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The posterior transverse cordotomy (PTC) can be performed for posterior glottic stenosis (PGS) in the operating room through suspension laryngoscopy. This procedure requires adequate exposure of the vocal cords. An alternative PTC approach was carried out on a 56-year-old man without a view of the vocal cords during suspension laryngoscopy. After anteriorizing the hypertrophic tongue with a glide scope, the surgeon reached the vocal folds through a flexible nasofibroscopy with an operative channel. The adequate exposure of the posterior part of the vocal cords allowed the blue laser PTC through the operative channel in the cut setting. The postoperative outcomes were adequate in the follow-up period. This paper describes an alternative approach to PTC in a patient with PGS and several unsuccessful laryngeal exposures., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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., (© 2022. The Author(s).)

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.

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.

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.

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., (© 2015 by the American Diabetes Association. Readers may use this article as long as the work is properly cited, the use is educational and not for profit, and the work is not altered.)

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.

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.

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.

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.

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., (© 2014 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

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.

Purpose: The objective of our study was to examine horizontal smooth pursuit performance in strabismic children and in children with vergence deficits, and to compare these data with those recorded in a group of control age-matched children., Methods: Binocular eye movements were recorded by video-oculography in ten strabismic children (mean age: 9.8±0.8) and seven children with vergence deficits (mean age: 10.8±0.6). Data were compared to that of age-matched control children (mean age: 9.8±0.8 years)., Results: Catch-up saccades amplitude in strabismic children and in children with vergence deficits were significantly higher than in control age-matched children. Moreover, in strabismic children the amplitude of catch-up saccades was significantly higher in rightward than in leftward direction. The number of catch-up saccades was also significantly higher in rightward than in leftward direction. The gain value of pursuits in rightward direction was significantly higher in the right eye than in the left one; for the right eye, the gain value was significantly higher in rightward than in leftward direction. Binocular coordination of pursuit was better in control age-matched children than in children with vergence deficits and than in strabismic children., Conclusions: Binocular coordination of pursuit is abnormal in children with vergence deficits and worse in strabismic children. Binocular vision plays an important role in improving binocular coordination of pursuit.

The influence of a secondary task on concurrent postural control was explored in twenty-one dyslexic children (mean age: 10.4 ± 0.3 years). Data were compared with twenty age-matched non-dyslexic children. As a secondary task, a modified Stroop test was used, in which words were replaced with pictures of fruits. The postural control of children was recorded in standard Romberg condition as the children were asked to name the colour of fruits appearing consecutively on a computer screen. Two conditions were tested: a congruent condition, in which the fruit was drawn in its natural ripe colour, and a non-congruent colour condition (NC), in which the fruit was drawn in three abnormal colours. A fixating condition was used as baseline. We analyzed the surface, length and mean speed of the center of pressure and measured the number of correct responses in the Stroop-like tasks. Dyslexic children were seen to be significantly more unstable than non-dyslexic ones. For both groups of children, the secondary task significantly increased postural instability in comparison with the fixating condition. The number of correct responses in the modified Stroop task was significantly higher in the non-dyslexic than in the dyslexic group. The postural instability observed in dyslexic children is in line with the cerebellar hypothesis and supports the idea of a deficit in automatic performance in such children. Furthermore, in accordance with cross domain competition model, our findings show that attentional resources are used to a greater extent by the secondary task than in controlling body stability.

Purpose: To our knowledge, studies comparing binocular eye movements during reading task in strabismic children are scarce. The goal of our study was to examine binocular coordination of saccades during reading in strabismic children., Methods: Binocular eye movements were recorded by an infrared system (mobile EBT) in 18 strabismic children 6.8 to 16 years old (mean age 10.2 ± 3) who were asked to read a four-line text silently during binocular viewing. Data were compared to that of 18 age-matched non-strabismic control children., Results: Saccade amplitude was similar in strabismic and control children. In contrast, binocular coordination during and after the saccades was significantly poorer in strabismic children as opposed to control children. The duration of fixation also was significantly longer in strabismic children compared to controls. Binocular coordination in strabismic children who have binocular vision was better than those without binocular vision, but it still was worse than in control subjects., Conclusions: In strabismic children binocular saccade coordination is deficient and could be responsible for impaired reading capabilities. Binocular vision has an important role in improving binocular saccade yoking.

