Evaluation of Strabocheck® as an objective measurement method of comitant horizontal strabismus in children undergoing surgery.

The gold standard of angle measurement is the prism alternating cover test (PCT). This method requires cooperation from the child, experience, and presents a significant inter-observer variability. Strabocheck®(SK) is a new simple tool for objective and semi-automated angle measurement. Our purpose is to evaluate Strabocheck® in children undergoing surgery for comitant horizontal strabismus. The study population was divided into 3 groups: infantile esotropia, partially accommodative esotropia and intermittent exotropia. The primary endpoint was the agreement between Strabocheck®and the PCT. A total of 44 children were included prospectively. The correlation between the angle measured by the PCT and the angle measured by SK was strong (R = 0.87). The mean absolute difference in the angle measured by the two methods was Δ = 11.9+/- 9.8 diopters. The Bland-Altman plot shows a 95% interval limit between -30.0 [-34.4; -25.6] and 31.0 [26.7; 35.4] diopters. SK is an interesting tool to evaluate the angle of strabismus in children. However, the residual discordance between PCT and SK leads us to question the real value of the angle, which can only be approximated. A better clinical experience of this new tool in relation to the clinical condition and the PCT will allow to have a more accurate idea on the true angle and will probably help the surgeon to adapt this procedure.

Amblyopia: A review of unmet needs, current treatment options, and emerging therapies.

Amblyopia is a global public health issue with extensive, multifaceted impacts on vision and quality of life (QoL) for both patients and families. Geographical variation exists in the management of amblyopia, with traditional mainstay treatments, optical correction, and fellow eye occlusion most successful when implemented at an early age. In recent years, however, studies demonstrating meaningful improvements in older children and adults have challenged the concept of a complete loss of visual processing plasticity beyond the critical period of visual development, with growing evidence supporting the potential efficacy of emerging, more engaging, binocular therapies in both adults and children. Binocular approaches aim to restore deficits in amblyopia that extend beyond monocular visual acuity impairment, including binocular fusion and visuomotor skills. In view of this, incorporating outcome measures that evaluate the visual performance and functional ability of individuals with amblyopia will provide a clearer understanding of the effect of amblyopia on QoL and a more comprehensive evaluation of amblyopia therapies.

Turner syndrome: French National Diagnosis and Care Protocol (NDCP; National Diagnosis and Care Protocol).

Turner syndrome (TS; ORPHA 881) is a rare condition in which all or part of one X chromosome is absent from some or all cells. It affects approximately one in every 1/2500 liveborn girls. The most frequently observed karyotypes are 45,X (40-50%) and the 45,X/46,XX mosaic karyotype (15-25%). Karyotypes with an X isochromosome (45,X/46,isoXq or 45,X/46,isoXp), a Y chromosome, X ring chromosome or deletions of the X chromosome are less frequent. The objective of the French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins) is to provide health professionals with information about the optimal management and care for patients, based on a critical literature review and multidisciplinary expert consensus. The PNDS, written by members of the French National Reference Center for Rare Growth and Developmental Endocrine disorders, is available from the French Health Authority website. Turner Syndrome is associated with several phenotypic conditions and a higher risk of comorbidity. The most frequently reported features are growth retardation with short adult stature and gonadal dysgenesis. TS may be associated with various congenital (heart and kidney) or acquired diseases (autoimmune thyroid disease, celiac disease, hearing loss, overweight/obesity, glucose intolerance/type 2 diabetes, dyslipidemia, cardiovascular complications and liver dysfunction). Most of the clinical traits of TS are due to the haploinsufficiency of various genes on the X chromosome, particularly those in the pseudoautosomal regions (PAR 1 and PAR 2), which normally escape the physiological process of X inactivation, although other regions may also be implicated. The management of patients with TS requires collaboration between several healthcare providers. The attending physician, in collaboration with the national care network, will ensure that the patient receives optimal care through regular follow-up and screening. The various elements of this PNDS are designed to provide such support.

Evaluation of the Spot Vision Screener for children with limited access to ocular health care.

