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Objective Stagnation of skull growth is correlated with papilledema in craniosynostosis. In this retrospective cohort study, we describe the postoperative skull growth after surgical correction for sagittal synostosis and its relation to the development of papilledema. Methods Patients with isolated sagittal synostosis at our center between 2005 and 2012 were included. Occipitofrontal circumference (OFC) was analyzed, at 3 time points (preoperative, 2 years postoperative, and last OFC measurement) and 3 phases (initial postoperative growth, long-term growth, and overall growth), and related to papilledema on fundoscopy. Results In total, 163 patients were included. The first time interval showed a decline in skull growth, with subsequent stabilization at long term. Papilledema occurred postoperatively in 10 patients. In these patients, the OFC at 2 years and at last follow-up (T3) were significantly smaller than in patients without papilledema. A larger OFC resulted in a decreased odds of developing papilledema at both postoperative time points (at T2 (OR = 0.40, p = 0.01) and at T3 (OR 0.29, p Conclusion A small OFC is correlated with the occurrence of papilledema. A decline in OFC within 2 years postoperatively is common in sagittal synostosis and is acceptable up to a value of 0.25SD. Patients with an OFC at last follow-up of less than 0.5SD are at risk for developing papilledema.

Purpose: To explore parents' experiences, preferences and information needs when either patching treatment or dichoptic action video gaming is used as an amblyopia treatment for their child. Methods: A qualitative study was carried out on parents whose newly diagnosed amblyopic children participated in a randomised controlled trial (RCT) comparing the effects of dichoptic action video gaming versus patching. A purposive heterogenic sample was selected for an additional interview after the study period. Semi-structured interviews were conducted with one or both parents and transcribed verbatim, and a thematic analysis was performed. Results: Ten families agreed to participate: seven in the patching group and three in the gaming group. Two themes emerged from the data exploring experiences with treatment: (1) factors influencing compliance and (2) burden with treatment. Parents reported creating a routine which improved compliance with patching, as opposed to gaming where parents felt less need to conduct the treatment themselves as it was performed in the outpatient clinic. In both groups, parents experienced an information hiatus regarding the role of refractive error. In deciding the type of treatment to be used, parents preferred to deliberate the choice with the healthcare professional and discuss considerations resulting in shared decisions. The emerging themes were (1) effect and efficiency of treatment, (2) organisational aspects of treatments and (3) their child's traits. Conclusion: This study provides insight into the experiences of parents whose children underwent different types of amblyopia therapy. Both treatments have their own advantages and disadvantages. For parents, the effectiveness and efficiency of treatment were the most important aspects when deciding the method of management. Parents wish to come to a well-informed, shared decision regarding the type of amblyopia treatment.

Purpose To obtain pediatric normative reference values and determine whether optical coherence tomography (OCT) corresponds better with clinical signs of intracranial hypertension (ICH) compared to the traditional screening method fundoscopy in a large cohort of one type of single suture craniosynostosis. Methods Control subjects without optic nerve diseases and isolated sagittal synostosis patients aged 3–10 years who underwent fundoscopy and OCT were included in this prospective cohort study. Normative reference values were obtained through bootstrap analysis. Main outcome was the association between peripapillary total retinal thickness (TRT) and total retinal volume (TRV) and appearance on fundoscopy. Signs and symptoms suggestive of ICH, including skull growth arrest, fingerprinting, and headache, were scored. Results Sixty-four healthy controls and 93 isolated sagittal synostosis patients were included. Normative cut-off values for mean TRT are 504 μm and for mean TRV 3 and > 0.39 mm3. TRT was increased in 16 (17%) and TRV in 15 (16%) of 93 patients, compared to only 4 patients with papilledema on fundoscopy (4%). Both parameters were associated with papilledema on fundoscopy (OR = 16.7, p = 0.02, and OR = 18.2, p = 0.01). Skull growth arrest was significantly associated with abnormal OCT parameters (OR = 13.65, p Conclusions The established cut-off points can be applied to screen for ICH in pediatrics. The present study detected abnormalities with OCT more frequent than with fundoscopy, which were associated with skull growth arrest. Therefore, a combination of OCT, fundoscopy, and skull growth arrest can improve clinical decision-making in craniosynostosis.

