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Ocular following eye movements have provided insights into how the visual system of humans and monkeys processes motion. Recently, it has been shown that they also reliably reveal stereoanomalies, and, thus, might have clinical applications. Their translation from research to clinical setting has however been hindered by their small size, which makes them difficult to record, and by a lack of data about their properties in sizable populations. Notably, they have so far only been recorded in adults. We recorded ocular following responses (OFRs)–defined as the change in eye position in the 80–160 ms time window following the motion onset of a large textured stimulus–in 14 school-age children (6 to 13 years old, 9 males and 5 females), under recording conditions that closely mimic a clinical setting. The OFRs were acquired non-invasively by a custom developed high-resolution video-oculography system, described in this study. With the developed system we were able to non-invasively detect OFRs in all children in short recording sessions. Across subjects, we observed a large variability in the magnitude of the movements (by a factor of 4); OFR magnitude was however not correlated with age. A power analysis indicates that even considerably smaller movements could be detected. We conclude that the ocular following system is well developed by age six, and OFRs can be recorded non-invasively in young children in a clinical setting.

Background:Congenital dacryostenosis is one of the most common ophthalmological disorders in infants, with a high spontaneous resolution rate. In patients unresponsive to conservative treatment, the first-line approach is lacrimal drainage system probing, thought there is no clear consensus on optimal timing of surgery. The optimal treatment of patients unresponsive to primary probing is also controversial.Objectives:The aim of this study is to assess the optimal timing of probing in children with congenital dacryostenosis. Other purposes are to evaluate the efficacy of repeated probing and dacryointubation in patients unresponsive to the initial surgery without evident lacrimal outflow dysgenesis, and to determine the epidemiology of these maldevelopments.Methods:A retrospective consecutive cohort study was conducted in 625 eyes of 457 patients aged 7–48 months who underwent surgery for dacryostenosis. Patients were divided into 4 cohorts according to the timing of surgery. Data were analyzed using Fisher's test.Results:The success rate of primary probing was high, without significant differences between cohorts. One-third of recurrences were related to maldevelopments, the other two-thirds were treated with a second probing or dacryointubation, with high success rates, that did not significantly differ between the procedures. All cases unresponsive to the second surgery were resolved with dacryointubation.Conclusions:Probing is highly effective and its outcome is not affected by timing of surgery. Nevertheless, we advocate for early intervention, in order to identify possible maldevelopments, which require more invasive management. In patients unresponsive to primary probing, without evident maldevelopments, repeated probing should still be considered as the first-line approach, since it's less invasive but similarly effective to dacryointubation.

Purpose: First, to create an optic nerve growth curve from normal optic nerve sheath diameter (ONSD) values measured by using B-scan ultrasonography in subjects 0-18 years of age. Second, to identify age-appropriate cutoff values of ONSD to be used in the diagnosis of intracranial hypertension (IHT). Design: Prospective cross-sectional study. Methods: B-scan ocular ultrasonography was performed on both eyes of 215 subjects 0-18 years of age, divided into 3 groups: 165 healthy children, 29 children with IHT (all >4 years of age), and 21 children with optic disc drusen (ODD). Results: There were no statistically significant differences in between the ONSDs of healthy children and those in subjects with ODD. An optic nerve growth curve was created by using ONSDs measured in healthy subjects 0-18 years of age, using the equation: ONSD = ln [33.15] – (–0.18 × ln [children's age]). The curve showed a progressive increase of ONSD up to 10 years of age, and it remained constant until the age of 18. For this reason, 2 different cutoff values were calculated for age groups 4-10 and 11-18. Values were 4.10 mm and 4.4 mm, respectively, with a 100.0% sensitivity and a specificity ranging from 83.9% to 98.8%. A sensitivity of 28.6% was reached for the population of subjects 4-18 years of age with a threshold value of 5 mm, as used in published reports. Conclusions: The ONSD continued to enlarge gradually until the age of 10. Therefore, 2 different cutoff values for the age groups 4-10 and 11-18 were calculated, considering the ONSDs of subjects 11-18 years of age overlapping with those of adults. No patients with IHT

Purpose: To disclose the association between spinocerebellar ataxia with oculomotor apraxia and high grade (7 diopters) congenital astigmatism. Methods: Single observational case report. A 39-year-old patient affected by spinocerebellar ataxia from the age of 20 was submitted to genetic and ophthalmic investigations to reach a diagnosis. Results: Genetic testing did not lead to a sure diagnosis, while clinical and instrumental ophthalmic examinations pointed out an oculomotor apraxia and a congenital severe astigmatism. Conclusion: To conclude the eye movement recording permitted to identify an oculomotor apraxia in this case of spinocerebellar ataxia. This is the first report of severe astigmatism in cases of ataxia with oculomotor apraxia.