PURPOSE: To compare the sensitivity, specificity, and referral rate of the Spot Vision Screener (Welch Allyn Inc, Skaneateles Falls, NY) with the gold standard cycloplegic measurements acquired using the Retinomax in a population of underprivileged children and teenagers with limited access to medical care. METHODS: Children were recruited for the study by social workers in the vicinity of Robert Debre Hospital, Paris, France. Refractive errors (hyperopia of ≥ +2.00 D spherical equivalent [SE]; myopia of ≤ -0.50 D SE; astigmatism of ≥1.00 D between the two main meridians; anisometropia of ≥1.00 D SE difference between eyes) were assessed using the Spot Vision Screener and the Retinomax. Sensitivity (true positive rate), specificity (true negative rate), and referral rate of this Spot Vision screening program were evaluated. RESULTS: A total of 82 eyes of 41 subjects (19 males) were included; mean age was 126 months of age (range, 48-246). The sensitivity of the Spot Vision Screener for the detection of refractive errors was 82.35%; specificity was 91.67%. The sensitivity of the Spot Vision Screener to detect hyperopia, myopia, astigmatism, and anisometropia was 27.27%, 84.61%, 78.57%, and 66.67%, respectively. Its specificity to detect hyperopia, myopia, astigmatism, and anisometropia was 100%, 98.55%, 89.71% and 94.29%, respectively. CONCLUSIONS: The specificity of the Spot Vision Screener to detect refractive errors was found to be relatively high (>90%). However, its low sensitivity for hyperopia seems to remain a major limitation of the device, because hyperopia is particularly important to detect in children given its high prevalence and possible adverse consequences. Global programs using cycloplegic measurements should be considered an alternative.

Beyond Rehabilitation of Acuity, Ocular Alignment, and Binocularity in Infantile Strabismus.

Infantile strabismus impairs the perception of all attributes of the visual scene. High spatial frequency components are no longer visible, leading to amblyopia. Binocularity is altered, leading to the loss of stereopsis. Spatial perception is impaired as well as detection of vertical orientation, the fastest movements, directions of movement, the highest contrasts and colors. Infantile strabismus also affects other vision-dependent processes such as control of postural stability. But presently, rehabilitative therapies for infantile strabismus by ophthalmologists, orthoptists and optometrists are restricted to preventing or curing amblyopia of the deviated eye, aligning the eyes and, whenever possible, preserving or restoring binocular vision during the critical period of development, i.e., before ~10 years of age. All the other impairments are thus ignored; whether they may recover after strabismus treatment even remains unknown. We argue here that medical and paramedical professionals may extend their present treatments of the perceptual losses associated with infantile strabismus. This hypothesis is based on findings from fundamental research on visual system organization of higher mammals in particular at the cortical level. In strabismic subjects (as in normal-seeing ones), information about all of the visual attributes converge, interact and are thus inter-dependent at multiple levels of encoding ranging from the single neuron to neuronal assemblies in visual cortex. Thus if the perception of one attribute is restored this may help to rehabilitate the perception of other attributes. Concomitantly, vision-dependent processes may also improve. This could occur spontaneously, but still should be assessed and validated. If not, medical and paramedical staff, in collaboration with neuroscientists, will have to break new ground in the field of therapies to help reorganize brain circuitry and promote more comprehensive functional recovery. Findings from fundamental research studies in both young and adult patients already support our hypothesis and are reviewed here. For example, presenting different contrasts to each eye of a strabismic patient during training sessions facilitates recovery of acuity in the amblyopic eye as well as of 3D perception. Recent data also demonstrate that visual recoveries in strabismic subjects improve postural stability. These findings form the basis for a roadmap for future research and clinical development to extend presently applied rehabilitative therapies for infantile strabismus.

Importance of Proprioceptive Information for Postural Control in Children with Strabismus before and after Strabismus Surgery.

The objective of this study is to examine the role of proprioception in postural balance in children with strabismus before and after realignment of their visual axes by eye surgery. Postural recordings were made with the TechnoConcept® force platform in 23 children. Several conditions were studied, whether the subjects had both eyes open, or either the dominant or the non-dominant eye open, without and with foam pads of 4 mm underfoot. Recordings were performed before and after strabismus surgery. The surface area, the length and the mean speed of the center of pressure (CoP) were analyzed. Before strabismus surgery, all children showed better stability with both eyes open with respect to the condition with the non-dominant eye open; furthermore postural stability improved in the presence of foam pads. After surgery, the surface area of CoP decreased significantly, especially in the non-dominant eye viewing condition. We suggest that strabismic children use mainly proprioceptive information in order to control their posture, but also visual inputs, which are important for obtaining a good postural stability. The alignment of the visual axes after surgery provides enhanced postural stability, suggesting, again the major role of visual inputs in the control of posture. Proprioceptive plasticity after strabismus surgery may allow better visual rehabilitation.