Purpose: To compare the effectiveness and efficiency of supervised dichoptic action-videogame play to occlusion therapy in children with amblyopia. Methods: Newly diagnosed children with amblyopia aged 4–12 years were recruited, excluding strabismus >30PD. After 16 weeks of refractive adaptation children were randomized to gaming 1 h/week supervised by the researcher, or electronically monitored occlusion 2 h/day. The gaming group played a dichoptic action-videogame using virtual reality goggles, which included the task of catching a snowflake presented intermittently to the amblyopic eye. Contrast for the fellow eye was self-adjusted until 2 identical images were perceived. The primary outcome was visual acuity (VA) change from baseline to 24 weeks. Results: We recruited 96 children, 29 declined and 2 were excluded for language or legal issues. After refractive adaptation, 24 of the remaining 65 no longer met the inclusion criteria for amblyopia, and 8 dropped out. Of 16 children treated with gaming, 7 (6.7 years) completed treatment, whereas 9 younger children (5.3 years) did not. Of 17 treated with occlusion, 14 (5.1 years) completed treatment and 3 (4.5 years) did not. Of 5 children with small-angle strabismus, 3 treated with occlusion completed treatment and 2 treated with gaming did not. Median VA improved by 0.30 logMAR (IQR 0.20–0.40) after gaming, 0.20 logMAR (0.00–0.30) after occlusion (p = 0.823). Treatment efficiency was 1.25 logMAR/100 h (range 0.42–2.08) with gaming, 0.08 (−0.19–0.68) with occlusion (p < 0.001). Conclusion: Dichoptic gaming seems a viable alternative for older children with refractive amblyopia after glasses adaptation. Treatment efficiency with gaming under continuous supervision was 15 times higher than with occlusion at home.

Contains fulltext : 249568.pdf (Publisher’s version ) (Open Access) This study describes the clinical spectrum and genetic background of high myopia caused by mutations in the ARR3 gene. We performed an observational case series of three multigenerational families with high myopia (SER≤-6D), from the departments of Clinical Genetics and Ophthalmology of a tertiary Dutch hospital. Whole-exome sequencing (WES) with a vision-related gene panel was performed, followed by a full open exome sequencing. We identified three Caucasian families with high myopia caused by three different pathogenic variants in the ARR3 gene (c.214C>T, p.Arg72*; c.767+1G>A; p.?; c.848delG, p.(Gly283fs)). Myopia was characterized by a high severity (

PURPOSE This study is the first to evaluate the prevalence of retinal thinning and the correlation with papilledema and visual acuity (VA) in a large population with craniosynostosis. DESIGN Prospective clinical cohort study. METHODS All and complex patients syndromic and complex with craniosynostosis who visited the only national referral center between 2018 and 2020 were included. Retinal layers were segmented using optical coherence tomography. Patients were seen by an ophthalmologist for VA assessment and fundoscopy. Multivariate regression models were developed to evaluate correlations between retinal thickness, papilledema and VA. RESULTS We included 127 patients. Retinal thinning was most prevalent in the peripapillary retinal nerve fiber layer (≤38%). A longer duration of papilledema in early childhood correlated with an increased peripapillary retinal nerve fiber layer and total retinal thickness optic nerve head later in life (+0.3 ± 0.2, P = .04 and +1.0 ± 1.0, P = .003); however, its thickness was not correlated with the VA (P = .20 and P = .53). Macular retinal thinning was associated with a worse VA (P = .01); however, it was not correlated with the duration of papilledema (P = .95). CONCLUSIONS Following a preventative treatment strategy for syndromic and complex craniosynostosis, the prevalence of retinal ONH thinning is low. Although the prevalence of peripapillary retinal nerve fiber layer thinning is considerable, its thickness is not correlated with VA. In contrast, macular thinning is correlated with worse VA scores and should, therefore, be evaluated during follow-up. Future studies should evaluate the (1) causative mechanism for macular thinning, (2) correlation between the time to surgery and macular thinning, and (3) results of reactive treatment strategies and compare those results to the current study.