Purpose: Consecutive exotropia is a frequent consequence of surgery for convergent strabismus that may develop at differing rates postoperatively. Several surgical options on horizontal recti have been proposed, but none report a clearly standardized amount of surgery. The present study provides further results of the medial rectus muscle advancement. Methods: Twenty-eight patients, age range 6 - 55 years, who had undergone unilateral or bilateral medial rectus advancement to the physiological insertion, with or without contemporary unilateral lateral rectus muscle recession, were included in the study. Factors leading to the onset of consecutive exotropia were analyzed. Results: After 2 - 3 years, the overall mean angle reduction was 21.3 prismatic diopters (PD) for distance and 22.8 PD for near, with an effectiveness on near vision lower than expected. Only in the 7 cases with a preoperatively larger exotropia at near (of at least 10 PD), the reduction was 17.0 PD for distance and 24.6 PD for near. In unilateral medial rectus muscle advancement, the mean reduction was 14.3 PD for distance and 16.3 PD for near; in bilateral advancement it was 25.8 PD and 25.2 PD respectively. Conclusion: Medial rectus advancement is preferable to other options, especially in cases with convergence deficit, if no excess of divergence or limitation of bulb rotation is present, for which a lateral rectus muscle recession is indicated. Unilateral and bilateral surgeries are useful for exotropia of about 15 PD and 25 PD respectively. The effectiveness depends weakly or not at all by the amount of the previously executed recession.

Many years after surgical correction, a complete unilateral or bilateral cleft is inclined to show an inaesthetism often associated with functional defects. This sequela disturbs the facial growth during childhood. Across the world, each surgical school uses its own protocol, but which is the best surgical protocol for patients with cleft? The aim of this study was to present a review of international literature concerning surgical techniques for the repair of cleft lip and palate (CLP) in children and to report our personal surgical techniques in this field. We focus on the main role of the primary surgery and propose a personalized protocol therapy, depending on the severity of the cleft. On 36 patients, most of them showed unilateral CLP at birth; only 4 showed bilateral cleft. In this study, we used 36 patients without cleft but with class I occlusion for comparison purposes. Analysis of the 2 groups regarding the development of the maxillary arch and the evaluation of palatal morphology was carried out using lateral cephalograms and dental casts. The main result showed 28 patients with acceptable teeth occlusion and speech quality, a valid nasal function, and a proper aesthetic aspect. Controversy still exists regarding the optimum timing and surgical technique for CLP repair. We propose the creation of a scientific database on internationally recognized protocol as a starting point depending on the severity of the cleft, thus avoiding controversies in CLP therapeutic treatment.

The majority of optic pathway tumors associated with Neurofibromatosis type-1 (NF-1) are benign, slow-growing lesions: however, rapidly growing tumors, which cause proptosis and visual loss, can sometimes occur. Optimal management of these tumors is still unclear. We report the case of a 12-year-old male, affected by NF-1 who was found to have a rapidly growing right optic nerve glioma, treated with carboplatin and vincristine. During chemotherapy, optic disc swelling rapidly disappeared; the orbital tumor decreased in size, with magnetic resonance imaging (MRI) evidence of tumor shrinkage. There was a progressive improvement in visual acuity and Visually Evoked Potential (VEP) amplitudes. Children with NF-1 associated optic pathway tumors should not receive chemotherapy unless there is documented progressive disease. Nevertheless, in contrast with previous studies, this case demonstrates that chemotherapy can be a safe and effective treatment for NF-1-associated optic nerve glioma, and should not be rese...