Immaturity of Visual Fixations in Dyslexic Children.

To our knowledge, behavioral studies recording visual fixations abilities in dyslexic children are scarce. The object of this article is to explore further the visual fixation ability in dyslexics compared to chronological age-matched and reading age-matched non-dyslexic children. Fifty-five dyslexic children from 7 to 14 years old, 55 chronological age-matched non-dyslexic children and 55 reading age-matched non-dyslexic children participated to this study. Eye movements from both eyes were recorded horizontally and vertically by a video-oculography system (EyeBrain(®) T2). The fixation task consisted in fixating a white-filled circle appearing in the center of the screen for 30 s. Results showed that dyslexic children produced a significantly higher number of unwanted saccades than both groups of non-dyslexic children. Moreover, the number of unwanted saccades significantly decreased with age in both groups of non-dyslexic children, but not in dyslexics. Furthermore, dyslexics made more saccades during the last 15 s of fixation period with respect to both groups of non-dyslexic children. Such poor visual fixation capability in dyslexic children could be due to impaired attention abilities, as well as to an immaturity of the cortical areas controlling the fixation system.

Importance of visual inputs quality for postural stability in strabismic children.

OBJECTIVE: The first goal of the present study is to analyze the postural control in strabismic children in four different visual conditions (eyes open, eyes closed and monocular viewing with squint eye and non-squint eye). Secondly, we wish to explore the effect of strabismus surgery in postural control. METHOD: Postural stability was recorded with a platform (TechnoConcept) in 23 strabismic children aged from 4.4 to 14.8 years old (mean age: 8.4 years); 12 of these children were also examined at least two months after strabismus surgery. We analyzed the surface, the length, and the mean speed of the Center of Pressure displacement (CdP), and we calculated the Romberg's Quotient (that is for each postural parameter the ratio between eyes closed relative to eyes open). RESULTS: Strabismic children are significantly more stable when they can use their visual information to control their posture. Also, postural stability was better when the non-squint eye was viewing. For the first time, we observed the important role of vision (by calculating Romberg's Quotient) in strabismic children with binocular vision in comparison of strabismic children without binocular vision. Finally, we found that eye surgery improves postural stability. CONCLUSION: Strabismic children use visual inputs to control their posture. Moreover, binocular vision plays an important role in postural control. Strabismus surgery improves postural stability. SIGNIFICANCE: Visual inputs from the non-squint eye and binocular vision are important to control stability.

Sulfonylurea Therapy Benefits Neurological and Psychomotor Functions in Patients With Neonatal Diabetes Owing to Potassium Channel Mutations.

OBJECTIVE: Neonatal diabetes secondary to mutations in potassium-channel subunits is a rare disease but constitutes a paradigm for personalized genetics-based medicine, as replacing the historical treatment with insulin injections with oral sulfonylurea (SU) therapy has been proven beneficial. SU receptors are widely expressed in the brain, and we therefore evaluated potential effects of SU on neurodevelopmental parameters, which are known to be unresponsive to insulin. RESEARCH DESIGN AND METHODS: We conducted a prospective single-center study. Nineteen patients (15 boys aged 0.1-18.5 years) were switched from insulin to SU therapy. MRI was performed at baseline. Before and 6 or 12 months after the switch, patients underwent quantitative neurological and developmental assessments and electrophysiological nerve and muscle testing. RESULTS: At baseline, hypotonia, deficiencies in gesture conception or realization, and attention disorders were common. SU improved HbA1c levels (median change -1.55% [range -3.8 to 0.1]; P < 0.0001), intelligence scores, hypotonia (in 12 of 15 patients), visual attention deficits (in 10 of 13 patients), gross and fine motor skills (in all patients younger than 4 years old), and gesture conception and realization (in 5 of 8 older patients). Electrophysiological muscle and nerve tests were normal. Cerebral MRI at baseline showed lesions in 12 patients, suggesting that the impairments were central in origin. CONCLUSIONS: SU therapy in neonatal diabetes secondary to mutations in potassium-channel subunits produces measurable improvements in neuropsychomotor impairments, which are greater in younger patients. An early genetic diagnosis should always be made, allowing for a rapid switch to SU.