Purpose In an ongoing randomised clinical trial comparing dichoptic VR video games with patching for amblyopia, we evaluated any potential barriers to successful use of this novel amblyopia treatment method. Methods From December 2017, all newly diagnosed amblyopic children were recruited. Excluded were children under age 4 and patients with strabismus exceeding 30PD. The video game was played for 1 h per week at the outpatient clinic under direct supervision. Records were kept of difficulties encountered during treatment and categorised into domains. Factors influencing the successful completion of this treatment were identified and related to patient characteristics. Results Ninety-one children were recruited for the trial, 20 parents refused participation before randomisation, because of the logistical challenges the outpatient dichoptic treatment would cause them. Of the 17 children who commenced dichoptic treatment (median age 6.2 years; IQR 4.9–8.4 years), 10 did not complete treatment. Children under age 5.5 years were unable to comprehend the game settings or the game itself. Older children (N = 7; 41%) were less willing to comply with the video game. Loss of interest in the game (N = 8; 47%) was found to be a limiting factor at all ages. Conclusion Half of the children failed to complete VR dichoptic treatment, mainly due to young age. In countries with nationwide screening where amblyopia is detected before age 6, the applicability of such dichoptic treatment is limited.

Purpose: To determine change in visual acuity (VA) in the population of a previous amblyopia treatment study (Loudon 2006) and assess risk factors for VA decrease. Methods: Subjects treated between 2001 and 2003 were contacted between December 2015 and July 2017. Orthoptic examination was conducted under controlled circumstances and included subjective refraction, best corrected VA, reading acuity, binocular vision, retinal fixation, cover-uncover and alternating cover test. As a measure for degree of amblyopia, InterOcular VA Difference (IOD) at the end of occlusion therapy was compared with IOD at the follow-up examination using Wilcoxon’s signed-rank test. Regression analysis was conducted to determine the influence of clinical and socio-economic factors on changes in IOD. Results: Out of 303 subjects from the original study, 208 were contacted successfully, 59 refused and 15 were excluded because of non-amblyopic cause of visual impairment. Mean IOD at end of therapy (mean age 6.4 years) was 0.11 ± 0.16 logMAR, and IOD at follow-up examination (mean age 18.3 years) was 0.09 ± 0.21 logMAR; this difference was not significant (p = 0.054). Degree of anisometropia (p = 0.008; univariable analysis), increasing anisometropia (p = 0.009; multivariable), eccentric fixation (p

Aim: To assess the long-term outcomes of our management protocol for Saethre–Chotzen syndrome, which includes one-stage fronto-orbital advancement. Method: All patients born with Saethre–Chotzen syndrome between January 1992 and March 2017 were included. Evaluated parameters included occipital frontal head circumference (OFC), fundoscopy, neuroimaging (ventricular size, tonsillar position, and the presence of collaterals/an abnormal transverse sinus), polysomnography, and ophthalmological outcomes. The relationship between papilledema and its associated risk factors was evaluated with Fisher’s exact test. Results: Thirty-two patients (21 females, 11 males) were included. Median (SD) age at first surgery was 9.6 months (3.1mo) for patients who were primarily referred to our center (range: 3.6–13.0mo), the median (SD) age at last follow-up was 13 years (5y 7mo; range: 3–25y). Seven patients had papilledema preoperatively, which recurred in two. Two patients had papilledema solely after first surgery. Second cranial vault expansion was indicated in 20%. Thirteen patients had an OFC deflection, indicating restricted skull growth, one patient had ventriculomegaly, and none developed hydrocephalus. Eleven patients had emissary veins, while the transverse sinus was aberrant unilaterally in 13 (hypoplastic n=10 and absent n=3). Four patients had mild tonsillar descent, one of which was a Chiari type I malformation. Four patients had obstructive sleep apnoea (two mild, one moderate, and one severe). An aberrant transverse sinus was associated with papilledema (p=0.01). Interpretation: Single one-stage fronto-orbital advancement was sufficient to prevent intracranial hypertension for 80% of our patients with Saethre–Chotzen syndrome. Follow-up should focus on OFC deflection and venous anomalies.

Contains fulltext : 282474.pdf (Publisher’s version ) (Open Access) High myopia [refractive error ≤ -6 diopters (D)] is a heterogeneous condition, and without clear accompanying features, it can be difficult to pinpoint a genetic cause. This observational study aimed to evaluate the utility of whole exome sequencing (WES) using an eye disorder gene panel in European patients with high myopia. Patients with high myopia were recruited by ophthalmologists and clinical geneticists. Clinical features were categorized into isolated high myopia, high myopia with other ocular involvement or with systemic involvement. WES was performed and an eye disorder gene panel of ~500 genes was evaluated. Hundred and thirteen patients with high myopia [mean (SD) refractive error - 11.8D (5.2)] were included. Of these, 53% were children younger than 12 years of age (53%), 13.3% were aged 12-18 years and 34% were adults (aged > 18 years). Twenty-three out of 113 patients (20%) received a genetic diagnosis of which 11 patients displayed additional ocular or systemic involvement. Pathogenic variants were identified in retinal dystrophy genes (e.g. GUCY2D and CACNA1F), connective tissue disease genes (e.g. COL18A1 and COL2A1), non-syndromic high myopia genes (ARR3), ocular development genes (e.g. PAX6) and other genes (ASPH and CNNM4). In 20% of our high myopic study population, WES using an eye gene panel enabled us to diagnose the genetic cause for this disorder. Eye genes known to cause retinal dystrophy, developmental or syndromic disorders can cause high myopia without apparent clinical features of other pathology.