Hereditary Hearing Loss (HHL) is an extremely heterogeneous disorder. Approximately 30 out of 80 known HHL genes are associated with autosomal dominant forms. Here, we identified PSIP1/LEDGF (isoform p75) as a novel strong candidate gene involved in dominant HHL. Using exome sequencing we found a frameshift deletion (c.1554_1555del leading to p.E518Dfs*2) in an Italian pedigree affected by sensorineural mild-to-moderate HHL but also showing a variable eye phenotype (i.e. uveitis, optic neuropathy). This deletion led to a premature stop codon (p.T519X) with truncation of the last 12 amino acids. PSIP1 was recently described as a transcriptional co-activator regulated by miR-135b in vestibular hair cells of the mouse inner ear as well as a possible protector against photoreceptor degeneration. Here, we demonstrate that it is ubiquitously expressed in the mouse inner ear. The PSIP1 mutation is associated with a peculiar audiometric slope toward the high frequencies. These findings indicate that PSIP1 likely plays an important role in HHL.

Objective To assess the effects of hormone replacement therapy (HRT) on visual function after menopause. Design This study was conducted on 80 postmenopausal women aged 52 to 70 years. Women were randomly divided into two groups: 40 women were treated by oral HRT (equine conjugated estrogens 0.625 mg/day + dydrogesterone 5 mg/day in a continuous combined regimen), and 40 women were not treated with hormones (control group). Each woman underwent a contrast sensitivity test, a Schirmer test, and an evaluation of intraocular pressure before starting the study and 1 year after the beginning of the study. Statistical analysis was performed by Student's test and Fisher's exact test. Results Contrast sensitivity function was significantly improved in all spatial frequencies (1.5, 3, 6, and 12 cycles per degree) with the exception of 18 cycles per degree in the HRT group 1 year after the beginning of treatment, whereas the control group demonstrated significant impairment at the lowest spatial frequencies (1.5, 3, and 6 cycles per degree). Tear production was significantly improved in the HRT group 1 year after the beginning of treatment, and intraocular pressure was similar in the two groups before and after the beginning of the study. Conclusions HRT improves visual function, promoting a better contrast sensitivity and a higher tear production, but does not modify intraocular pressure.

We report the case of a girl, aged 17 years, who was diagnosed at the age of 3 years with a chronic bilateral iridocyclitis. Visual acuity was light perception in the right eye and 20/80 in the lef...

To describe a rare case of congenital self-healing Langerhans cell histiocytosis (CSHLCH) presenting with atypical eye involvement.Case report.A female newborn presented with purpuric lesions over the trunk, limbs, and face. Liver ultrasonography revealed hypoechogenic lesions with blurred borders. Biomicroscopy showed right posterior synechiae with fibrinoid deposits on the lens. At 7 months she presented with right acute glaucoma.Biomicroscopy showed the presence of inflammatory pseudo-membrane covering the anterior surface of the lens, iris, and iridocorneal angle. Ab externo trabeculotomy was performed; access to the anterior chamber with capsulorrhexis forceps permitted a peeling of the pseudo-membrane with normalization of the intraocular pressure. Histologic examination of the membrane revealed an inflammatory tissue with CD1a and S-100 positive histiocytic cells.This is the first case of CSHLCH describing acute glaucoma secondary to a pseudo-inflammatory membrane with typical histiocytic cells, occluding the iridocorneal angle.

Acta Ophthalmol. 2010: 88: 692–694 Abstract. Purpose: To evaluate central corneal thickness (CCT), intraocular pressure (IOP) and eye refraction in patients with congenital growth hormone (GH) deficiency. Methods: Retrospective case series. Forty-five patients with growth defect treated with recombinant GH and 45 healthy children underwent ophthalmological examination, including CCT measurements, applanation tonometry and cycloplaegic refraction. Results: The average CCT in the GH deficiency group was 570.6 μm [standard deviation (SD) 37.4]. In the control group, it was 546.0 (SD 24.9). The average IOP in the GH deficiency group was 18.2 mmHg (SD 3.4). In the control group, it was 14.6 (SD 2.0). The mean refractive error (spherical equivalent) in the GH deficiency group was 0.59 D (SD 1.9). In the control group, it was 0.11 (SD 2.1). Conclusion: GH and insulin-like growth factor 1 are involved in ocular growth by influencing the synthesis of the extracellular matrix of the sclera. Children with congenital GH deficiency or insensitivity have a mean hyperopic defect related to a shorter axial length. A number of studies have demonstrated that CCT in newborns is significantly greater than in adults; a decrease in CCT is closely correlated with an increase in corneal diameter. This finding suggests that the growth of the eye, with possible remodelling and stretching of collagen fibres, may play an important role in the reduction of corneal thickness in the first years of life. Therefore, we conclude that a greater CCT can represent a sign of a delayed growth of the eye in patients with GH deficiency. Finally, our study confirms the influence of corneal thickness on IOP measures, and the prevalence of hyperopia among children with growth defect.