In the mammalian primary visual cortex, the corpus callosum contributes to the unification of the visual hemifields that project to the two hemispheres. Its development depends on visual experience. When this is abnormal, callosal connections must undergo dramatic anatomical and physiological changes. However, data concerning these changes are sparse and incomplete. Thus, little is known about the impact of abnormal postnatal visual experience on the development of callosal connections and their role in unifying representation of the two hemifields. Here, the effects of early unilateral convergent strabismus (a model of abnormal visual experience) were fully characterized with respect to the development of the callosal connections in cat visual cortex, an experimental model for humans. Electrophysiological responses and 3D reconstruction of single callosal axons show that abnormally asymmetrical callosal connections develop after unilateral convergent strabismus, resulting from an extension of axonal branches of specific orders in the hemisphere ipsilateral to the deviated eye and a decreased number of nodes and terminals in the other (ipsilateral to the non-deviated eye). Furthermore this asymmetrical organization prevents the establishment of a unifying representation of the two visual hemifields. As a general rule, we suggest that crossed and uncrossed retino-geniculo-cortical pathways contribute successively to the development of the callosal maps in visual cortex.

Several studies have examined postural control in dyslexic children; however, their results were inconclusive. This study investigated the effect of a dual task on postural stability in dyslexic children. Eighteen dyslexic children (mean age 10.3±1.2 years) were compared with eighteen non-dyslexic children of similar age. Postural stability was recorded with a platform (TechnoConcept®) while the child, in separate sessions, made reflex horizontal and vertical saccades of 10° of amplitude, and read a text silently. We measured the surface and the mean speed of the center of pressure (CoP). Reading performance was assessed by counting the number of words read during postural measures. Both groups of children were more stable while performing saccades than while reading a text. Furthermore, dyslexic children were significantly more unstable than non-dyslexic children, especially during the reading task. Finally, the number of words read by dyslexic children was significantly lower than that of non-dyslexic children and, in contrast to the non-dyslexic children. In line with the U-shaped non-linear interaction model, we suggest that the attention consumed by the reading task could be responsible for the loss of postural control in both groups of children. The postural instability observed in dyslexic children supports the hypothesis that such children have a lack of integration of multiple sensorimotor inputs.

Studies comparing binocular eye movements during reading and visual search in dyslexic children are, at our knowledge, inexistent. In the present study we examined ocular motor characteristics in dyslexic children versus two groups of non dyslexic children with chronological/reading age-matched. Binocular eye movements were recorded by an infrared system (mobileEBT®, e(ye)BRAIN) in twelve dyslexic children (mean age 11 years old) and a group of chronological age-matched (N = 9) and reading age-matched (N = 10) non dyslexic children. Two visual tasks were used: text reading and visual search. Independently of the task, the ocular motor behavior in dyslexic children is similar to those reported in reading age-matched non dyslexic children: many and longer fixations as well as poor quality of binocular coordination during and after the saccades. In contrast, chronological age-matched non dyslexic children showed a small number of fixations and short duration of fixations in reading task with respect to visual search task; furthermore their saccades were well yoked in both tasks. The atypical eye movement's patterns observed in dyslexic children suggest a deficiency in the visual attentional processing as well as an immaturity of the ocular motor saccade and vergence systems interaction.

Purpose: Early studies reported some abnormalities in saccade and vergence eye movements in children with vertigo and vergence deficiencies. The purpose of this study was to further examine saccade and vergence performance in a population of 44 children (mean age: 12.3±1.6 years) with vertigo symptoms and with different levels of vergence abnormalities, as assessed by static orthoptic examination (near point of convergence, prism bar and cover-uncover test)., Methods: Three groups were identified on the basis of the orthoptic tests: group 1 (n = 13) with vergence spasms and mildly perturbed orthoptic scores, group 2 (n = 14) with moderately perturbed orthoptic scores, and group 3 (n = 17) with severely perturbed orthoptic scores. Data were compared to those recorded from 28 healthy children of similar ages. Latency, accuracy and peak velocity of saccades and vergence movements were measured in two different conditions: gap (fixation offset 200 ms prior to target onset) and simultaneous paradigms. Binocular horizontal movements were recorded by a photoelectric device., Results: Group 2 of children with vergence abnormalities showed significantly longer latency than normal children in several types of eye movements recorded. For all three groups of children with vergence abnormalities, the gain was poor, particularly for vergence movement. The peak velocity values did not differ between the different groups of children examined., Interpretation: Eye movement measures together with static orthoptic evaluation allowed us to better identify children with vergence abnormalities based on their slow initiation of eye movements. Overall, these findings support the hypothesis of a central deficit in the programming and triggering of saccades and vergence in these children.