Stagnation of skull growth is correlated with papilledema in craniosynostosis. In this retrospective cohort study, we describe the postoperative skull growth after surgical correction for sagittal synostosis and its relation to the development of papilledema.Patients with isolated sagittal synostosis at our center between 2005 and 2012 were included. Occipitofrontal circumference (OFC) was analyzed, at 3 time points (preoperative, 2 years postoperative, and last OFC measurement) and 3 phases (initial postoperative growth, long-term growth, and overall growth), and related to papilledema on fundoscopy.In total, 163 patients were included. The first time interval showed a decline in skull growth, with subsequent stabilization at long term. Papilledema occurred postoperatively in 10 patients. In these patients, the OFC at 2 years and at last follow-up (T3) were significantly smaller than in patients without papilledema. A larger OFC resulted in a decreased odds of developing papilledema at both postoperative time points (at T2 (OR = 0.40, p = 0.01) and at T3 (OR 0.29, p 0.001)). Sensitivity and specificity analysis indicated that an OFC below 0.25 SD at T2 (sensitivity 90%, specificity 65%) and below 0.49 at T3 (sensitivity 100%, specificity 60%) are related to the occurrence of papilledema.A small OFC is correlated with the occurrence of papilledema. A decline in OFC within 2 years postoperatively is common in sagittal synostosis and is acceptable up to a value of 0.25SD. Patients with an OFC at last follow-up of less than 0.5SD are at risk for developing papilledema.

Learning Objectives Upon completion of this activity, participants will be able to:Distinguish the most salient pathological feature of high myopia.Evaluate the long-term tolerability of atropine eye drops for myopia.Analyze the long-term efficacy of atropine eye drops for myopia.Assess variables that might alter the efficacy of atropine eye drops for myopia. Continuing Medical Education In support of improving patient care, this activity has been planned and implemented by Medscape, LLC and Springer Nature. Medscape, LLC is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC), to provide continuing education for the healthcare team. Medscape, LLC designates this Journal-based CME activity for a maximum of 1.00 AMA PRA Category 1 Credit(s). Physicians should claim only the credit commensurate with the extent of their participation in the activity. All other clinicians completing this activity will be issued a certificate of participation. To participate in this journal CME activity: (1) review the learning objectives and author disclosures; (2) study the education content; (3) take the post-test with a 75% minimum passing score and complete the evaluation at www.medscape.org/journal/eye; (4) view/print certificate. Credit hours 1.0 Release date: Expiration date: 21 September 2021 Post-test link: https://medscape.org/eye/posttest934752 Authors/Editors disclosure information Sobha Sivaprasad, MD, has disclosed the following relevant financial relationships: served as an advisor or consultant for: Allergan, Inc.; Apellis; Bayer AG; Boehringer Ingelheim Pharmaceuticals, Inc.; Heidelberg Pharma GmbH; Novartis; Oculis; Optos; Oxurion; Roche. Served as a speaker or a member of a speakers bureau for: Allergan, Inc.; Bayer AG; Novartis Pharmaceuticals Corporation; Optos. Received grants for clinical research from: Allergan, Inc.; Bayer AG; Boehringer Ingelheim Pharmaceuticals, Inc.; Novartis Pharmaceuticals Corporation; Optos. Jan Roelof Polling, MD, has disclosed the following relevant financial relationships: served as an advisor or consultant for: Théa Laboratories; Nevakar, Inc. Emily Tan, MD, has disclosed no relevant financial relationships. Sjoerd Driessen, MD, has disclosed no relevant financial relationships. Sjoukje E. Loudon, MD, has disclosed no relevant financial relationships. Hoi-Lam Wong, MD, has disclosed no relevant financial relationships. Astrid van der Schans, MD, PhD, has disclosed no relevant financial relationships. J. Willem Tideman, MD, has disclosed no relevant financial relationships. Caroline C. W. Klaver, MD, PhD, has disclosed the following relevant financial relationships: served as an advisor or consultant for: Bayer AG; Nevakar, Inc.; Novartis Pharmaceuticals Corporation; Théa Laboratories. Journal CME author disclosure information Charles P. Vega, MD, has disclosed the following relevant financial relationships: served as an advisor or consultant for: GlaxoSmithKline; Johnson & Johnson Pharmaceutical Research & Development, L.L.C. Served as a speaker or a member of a speakers bureau for: Genentech, Inc.; GlaxoSmithKline., Background Atropine is the most powerful treatment for progressive myopia in childhood. This study explores the 3-year effectiveness of atropine in a clinical setting. Methods In this prospective clinical effectiveness study, children with progressive myopia ≥ 1D/year or myopia ≤ −2.5D were prescribed atropine 0.5%. Examination, including cycloplegic refraction and axial length (AL), was performed at baseline, and follow-up. Outcome measures were spherical equivalent (SER) and AL; annual progression of SER on treatment was compared with that prior to treatment. Adjustments to the dose were made after 1 year in case of low (AL ≥ 0.3 mm/year) or high response (AL

