with unilateral NLDO (63% vs 80%; relative risk = 0.78 [95% CI, 0.66-0.92]) and in eyes that had 2 or 3 clinical signs of NLDO compared with one (71% vs 83%; relative risk = 0.88 [95% CI, 0.81-0.96]). Treatment success did not appear to be related to age, specific clinical signs of NLDO, prior treatment, or research study.Performing nasolacrimal duct probing in the office successfully treats NLDO in the majority of cases in children aged 6 to15 months. The success rate is lower with bilateral disease or when more than one clinical sign of NLDO is present.

* BACKGROUND AND OBJECTIVE: To determine the effectiveness of strabismus surgery as an alternative treatment for high accommodative convergence/accommodation (AC/A) ratio (nonrefractive accommodative) esotropia. * PATIENTS AND METHODS: Twenty-three consecutive children with an AC/A ratio of 5: 1 or greater and a distance-near disparity of 10 prism diopters (PD) or more were studied in a prospective design. Each patient was treated by recession of both medial rectus muscles for the full amount of esotropìa measured at near fixation through the fiill distance hyperopic correction. The magnitude of eso deviation, the AC/A ratio, and the grade of binocularity were measured before and a minimum of 4 months after strabismus surgery. * RESULTS: Surgery reduced the mean preoperative eso deviation at near fixation by a factor of 4 (from a mean of 37 PD to a mean of 9 PD). Surgery eliminated the need for bifocals in 96% of the patients. The grade of binocular fusion or stereopsis improved in 70% of the patients, remained unchanged in 26%, and was degraded in 4%. Three (13%) of the children became exophoric; none of the children were exotropic. * CONCLUSION: These results demonstrate that strabismus surgery is an effective alternative to the long-term use of bifocals in the treatment of high AC/A ratio esotropia. Surgery may be more effective than bifocals in restoring normal stereopsis and binocular fusion. [Ophthalmic Surg Lasers 1996;27:342-348.]

Purpose: To determine whether macaque monkeys who had onset of natural, alternating esotropia in early infancy have the eye movement abnormalities and motion visually evoked potential (VEP) abnormalities observed in human infantile esotropes. Methods: Recordings were obtained of horizontal pursuit eye movements and fixation nystagmus under conditions of monocular viewing. Motion VEPs in response to horizontal motion were recorded with the animals sedated to reduce the possibility of eye movement artifact. Results: The strabismic monkeys had a naso-temporal asymmetry of horizontal pursuit favoring nasally directed motion when viewing with either eye. When fixating stationary targets, latent nystagmus was apparent; the eyes drifted conjugately and the slow phase of the nystagmus was always nasally directed with respect to the fixating eye. Motion VEPs were characterized by a strong horizontal asymmetry with the directional bias inverted approximately 180 deg in the two eyes. These eye movement and motion VEP asymmetries were not observed in a normal macaque. Conclusions: Macaque monkeys who have infantile esotropia possess asymmetries of horizontal pursuit and motion VEPs like those documented in strabismic humans. Macaques with infantile esotropia appear to be an appropriate animal model for study of neural mechanisms in strabismus.

Background To investigate the structural basis for functional deficits in infantile strabismus we used a neuroanatomic tracer and a histochemical label to examine the primary visual cortex (area V1) of adult esotropic macaque monkeys. Animals and methods The animals had developed natural esotropia in the first months of life, alternated fixation, and exhibited the ocular motor signs that typify strabismus with onset in infancy. After behavioral and VEP testing, ocular dominance columns (ODCs) in V1 were injected with a neuronal traser and labeled for cytochrome oxidase activity. Results The strabismic monkeys showed striking deficits in binocular luminance visually-evoked potentials (VEPs) and characteristic directional asymmetries in motion VEPs. Binocular horizontal connections between ODCs were reduced an average of 50-60% in strabismic as compared to normal monkeys. ODCs also showed unequal metabolic activity with contralaterally (i.e. nasal retina) driven ODCS showing great-er activity in each V1. Discussion and conclusion The maldevelopment of connections in upper V1 layers correlates with the abnormalities in binocular and motion VEPs. The unequal metabolic activity suggests interocular suppression. These results provide the first neuroanatomic evidence for cerebral cortex maldevelopments in natural infantile esotropia.

Tracking of large visual targets by normal neonates and by infants with visual cortex damage has led to the suggestion that infants use subcortical vision to guide visuomotor behaviors. To test this assertion, the author recorded eye movements in a five-month-old infant who became cor-tically-blind following an anoxic episode. Use of a full-field, high-contrast visual stimulus failed to elicit any evidence of optokinetic nystagmus. The findings in this infant lead to the conclusion that retinotectal visual inputs are incapable of driving eye movements in the young human in the absence of cortical vision.

A small number of children who develop disconjugate nystagmus, torticollis, and head titubation (spasmus nutans) have been found to have optic chiasm or third ventricle gliomas. However, the prevalence of glioma or other developmental abnormalities in this disorder is unknown because no large series of spasmus nutans cases has previously been reported. A reviewer of the records of 67 consecutive children initially diagnosed with spasmus nutans and followed for an average of 3.3 years at the St Louis Children's Hospital revealed the following: 61% had a history of prematurity, developmental delay, or other systemic abnormality; strabismus, most commonly infantile esotropia, developed in 55%; 43% had neuroimaging studies; and 0% had evidence of a glioma or showed signs of tumor on follow-up examinations. From this consecutive patient series, we estimate the prevalence of tumor in spasmus nutans to be less than 1.4%. Without other evidence of an intracranial mass lesion, neuroimaging of infants initially diagnosed with spasmus nutans may not be immediately warranted.

To explore the structural basis for visuomotor deficits in infantile esotropia, we examined binocular connections and metabolic activity in the primary visual cortex of two strabismic macaque monkeys. The animals were documented to have onset of natural esotropia in early infancy. Behavioral testing showed that the animals had normal visual acuity in both eyes and the ocular motor deficits that characterize strabismus with onset in infancy. The neuronal tracer substance biotinylated-dextran-amine was injected into ocular dominance columns (ODC) in area V-1 (striate cortex), revealing a paucity of binocular connections between right-eye and left-eye ODCs. The metabolic label cytochrome-oxidase was used to stain neighboring right-eye and left-eye ODCs, revealing inequalities in metabolic activity compatible with interocular suppression. These results show that infantile esotropes have abnormalities of visual cortex structure that correlate with abnormalities in binocular behaviors.