The 4q deletion syndrome shows varying phenotype, ranging from severe and complex malformations, unconformable with life, to more specific findings, as genitourinary, gastrointestinal and cardiac malformations, cleft palate, microcephaly, hypertelorism and abnormal ears and limbs. Strabismus, nystagmus, ophthalmoplegia, and optic nerve anomalies have been rarely described in literature. We report an original case of simultaneous deletion and duplication of chromosome 4q, confirmed by SNPs-array analysis of DNA, and characterized by a previously unreported association between optic nerve hypoplasia and progressive external ophthalmoplegia.

This report describes the case of a 9-year-old girl affected by fetal alcohol syndrome who presented at birth with blepharophimosis and a cleft palate, which was submitted to surgery. She was referred to our hospital for a visual acuity reduction, where a diagnosis of keratoconus was made. This case highlights the rarity of the association between fetal alcohol syndrome and cleft palate and the previously unreported association involving fetal alcohol syndrome–keratoconus and cleft palate–keratoconus.

A novel INDEL mutation in theEDA gene resulting in a distinctX- linked hypohidroticectoder mal dysplasia phenotypein an Italian familyEditorX-Linked Hypohidrotic Ectodermal Dysplasia (XL-HED; MIM305100) is characterized by hypodontia, misshaped teeth, hypo-hidrosis, sparse hair, peculiar facial features,1,2and occurs in lessthan 1 in every 100.000 individuals.1XL-HED is caused bymutations in the Ectodysplasin-A (EDA) gene located at Xq12-q13 with more than 100 causative mutations reported todate.1,3,4The identification of disease-causing mutations con-firms the diagnosis, however, does not automatically imply agenotype–phenotype correlation.

Gaucher's disease (GD) is an autosomal-recessive disorder that leads to the storage of sphingolipid material (glucocerebroside) in different peripheral tissues and sometimes in the central nervous system. Among its three existing forms, the most frequent non-neurological form (type 1: GD1) is treatable with appropriate amounts of exogenous enzyme-replacement therapy (ERT), whereas in the type 3 form (GD3), progression of the neurological involvement may be slowed down or halted by much higher doses of ERT than those used in GD1 because of the inability of ERT to cross the blood-brain barrier.

Post-saccadic drift has been analyzed in strabismic children 11–18 yr old, before and after surgery. Before surgery all the subjects had a large multi-component post-saccadic drift. A disconjugate compensation of the drift was not active, but an optimized cyclopean compensation occurred, aimed at minimizing the drift size in both eyes, either with binocular or monocular vision. The drift was directed toward the static offset of the eyes. One week after surgery the drift increased in most cases but the cyclopean compensation still occurred. One week later, a partial disconjugate compensation decreased the drift to levels lower than before surgery. In one subject surgery produced very big three-component drift, exceeding 20 deg. One year after the drift was decreased to < 1 deg. A mathematical model is presented accounting for most of the results. Copyright © 1996 Elsevier Science Ltd.

The first case of bilateral orbital preseptal cellulitis complicating combined adenotonsillectomy and strabismus surgery is reported. The issues of antimicrobial prophylaxis are discussed. The authors speculate about the possible routes of surgical site infection. Transient bacteraemia secondary to adenotonsillectomy may be theoretically a source of distant surgical site infection to the orbit, raising the issue of distant surgical site contamination during multidisciplinary surgery. Combined adenotonsillectomy and eye surgery might benefit from prophylactic systemic antibiotic administration.