Background: Treacher Collins syndrome (TCS) is a rare craniofacial disorder characterized by bilateral hypoplasia of facial structures and periorbital, ocular, and adnexal anomalies. The purpose of this multicenter study was to report the prevalence of ocular and adnexal anomalies in TCS and to identify patients at risk for visual impairment. Methods: The medical records of patients seen at four craniofacial centers were reviewed retrospectively. The following data were reported: primary and secondary ocular and adnexal anomalies, orthoptic and ophthalmological findings, and severity of TCS based on the facial deformity. Results: A total of 194 patients were included, of whom 49.5% were examined by an ophthalmologist or optometrist. The mean age at the first visual acuity measurement was 6.96 ± 6.83 years (range, 1.50-47.08); at final measurement, 11.55 ± 10.64 years (range, 1.75-62.58). Primary ocular anomalies were reported in 98.5% of cases, secondary anomalies in 34.5%, strabismus in 27.3%, refractive errors in 49.5%, and visual impairment in 4.6%. We found no association between ocular anomalies and visual impairment or between the severity of TCS and ocular anomalies or visual impairment, except for an increased prevalence of secondary ocular anomalies in patients with more severe manifestations of TCS. Conclusions: Ocular anomalies were present in nearly all patients with TCS, even in mild cases.

OBJECTIVEThe authors evaluated the long-term outcome of their treatment protocol for Muenke syndrome, which includes a single craniofacial procedure.METHODSThis was a prospective observational cohort study of Muenke syndrome patients who underwent surgery for craniosynostosis within the first year of life. Symptoms and determinants of intracranial hypertension were evaluated by longitudinal monitoring of the presence of papilledema (fundoscopy), obstructive sleep apnea (OSA; with polysomnography), cerebellar tonsillar herniation (MRI studies), ventricular size (MRI and CT studies), and skull growth (occipital frontal head circumference [OFC]). Other evaluated factors included hearing, speech, and ophthalmological outcomes.RESULTSThe study included 38 patients; 36 patients underwent fronto-supraorbital advancement. The median age at last follow-up was 13.2 years (range 1.3–24.4 years). Three patients had papilledema, which was related to ophthalmological disorders in 2 patients. Three patients had mild OSA. Three patients had a Chiari I malformation, and tonsillar descent < 5 mm was present in 6 patients. Tonsillar position was unrelated to papilledema, ventricular size, or restricted skull growth. Ten patients had ventriculomegaly, and the OFC growth curve deflected in 3 patients. Twenty-two patients had hearing loss. Refraction anomalies were diagnosed in 14/15 patients measured at ≥ 8 years of age.CONCLUSIONSPatients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.

