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To report long-term ophthalmic findings in Wolfram syndrome, including rates of visual decline, macular thinning, retinal nerve fiber layer (RNFL) thinning, and outer plexiform layer (OPL) lamination.Single-center, cohort study.A total of 38 participants were studied, who underwent a complete ophthalmic examination as well as optical coherence tomography imaging of the macula and nerve on an annual basis. Linear mixed-effects models for longitudinal data were used to examine both fixed and random effects related to visual acuity and optic nerve quadrants of RNFL and macula thickness.Participants completed a mean of 6.44 years of follow-up (range 2-10 years). Visual acuity declined over time in all participants, with a mean slope of 0.059 logMAR/y (95% CI = 0.07-0.05 logMAR/y), although nearly 25% of participants experienced more rapid visual decline. RNFL thickness decreased in superior, inferior, and nasal quadrants (β = -0.5 µm/y, -0.98 µm/y, -0.28 µm/y, respectively). OPL lamination was noted in 3 study participants, 2 of whom had autosomal dominant mutations.Our study describes the longest and largest natural history study of visual acuity decline and retinal morphometry in Wolfram syndrome to date. Results suggest that there are slower and faster progressing subgroups and that OPL lamination is present in some individuals with this disease.

To determine whether rates of strabismus and associated visuomotor deficits differed among children with different severities of periventricular leukomalacia (PVL).Retrospective, case-control study.Brain magnetic resonance images (MRI) obtained from 98 children aged ≥2 years were analyzed using a standardized scoring system: 67 of 98 had PVL (mean GA 31 weeks) and 31 of 98 did not have PVL (mean GA 29 weeks). Severity of PVL was scored as degree of damage to the posterior optic radiations and the splenium of the corpus callosum on MRI. Ophthalmologic examination data were collated to assess the prevalence of visuomotor deficits and the relationship to PVL severity (grades 1-3, mild to severe).Infantile strabismus was documented in 61% of children with mild, 74% with moderate, and 88% with severe PVL (esotropia: exotropia ratio 3.5:1). Associated ocular motor deficits also increased systematically with PVL severity: latent ("fusion maldevelopment") nystagmus (20%, 47%, and 40%, respectively), dissociated vertical deviation (13%, 28%, and 30%), and nasotemporal pursuit/optokinetic nystagmus asymmetry (23%, 38%, and 54%). Additionally, the prevalence of retrograde optic neuropathy increased with PVL severity (5%, 26%, and 38%). The prevalence of each of these signs was substantially lower in children who had no PVL.Children who suffer PVL are likely to develop the deficits of the infantile strabismus complex. The deficits tend to increase systematically as a function of PVL severity. These findings provide evidence that infantile strabismus is linked to perinatal damage to cerebral vergence and gaze pathways.

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.

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.

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.

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)

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.

Caudal regression syndrome (CRS) is a rare embryopathy characterized by maldevelopment of the vertebrae, visceral organs, and lower extremities. Patients with CRS have deformities of the sacrococcygeal vertebrae and develop neurogenic bladder from dysfunction of the sacral nerve root. 1-3 Additional anomalies include congenital heart defects, intestinal malrotation, agenesis of the genitourinary system, imperforate anus, and malformation of the lower limbs. 1-3 Ocular findings described in the nonophthalmic literature have been confined to hypertelorism, epibulbar dermoid, and anophthalmia. 3,4 We report a patient with CRS who presented with infantile glaucoma and punctal atresia. A computerized search of the medical literature showed no previous reports of CRS in ophthalmic journals and no previous reports of an association with glaucoma or nasolacrimal anomalies. CASE REPORT A 10-week-old infant presented with a 1-week history of bilateral epiphora and photophobia. The patient had neonatal documentation of lumbosacral vertebral anomalies, atrioseptal defect, bifid clitoris, imperforate anus, dislocated hip, and syndactyly of both feet (Fig 1). At 9 weeks, she underwent repair of a malrotated intestine and vesicostomy for neurogenic bladder. The mother’s health and prenatal care were excellent, and the pregnancy and delivery were unremarkable. Family history was negative for parental consanguinity or hereditary ocular or systemic disease. The infant was noted to have hypertelorism, and lower lid punctal agenesis. Both corneas exhibited diffuse stromal thickening, which was more severe in the left eye. Horizontal corneal diameters measured 11.5 mm in the right eye and 12.5 mm in the left eye. Intraocular pressures (IOP) measured serially using a hand-held applanation tonometer were 30 mm Hg in each eye. Portable slit-lamp examination showed no evidence of anterior segment dysgenesis. Fundoscopic examination showed cup-to-disc ratios of 0.5 (right eye) and 0.6 (left eye). The next day, an examination was performed under anesthesia to confirm the diagnosis of glaucoma, after which 90° goniotomies were performed first in the right eye and 1 week later in the left eye. The corneal edema progressively resolved during the next month, and the cup-to-disc ratios decreased to 0.3 in both eyes. At 14 months, bilateral punctoplasty and nasolacrimal duct reconstruction were carried out to repair the punctal atresia. At 3 years, repeat bilateral goniotomies were performed

Purpose To assess the effect of a pediatric eyelid speculum on the measurement of intraocular pressure (IOP) in children. Design Observational case series. Methods Intraocular pressure measurements were performed during examination under anesthesia immediately after induction. The measurements were made with and without a pediatric eyelid speculum in place. Results Intraocular pressure measurements were made in 77 eyes of 45 consecutive children. The mean IOP without the speculum was 16.33 mm Hg in the right eye and 16.55 mm Hg in the left eye. With the lid speculum in place the mean IOP was 20.26 mm Hg in the right eye and 20.05 mm Hg in the left eye ( P Conclusions The presence of an eyelid speculum raised IOP an average of 4 mm Hg. This effect should be considered when interpreting IOP measurements in children.

sotemporal Pursuit Asymmetries in Strabismic Macaque Monkeys Aasim Hasany, Agnes Wong MD, Paul Foeller MSc, Dolores Bradley PhD, Lawrence Tychsen MD; Washington University School of Medicine, St. Louis, MO Purpose: Infantile esotropia is associated with nasotemporal asymmetries of pursuit during monocular viewing. The purpose of this study was to determine how the duration of binocular decorrelation, which can be strictly controlled in primates, influences directional pursuit asymmetries. Methods: Optical strabismus was created in seven infant macaques by fitting them with prism goggles on DOL 1. The goggles were removed after 3 weeks (n 2), 3 months (n 1), or 6 months (n 2), emulating surgical repair of strabismus in humans at 3, 12, and 24 months of age, respectively. Two control monkeys wore plano lenses. After the goggles were removed, horizontal pursuit was recorded using search coils, evoked by “stepramp” target motion. A nasal bias index (NBI) was calculated as (Sn St)/(Sn St), where Sn is nasally directed and St is temporally directed mean eye speed. Results: Each animal in the 3and 6-month-duration groups showed asymmetries of pursuit at both target speeds. The longer the duration, the more severe the asymmetry: for 15 deg/s targets, the NBI in the 6-month group was 5 greater than that in the 3-month group, and 30 X greater than that in the 3-week group (ANOVA, P 0.03). For 30 deg/s target motion, the NBI in the 6-month-duration group was 4 greater than that in the 3-month group, and 12 greater than that in the 3-week group (ANOVA, P 0.05). Controls had symmetric pursuit. Conclusions: Early correction of strabismus (ie, elimination of binocular decorrelation) prevents maldevelopment of the pursuit system. These findings indicate that early correction of infantile strabismus is beneficial for brain development.