Spatial and temporal analyses of posture in strabismic children.

PURPOSE: To analyse postural performances of strabismic children, both in the spatial and the temporal domains, by wavelet transformation, comparing both stable and unstable situations. METHODS: Twenty-six strabismic children aged from 4 to 11 years old and 26 age-matched normal children participated in the study. Postural performances were evaluated using the Framiral® platform. Posture was recorded in the following conditions: eyes open fixating a target and eyes closed on stable and unstable platforms. RESULTS: For both strabismic and non-strabismic children, the surface and the mean velocity of the center of pressure (CoP) were significantly larger in the eyes closed on unstable platform condition, but this was much more pronounced in case of strabismus. Spectral power index and cancelling time were also found to be altered in strabismic children compared to non-strabismic children. CONCLUSIONS: This data demonstrates poor postural stability for both groups on an unstable platform with the eyes closed. However, strabismic children had significantly worse performance than non-strabismic children. Strabismic children also engage more energy to stabilize their posture by using visuo-vestibular sensory inputs to compensate their altered vision due to strabismus, in comparison to non-strabismic children.

Spatial and temporal analysis of postural control in dyslexic children.

OBJECTIVE: The aim of this study is to examine postural control of dyslexic children using both spatial and temporal analysis. METHODS: Thirty dyslexic (mean age 9.7±0.3years) and thirty non-dyslexic age-matched children participated in the study. Postural stability was evaluated using Multitest Equilibre from Framiral®. Posture was recorded in the following conditions: eyes open fixating a target (EO) and eyes closed (EC) on stable (-S-) and unstable (-U-) platforms. RESULTS: The findings of this study showed poor postural stability in dyslexic children with respect to the non-dyslexic children group, as demonstrated by both spatial and temporal analysis. In both groups of children postural control depends on the condition, and improves when the eyes are open on a stable platform. Dyslexic children have spectral power indices that are higher than in non-dyslexic children and they showed a shorter cancelling time. CONCLUSION: Poor postural control in dyslexic children could be due to a deficit in using sensory information most likely caused by impairment in cerebellar activity. SIGNIFICANCE: The reliability of brain activation patterns, namely in using sensory input and cerebellar activity may explain the deficit in postural control in dyslexic children.

The influence of oculomotor tasks on postural control in dyslexic children.

Dual task is known to affect postural stability in children. We explored the effect of visual tasks on postural control in thirty dyslexic children. A selected group of thirty chronological age-matched non-dyslexic children (mean age: 9.92 ± 0.35 years) and a group of thirty reading age-matched non-dyslexic children (mean reading age: 7.90 ± 0.25 years) were chosen for comparison. All children underwent ophthalmologic and optometric evaluation. Eye movements were recorded by a video-oculography system (EyeBrain® T2) and postural sway was recorded simultaneously by a force platform (TechnoConept®). All children performed fixations, pursuits, pro- and anti-saccades tasks. Dyslexic children showed significantly poor near fusional vergence ranges (convergence and divergence) with respect to the non-dyslexic children groups. During the postural task, quality of fixation and anti-saccade performance in dyslexic children were significantly worse compared to the two non-dyslexic children groups. In contrast, the number of catch-up saccades during pursuits and the latency of pro- and anti-saccades were similar in the three groups of children examined. Concerning postural quality, dyslexic children were more unstable than chronological age-matched non-dyslexic children group. For all three groups of children tested we also observed that executing saccades (pro- and anti-saccades) reduced postural values significantly in comparison with fixation and pursuit tasks. The impairment in convergence and divergence fusional capabilities could be due to an immaturity in cortical structures controlling the vergence system. The poor oculomotor performance reported in dyslexic children suggested a deficit in allocating visual attention and their postural instability observed is in line with the cerebellar impairment previously reported in dyslexic children. Finally, pro- or anti-saccades reduce postural values compared to fixation and pursuit tasks in all groups of children tested, suggesting a different influence of visual tasks on postural control according to their attentional demand.