Background An increasing amount of children suffer from brain damage-related visual processing dysfunctions (VPD). At present, there is a lack of evidence-based rehabilitation methods that can be used early in development. We developed a visual rehabilitation protocol suitable from 1 year of age. The protocol contains objective, quantitative outcomes and is structured, comprehensive and individually-adaptive. Our aim is to investigate effectiveness of this first visual rehabilitation program for young children with (a risk of) VPD. Methods We conduct a single-blind, placebo-controlled trial that is embedded within standard clinical care. The study population consists of 100 children born very or extremely preterm (

OBJECTIVEUnicoronal synostosis results in frontal plagiocephaly and is preferably treated before the patient is 1 year of age to prevent intracranial hypertension (ICH). However, data on the prevalence of ICH in these patients is currently lacking. This study aimed to establish the prevalence of preoperative and postoperative signs of ICH in a large cohort of patients with unicoronal synostosis and to test whether there is a correlation between papilledema and occipitofrontal head circumference (OFC) curve stagnation in unicoronal synostosis.METHODSThe authors included all patients with unicoronal synostosis treated before 2 years of age at a single center between 2003 and 2013. The presence of ICH was evaluated by routine fundoscopy. The OFC growth curve was analyzed for deflection and in relationship to signs of ICH.RESULTSIn total, 104 patients were included in this study, 84 (81%) of whom were considered to have nonsyndromic unicoronal synostosis. Preoperatively, none of the patients had papilledema as determined by fundoscopy (mean age at surgery 11 months). Postoperatively, 5% of patients with syndromic synostosis and 3% of those with nonsyndromic synostosis had papilledema, and this was confirmed by optical coherence tomography. Raised intracranial pressure was confirmed in 1 patient with syndromic unicoronal synostosis. Six of 78 patients had OFC stagnation, which was not significantly correlated to papilledema (p = 0.22). One child with syndromic unicoronal synostosis required repeated surgery for ICH (0.96%).CONCLUSIONSPapilledema was not found in patients with unicoronal synostosis when they underwent surgery before the age of 1 year and was also very rare during follow-up. There was no relationship between papilledema and OFC stagnation.

Trigonocephaly is caused by metopic suture synostosis. It is treated by fronto-orbital remodeling, not only to correct the deformity but also to prevent intracranial hypertension, the reported prevalence in trigonocephaly of which ranges from 0 to 33 percent. To support treatment analysis and the design of a treatment protocol for intracranial hypertension in these patients, the authors wished to more accurately quantify the prevalence of preoperative and postoperative intracranial hypertension in a large patient cohort.The authors included all trigonocephaly patients born between 2001 and 2013 who had all been operated on at a single center. During follow-up, the presence of intracranial hypertension was evaluated by funduscopy, and occipitofrontal head circumference was measured. The occipitofrontal head circumference curve was analyzed and its relation to intracranial hypertension assessed.In total, 262 patients with trigonocephaly were included. Before surgery, 1.9 percent of them had intracranial hypertension; after surgery, 1.5 percent did (mean age at last follow-up, 4.9 years). Sixteen of 176 patients (9 percent) had occipitofrontal head circumference curve stagnation, which was significantly related to intracranial hypertension (p = 0.001, Fisher's exact test).Intracranial hypertension occurs only sporadically in patients with metopic suture synostosis. Occipitofrontal head circumference measurement should take a prominent place in the postoperative follow-up of metopic suture synostosis patients; stagnation of the occipitofrontal head circumference requires additional screening for intracranial hypertension.Therapeutic, IV.

Background: Children with Apert and Crouzon-Pfeiffer syndromes are at risk of intracranial hypertension. Until 2005, when the authors switched to occipital expansion, their institution's preferred treatment was fronto-orbital advancement. However, it was still unclear whether (1) occipitofrontal head circumference (i.e., intracranial volume) was greater after occipital expansion than after fronto-orbital advancement; (2) the incidences of tonsillar herniation and papilledema were lower; and (3) visual acuity was better during follow-up. In these patients, the authors therefore compared fronto-orbital advancement with occipital expansion as the first surgical procedure. Methods: Measurements included repeated occipitofrontal head circumference as a measure for intracranial volume; neuroimaging to evaluate tonsillar herniation; funduscopy to identify papilledema; and visual acuity testing. Results: The authors included 37 patients (Apert syndrome, n = 18; Crouzon-Pfeiffer syndrome, n = 19). Eighteen underwent fronto-orbital advancement and 19 underwent occipital expansion (age at surgery, 1.0 versus 1.5 years; p = 0.13). Follow-up time in both groups was 5.7 years. The increase in occipitofrontal head circumference (+1.09 SD) was greater after occipital expansion than after fronto-orbital advancement (+0.32 SD) (p = 0.03). After occipital expansion, fewer patients with Crouzon-Pfeiffer syndrome had tonsillar herniation (occipital, three of 11; fronto-orbital advancement, seven of eight; p = 0.02); for both syndromes together, fewer patients had papilledema (occipital, four of 19; fronto-orbital advancement, 11 of 18; p = 0.02). Visual acuity was similar after fronto-orbital advancement and occipital expansion (0.09 versus 0.13 logarithm of the minimum angle of resolution) (p = 0.28). Conclusions: The authors' preference for occipital expansion as the initial craniofacial procedure in Apert and Crouzon-Pfeiffer syndromes is supported by the greater increase it produces in intracranial volume (as evidenced by the occipitofrontal head circumference), which reduces the incidences of tonsillar herniation and papilledema.