Congenital cataract is a leading cause of visual impairment in children and brings approximately 10% of childhood blindness worldwide. Molecular analysis revealed ~60 loci to be associated with several phenotypes of childhood cataracts. Until now, more than 30 loci and 18 genes on different chromosomes have been associated with autosomal dominant congenital cataract (ADCC). Here, we present a three-generation Italian family with a non syndromic ADCC. A linkage analysis carried out using HumanCytoSNP-12 DNA Analysis BeadChip led us to identify ten genomic regions virtually involved in the disease. All the genes located in these regions were scored for possible relationship with ADCC and, according to a strict clinical and genetic selection, 4 genes have been analyzed. A novel sequence variant was found in the CRYBB2 gene (p.Ser143Phe). This variant affects a conserved aminoacid in the third Greek key motif of the protein, cosegregates with the disease phenotype in all affected individuals and is not present both in the unaffected family members and 100 healthy control subjects. Finally, we identified the first CRYBB2 mutation in an Italian family causing a clinical picture of ADCC.

Introduction The complete absence of the chiasm (chiasmal aplasia) is a rare clinical condition. Hypoplasia of the optic nerve and congenital nystagmus are almost invariably associated characteristics. Microphthalmos or anophthalmos are common features in chiasmal aplasia, while central nervous system abnormalities are less frequent. Esophageal atresia can be isolated or syndromic. In syndromic cases, it is frequently associated with cardiac, limb, renal or vertebral malformations and anal atresia. More rarely, esophageal atresia can be part of anophthalmia-esophageal-genital syndrome, which comprises anophthalmia or microphthalmia, genital abnormalities, vertebral defects and cerebral malformations. Here, a previously unreported case of chiasmal aplasia presenting without microphthalmos and associated with esophageal atresia is described. Case presentation Aplasia of the optic chiasm was identified in a Caucasian Italian 8-month-old boy with esophageal atresia. An ultrasound examination carried out at 21 weeks' gestation revealed polyhydramnios. Intrauterine growth retardation, esophageal atresia and a small atrial-septal defect were subsequently detected at 28 weeks' gestation. Repair of the esophageal atresia was carried out shortly after birth. A jejunostomy was carried out at four months to facilitate enteral feeding. The child was subsequently noted to be visually inattentive and to be neurodevelopmentally delayed. Magnetic resonance imaging revealed chiasmal aplasia. No other midline brain defects were found. His karyotype was normal. Conclusion If achiasmia is a spectrum, our patient seems to depict the most severe form, since he appears to have an extremely severe visual impairment. This is in contrast to most of the cases described in the literature, where patients maintain good--or at least useful-- visual function. To the best of our knowledge, the association of optic nerve hypoplasia, complete chiasmal aplasia, esophageal atresia and atrial-septal defect, choanal atresia, hypertelorism and psychomotor retardation has never been described before.

Background: To describe clinical and genetic observations in a patient with horizontal gaze palsy and progressive scoliosis (HGPPS) without identified mutations in the ROBO3 gene. Materials and Methods: Neurologic and orthopedic evaluation of the proband; sequencing all exons, exon-intron boundaries, and promoter region of ROBO3 in the proband and his mother. Array CGH was also carried out in the proband and his mother to evaluate possible chromosomal deletion(s) and/or duplication(s). Results: The proband had complete horizontal gaze restriction with full vertical gaze and small amplitude horizontal pendular nystagmus. He also had severe scoliosis and brainstem hypoplasia pathognomonic of HGPPS. However, complete sequencing of ROBO3 twice in both forward and reverse directions did not reveal any mutations. Array CGH investigation revealed no chromosomal abnormalities. Conclusions: This patient had clinical and neuroimaging characteristics considered pathognomonic of HGPPS and yet did not have ROBO3 mutations. A clinical misdiagnosis is unlikely in the absence of facial weakness (typical of Moebius syndrome), deafness (typical of the HOXA1 spectrum), or mental retardation (typical of other central decussation abnormalities). It is perhaps more likely that a phenotype identical to HGPPS can be caused by abnormalities in ROBO3 splice variant expression, by mutations of a gene other than ROBO3, or by some environmental or epigenetic factor(s) inhibiting the action of ROBO3 or its protein product in the developing brainstem.