Binocular saccade coordination in reading and visual search: a developmental study in typical reader and dyslexic children.

Studies dealing with developmental aspects of binocular eye movement behavior during reading are scarce. In this study we have explored binocular strategies during reading and visual search tasks in a large population of dyslexic and typical readers. Binocular eye movements were recorded using a video-oculography system in 43 dyslexic children (aged 8-13) and in a group of 42 age-matched typical readers. The main findings are: (i) ocular motor characteristics of dyslexic children are impaired in comparison to those reported in typical children in reading task; (ii) a developmental effect exists in reading in control children, in dyslexic children the effect of development was observed only on fixation durations; and (iii) ocular motor behavior in the visual search tasks is similar for dyslexic children and for typical readers, except for the disconjugacy during and after the saccade: dyslexic children are impaired in comparison to typical children. Data reported here confirms and expands previous studies on children's reading. Both reading skills and binocular saccades coordination improve with age in typical readers. The atypical eye movement's patterns observed in dyslexic children suggest a deficiency in the visual attentional processing as well as an impairment of the ocular motor saccade and vergence systems interaction.

Origins of strabismus and loss of binocular vision.

Strabismus is a frequent ocular disorder that develops early in life in humans. As a general rule, it is characterized by a misalignment of the visual axes which most often appears during the critical period of visual development. However other characteristics of strabismus may vary greatly among subjects, for example, being convergent or divergent, horizontal or vertical, with variable angles of deviation. Binocular vision may also vary greatly. Our main goal here is to develop the idea that such "polymorphy" reflects a wide variety in the possible origins of strabismus. We propose that strabismus must be considered as possibly resulting from abnormal genetic and/or acquired factors, anatomical and/or functional abnormalities, in the sensory and/or the motor systems, both peripherally and/or in the brain itself. We shall particularly develop the possible "central" origins of strabismus. Indeed, we are convinced that it is time now to open this "black box" in order to move forward. All of this will be developed on the basis of both presently available data in literature (including most recent data) and our own experience. Both data in biology and medicine will be referred to. Our conclusions will hopefully help ophthalmologists to better understand strabismus and to develop new therapeutic strategies in the future. Presently, physicians eliminate or limit the negative effects of such pathology both on the development of the visual system and visual perception through the use of optical correction and, in some cases, extraocular muscle surgery. To better circumscribe the problem of the origins of strabismus, including at a cerebral level, may improve its management, in particular with respect to binocular vision, through innovating tools by treating the pathology at the source.

Isolated enophthalmos: an uncommon gateway to orbital tumors in pediatrics: 9 month-old female presenting with isolated enophthalmos as the unique sign of a metastatic orbital tumor: a case report.

BACKGROUND: If extra-axial proptosis is by far the most common symptom of infantile malignant orbital tumors, enophthalmos is a rare and undocumented sign. We report the first case of a pediatric metastatic orbital tumor revealed by enophthalmos alone. CASE PRESENTATION: A 9-month-old girl was diagnosed with isolated right-sided enophthalmos. An orbital tumor was suspected and computed tomography undertaken showing osteolysis and periosteal reaction of orbital walls, malar bones and zygomatic arches. A Thoracic- abdominal CT scan confirmed a stage-4 neuroblastoma. CONCLUSION: Enophthalmos can be the sole symptom of an orbital tumor and should lead to immediate imaging assessment. This association is not well known in pediatrics but is relevant to insure the best prognosis.

Vertical saccades in dyslexic children.

Vertical saccades have never been studied in dyslexic children. We examined vertical visually guided saccades in fifty-six dyslexic children (mean age: 10.5±2.56 years old) and fifty-six age matched non dyslexic children (mean age: 10.3±1.74 years old). Binocular eye movements were recorded using an infrared video-oculography system (mobileEBT®, e(ye)BRAIN). Dyslexic children showed significantly longer latency than the non dyslexic group, also the occurrence of anticipatory and express saccades was more important in dyslexic than in non dyslexic children. The gain and the mean velocity values were significantly smaller in dyslexic than in non dyslexic children. Finally, the up-down asymmetry reported in normal population for the gain and the velocity of vertical saccades was observed in dyslexic children and interestingly, dyslexic children also reported an up-down asymmetry for the mean latency. Taken together all these findings suggested impairment in cortical areas responsible of vertical saccades performance and also at peripheral level of the extra-ocular oblique muscles; moreover, a visuo-attentionnal bias could explain the up-down asymmetry reported for the vertical saccade triggering.