Purpose: We showed previously that an educational cartoon that explains without words why amblyopic children should wear their eye patch improves compliance, especially in children of immigrant parents who speak Dutch poorly. We now implemented this cartoon in clinics in low socioeconomic status (SES) areas with a large proportion of immigrants and clinics elsewhere in the Netherlands. Design: Clinical, prospective, nonrandomized, preimplementation, and postimplementation study. Participants: Amblyopic children aged 3 to 6 years who started occlusion therapy. Methods: Preimplementation, children received standard orthoptic care. Postimplementation, children starting occlusion therapy received the cartoon in addition. At implementation, treating orthoptists followed a course on compliance. In low SES areas, compliance was measured electronically during 1 week. Main Outcome Measures: The clinical effects of the cartoon— electronically measured compliance, outpatient attendance rate, and speed of reduction in interocular-acuity difference (SRIAD)—averaged over 15 months of observation. Results: In low SES areas, 114 children were included preimplementation versus 65 children postimplementation; elsewhere in the Netherlands, 335 versus 249 children were included. In low SES areas, mean electronically measured compliance was 52.0% preimplementation versus 62.3% postimplementation (P0.146); 41.8% versus 21.6% (P0.043) of children occluded less than 30% of prescribed occlusion time. Attendance rates in low SES areas were 60.3% preimplementation versus 76.0% postimplementation (P0.141), and 82.7% versus 84.5%, respectively, elsewhere in the Netherlands. In low SES areas, the SRIAD was 0.215 log/year preimplementation versus 0.316 log/year postimplementation (P0.025), whereas elsewhere in the Netherlands, these were 0.244 versus 0.292 log/year, respectively (P0.005; the SRIAD’s improvement was significantly better in low SES areas than elsewhere, P0.0203). This advantage remained after adjustment for confounding factors. Overall, 25.1% versus 30.1% (P0.038) had completed occlusion therapy after 15 months. Conclusions: After implementation of the cartoon, electronically measured compliance improved, attendance improved, acuity increased more rapidly, and treatment was shorter. This may be due, in part, to additional measures such as the course on compliance. However, that these advantages were especially pronounced in children in low SES areas with a large proportion of immigrants who spoke Dutch poorly supports its use in such areas. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. Ophthalmology 2012;119:2393–2401 © 2012 by the American Academy of Ophthalmology.

Purpose: To describe the frequency of refractive errors and amblyopia in unscreened children aged 2 months to 12 years from a rural town in Poland. Methods. Five hundred ninety-one children were identified by medical records and examined in a standardized manner. Visual acuity was measured using LogMAR charts; refractive error was determined using retinoscopy or autorefraction after cycloplegia. Myopia was defined as spherical equivalent (SE) ej0.50 D, emmetropia as SE between j0.5 D and +0.5 D, mild hyperopia as SE between +0.5 D and +2.0 D, and high hyperopia as SE Q+2.0 D. Amblyopia was classified as best-corrected visual acuity Q0.3 (e20/40) LogMAR, in combination with a 2 LogMAR line difference between the two eyes and the presence of an amblyogenic factor. Results. Refractive errors ranged from 84.2% in children aged up to 2 years to 75.5% in those aged 10 to 12 years. Refractive error showed a myopic shift with age; myopia prevalence increased from 2.2% in those aged 6 to 7 years to 6.3% in those aged 10 to 12 years. Of the examined children, 77 (16.3%) had refractive errors, with visual loss; of these, 60 (78%) did not use corrections. The prevalence of amblyopia was 3.1%, and refractive error attributed to the amblyopia in 9 of 13 (69%) children. Conclusions: Refractive errors are common in Caucasian children and often remain undiagnosed. The prevalence of amblyopia was three times higher in this unscreened population compared with screened populations. Greater awareness of these common treatable visual conditions in children is warranted. Copyright