Purpose To provide detailed information about opthalmological findings in a group of patients with Angelman syndrome (AS). Methods Consecutive patients with a genetically confirmed diagnosis of AS were submitted to ophthalmic and orthoptic examinations. Strabismus, visual acuity, cycloplegic refraction, and iris and fundus pigmentation were evaluated. Parents were also examined to compare the extent of fundus pigmentation. Results A total of 34 patients were identified, representing 3 genetic classes: deletion, uniparental disomy, and mutation. Ametropia >1 D was present in 97% of cases: myopia in 9%, hyperopia in 76%, and astigmatism in 94%. Myopia and anisometropia were found only in the genetic deletion group. Strabismus, most frequently exotropia, was found in 24 patients (75%). Ocular hypopigmentation was observed in 18 subjects (53%), with choroidal involvement in 3 cases and isolated iris involvement in 4. Hypopigmentation was observed in all of the 3 genetic classes. Conclusions Ophthalmic alterations in AS were observed more frequently than has been previously reported, except for ocular hypopigmentation, which was observed less frequently.

Some automatic methods have been proposed to identify keratoconus from corneal maps; among these methods, neural networks have proved to be useful. However, the identification of the early cases of this ocular disease remains a problem from both a diagnostic and a screening point of view. Another problem is whether a keratoconus screening must be performed taking into account both eyes of the same subject or each eye separately; hitherto, neural networks have only been used in the second alternative. In order to examine the differences of the two screening alternatives in terms of discriminative capability, several combinations of the number of input, hidden and output nodes and of learning rates have been examined in this study. The best results have been achieved by using as input the parameters of both eyes of the same subject and as output the three categories of clinical classification (normal, keratoconus, other alterations) for each subject, a low number of neurons in the hidden layer (lower than 10) and a learning rate of 0.1. In this case a global sensitivity of 94.1% (with a keratoconus sensitivity of 100%) in the test set as well as a global specificity of 97.6% (98.6% for keratoconus alone) have been reached.

Background.Elastic cords hitting the eyeball as high-speed projectiles can severely damage ocular structures and can produce permanent visual function impairment.Objectives.To evaluate the frequency, mechanics, and severity of eye injuries caused by elastic cords in children to adopt the most appropriate preventive measures.Methods.A retrospective medical records review of hospital admissions secondary to ocular trauma between 1991 and 1997 in a pediatric ophthalmology unit at an urban tertiary care pediatric hospital was performed to select all children admitted for ocular injury caused by an elastic cord.Results.Eight children fulfilled the inclusion criteria; the prevalence ratio was 2% of all pediatric trauma admissions. In all cases the mechanics of trauma was a combination of blunt and high-speed projectile injury. The mechanism of trauma in younger patients was typically a cord that was misused during unsupervised playtime, whereas cord slipping from car roof racks was noted in older patients. One patient suffered a severe permanent visual impairment caused by retinal detachment. All other children regained full visual acuity at the time of discharge and maintained it through a mean follow-up of 22 months (range: 18–29).Conclusion.Circumstances of injury in younger children are different from those found in older children, the latter being similar to those reported for adults. Prevention is the primary measure to be taken to reduce the prevalence of this injury and to lower the risk for ocular severe anatomic damage as much as possible. This can be achieved primarily by modifying the design of the hooks, intensifying educational campaigns, and keeping elastic cords out of children's reach.

Intraoperative axial length biometry was carried out in 30 patients treated for esotropia with posterior fixation suture and/or recession of the medial rectus muscles. The exact intraoperative determination of the location of the equator is recommended in order to prevent: 1) Excessive medial rectus recessions with consecutive postoperative muscle underactions (usually a recession of 10.5–11 mm from limbus is considered safe, but this location could be too posterior to the equator); 2) A significant limitation of rotation in eyes treated with posterior fixation sutures. In fact, the placement of these sutures based on an arbitrary number of millimeters from the limbus (or from the insertion of the medial rectus muscle, usually 13 mm) again creates an excessive slack in the muscle and remarkably decreases its contractile force; and 3) high rates of undercorrections in esodeviations exceeding 60 prism diopters when traditional bimedial rectus recessions are performed as initial treatment.