Postural control in strabismic children: importance of proprioceptive information.

OBJECTIVE: To examine the effect of proprioceptive information during postural control in strabismic children. METHODS: Postural stability was recorded with a platform (Techno Concept®) in 12 strabismic children aged from 4.9 to 10 years and data were compared to that of 12 control age-matched children. Two postural positions were performed: Romberg and Tandem. Two postural conditions: without and with foam pad. We analyzed the surface area, the length, the mean speed of the center of pressure (CoP) and the effect of proprioceptive information. RESULTS: Strabismic children are more instable than control age-matched children. The surface, the length and the mean speed of CoP are significantly higher in strabismic children than in control age-matched children. Both groups are more instable in Tandem position than in Romberg position. Finally, strabismic children use more proprioceptive information than control age-matched children. CONCLUSION: For both Romberg and Tandem position, strabismic children are more instable than control age-matched children. Strabismic children use proprioceptive information more than control age-matched children to control their posture. SIGNIFICANCE: Proprioceptive inputs are important for control posture particularly for strabismic population.

Effect of visual attention on postural control in children with attention-deficit/hyperactivity disorder.

We compared the effect of oculomotor tasks on postural sway in two groups of ADHD children with and without methylphenidate (MPH) treatment against a group of control age-matched children. Fourteen MPH-untreated ADHD children, fourteen MPH-treated ADHD children and a group of control children participated to the study. Eye movements were recorded using a video-oculography system and postural sway measured with a force platform simultaneously. Children performed fixation, pursuits, pro- and anti-saccades. We analyzed the number of saccades during fixation, the number of catch-up saccades during pursuits, the latency of pro- and anti-saccades; the occurrence of errors in the anti-saccade task and the surface and mean velocity of the center of pressure (CoP). During the postural task, the quality of fixation was significantly worse in both groups of ADHD children with respect to control children; in contrast, the number of catch-up saccades during pursuits, the latency of pro-/anti-saccades and the rate of errors in the anti-saccade task did not differ in the three groups of children. The surface of the CoP in MPH-treated children was similar to that of control children, while MPH-untreated children showed larger postural sway. When performing any saccades, the surface of the CoP improved with respect to fixation or pursuits tasks. This study provides evidence of poor postural control in ADHD children, probably due to cerebellar deficiencies. Our study is also the first to show an improvement on postural sway in ADHD children performing saccadic eye movements.

Influence of pulse pressure and spontaneous variations of macular thickness in patients with diabetic macular oedema.

PURPOSE: To study spontaneous variations of central macular thickness (CMT) and its relation to blood pressure (BP) in patients with diabetic macular oedema (DME). METHODS: 23 diabetic patients presenting with DME with a CMT ≥ 260 µm on optical coherence tomography (OCT-3, Carl Zeiss Meditec, CA) were followed every 2 weeks for 3 months. At baseline, ambulatory 24H-BP monitoring (ABPM) was performed, as well as five CMT measurements (9 am, 12 am, 3 pm, 6 pm and 9 am the day after). During follow-up, BP and CMT were simultaneously measured at 9 am. RESULTS: Significant spontaneous variations in CMT (at least one change in CMT greater than 11% compared to the median CMT value) were observed over 3 months in 48% of patients. Mean CMT decreased over the day and increased during the night, but not significantly (p = 0.1). During the 6 visits, the CMT at 9 am positively correlated with the pulse pressure (PP) measured at the same time (r = 0.29, p = 0.0008). In addition, the mean 24H-CMT was positively correlated with the mean 24H- PP (r = 0.48, p = 0.02). CONCLUSION: Significant spontaneous changes in CMT of patients with DME were observed in nearly half of cases over 3 months. Retinal thickness was correlated to PP levels (patients with higher CMT had higher PP levels). This high variability of macular oedema, and the influence of BP on retinal thickness, should be taken into consideration by practitioners when evaluating the benefit of a therapy in DME.