The variability throughout the test of the smooth pursuit (SP) response to a sinusoidal stimulation was studied in 10 normal children and and in 10 adults. This phenomenon is not described in the literature and its influence on values of the SP characteristics is unknown: similarly, the values of the SP characteristics in school ages have not yet been subject of experimental study. The parameters which describe the response to a sinusoidal stimulation studied here are SP phase and gain (peak eye velocity /peak target velocity) and overall phase and gain (peak eye position/ peak target position). It is shown that records obtained from children present a marked fluctuation, depending on less constant attention level. Nevertheless, these fluctuations are not responsible for the different mean values of the SP characteristics found in adults and children. These differences are only marginally due (i.e. at the highest stimulation frequency) to fatigue, while they can be mainly ascribed to an incomplete SP maturation in children. This study concerned also the possible influence of the duration of each test on the mean values of the SP parameters. Our data demonstrate that a correct evaluation of each parameter is obtained only from records of longer than 6 cycles. Moreover, for a valid comparison among experimental groups, the same criterion of characteristics evaluation is needed: therefore all authors should explicitly mention the method used in order to allow comparisons among literature data.

Pursuit responses to a sinusoidally moving target have been studied in subjects of pediatric age affected by late-onset strabismus. The smooth pursuit (SP) has been evaluated from the smooth components of eye velocity and the global pursuit (GP), due to the interaction of the smooth pursuit and the saccadic systems, has been studied on the eye position traces. From the comparison with the SP and GP performances of orthophoric children, both SP and GP gains resulted in any condition significantly lower in our strabismic children, indicating reduced capacity to pursue either with the smooth pursuit system alone or with the interaction of the saccadic system, the latter being also due to inaccuracy in programming corrective saccades. The deviated eye exhibited the lowest gains, showing the crucial role of single binocular vision in the preservation of an efficient pursuit. A further aspect of our investigation concerned the effect of surgery of strabismus upon the gain values. When calculated after surgery, they were always higher than the corresponding pre-surgical values.

Introduction Behçet's disease is a chronic multisystem vasculitis characterized by mucocutaneous, articular, neurological, gastrointestinal and ophthalmological lesions. Ocular involvement is mainly represented by recurrent uveitis, especially posterior uveitis; however, iridocyclitis, retinal and choroidal vasculitis, optic neuritis and retinal vascular occlusion can also occur. Case presentation A 12-year-old Caucasian boy with a history of recurrent buccal aphthosis and nonspecific gastrointestinal symptoms was admitted to our hospital with blurred vision associated with acute episcleritis and papillitis. The patient's pathergy test was positive, suggesting a diagnosis of Behçet's disease. Corticosteroid and cyclosporine therapy was started, but further episodes were noted in both eyes. The patient was then switched to intravenous infliximab, with complete resolution of the inflammation after the second infusion. Conclusion Episcleritis and papillitis should be added to the list of uncommon manifestations of pediatric Behçet's disease. Infliximab is an effective, new therapeutic approach for Behçet's disease that is refractory to the conventional corticosteroid and immunosuppressive therapy.

The age-related trend values and the normal intraocular pressure (IOP) increase curve from birth through the 16th year of life were studied in 460 subjects with a noncontact tonometer (Keeler Pulsair, Keeler, Ltd, Windsor, Berks, UK). Much lower values than in adults were recorded in subjects up to the age of 3 or 4 years. This finding leads us to believe that in the treatment of infantile glaucoma IOP should be kept within the age physiologic levels, in an attempt to prevent visual field loss and optic atrophy.

The characteristics of saccadic eye movements have been extensively studied in adults; researches have also been devoted to the saccades of preschool age children. On the contrary, for primary school-age children no data exist; we investigate the eye movements (recorded utilizing an infrared technique) of six children 7 to 11 years old. The main results indicate that the values of some parameters (for example the saccadic latency and duration) are in the same range as the values of the correspondent parameters in adults, while the values of other parameters (in particular peak velocity and mean velocity/peak velocity ratio) are distinctly different from the ones measured in adult subjects.

Publisher Summary This chapter discusses the latency of saccadic eye movements. Some papers note that the latency of saccadic eye movements slightly increases with the size of the target displacement, whereas others find no latency increase up to 20 degrees. There may be a gaze-position dependence of saccadic latency and accuracy, and understanding the central mechanisms responsible for the generation of saccadic commands as well as their possible levels of action is essential. The study described involved experimental subjects who were asked to follow a dot visual target either with maximum velocity or maximum accuracy. The target moved randomly in time, with jumps of amplitude in a range of +/–35 degrees in the horizontal plane. The experiment concludes that latency variations, which were previously thought to result from the amplitude of the target displacement, are instead caused by the final target eccentricity. The bidirectional map represents the percent probability of occurrence of a corrective saccade at the coordinates.