Occlusion therapy for amblyopia has been the mainstay of treatment for centuries, however, acceptance of the patch is often lacking. This study evaluated comfort of wear of the eye patch and assessed the mechanical properties in order to achieve a more individualized prescription.For 8 consecutive days, parents used each of the four main brands of patches for 2 consecutive days in a randomized fashion. After 2 days a 21-item questionnaire was completed to evaluate comfort of wear for each patch. Compliance was measured electronically using the Occlusion Dose Monitor (ODM). In addition, breathing capacity at 23°C and 33°C, resistance to water penetration, opacity, and strength of adhesion to the skin were measured.Twenty-four children participated. Overall, satisfaction was moderate: large differences in discomfort when removing the patch, skin reaction, and cosmetic appearance were found. In the material measurements large differences were found in opacity and strength of adhesion to the skin. In all brands breathing capability was minimal. Answers given by the parents matched the physical properties of the eye patch. There was no difference in electronically measured compliance between patches.We found large differences in comfort of wear and mechanical properties. Therefore, when prescribing a certain brand of patch, the wide variety needs to be taken into account. Further study into these properties seems warranted; especially breathing capability requires improvement since children often wear the patch for a longer period of time. This could contribute to increasing satisfaction and consequently may improve compliance.

PURPOSE. The Pediatric Vision Scanner (PVS) detects strabismus by identifying ocular fixation in both eyes simultaneously. This study was undertaken to assess the ability of the PVS to identify patients with amblyopia or strabismus, particularly anisometropic amblyopia with no measurable strabismus. METHODS. The PVS test, administered from 40 cm and requiring 2.5 seconds of attention, generated a binocularity score (BIN, 0%-100%). We tested 154 patients and 48 controls between the ages of 2 and 18 years. BIN scores of amblyopic children and controls were measured, and 21 children received sequential PVS measurements to detect any changes in BIN resulting from amblyopia treatment. RESULTS. With the pass/refer threshold set at BIN 60%, sensitivity and specificity were 96% for the detection of amblyopia or strabismus. Assuming a 5% prevalence of amblyopia or strabismus, the inferred positive and negative predictive values of the PVS were 56% and 100%, respectively. Fixation accuracy was significantly reduced in amblyopic eyes. In anisometropic amblyopia patients treated successfully, the BIN improved to 100%. CONCLUSIONS. The PVS identified children with amblyopia or strabismus with high sensitivity and specificity, while successful treatment restored normal BIN scores in amblyopic patients without strabismus. The results support the hypothesis that the PVS detects strabismus and amblyopia directly. Future strategies for screening by nonspecialists may thus be based on diagnostic detection of amblyopia and strabismus rather than the estimation of risk factors, allowing for rapid, accurate identification of children with amblyopia early in life when it is most amenable to treatment. (Invest Ophthalmol Vis Sci. 2011;52:5043-5048) DOI:10.1167/iovs.11-7503

Purpose: The efficacy of population-based vision screening is hampered by unsuccessful referral after a positive screening test. We studied the nature and causes of unsuccessful referral in a 7-year birth cohort study of vision screening in Rotterdam, the Netherlands. Methods: All parents of children who had been unsuccessfully referred were asked whether they recalled the referral. Reasons for noncompliance, if any, were identified using semi-structured interviews. Screening records were checked for written evidence of the referral. The parents' fluency in Dutch and their socioeconomic status were also assessed. Results: Of the 561 screen-positive children, 129 (23%) had not been referred successfully. For the current study, 97 parents were successfully contacted. Of these, 14 parents had been willingly noncompliant and 83 said they were unaware of the referral, with 47% having poor to moderate fluency in Dutch. In 53 cases, the screening charts contained no written evidence of any referral. Amblyopia was identified in 3 of the unsuccessful referrals. Conclusions: In this population-based screening program, 1 of 4 positively screened children was not successfully referred. Apart from parental noncompliance, the unsuccessful referrals can be explained by miscommunication, deficient documentation, and physician noncompliance with screening guidelines. An effective monitoring feedback system may improve the efficacy of child vision screening. © 2011 by the American Association for Pediatric Ophthalmology and Strabismus.

Background Compliance with occlusion therapy for amblyopia in children is low when their parents have a low level of education, speak Dutch poorly, or originate from another country. We determined how sociocultural and psychological determinants affect compliance. Methods Included were amblyopic children between the ages of 3 and 6, living in low socio-economic status (SES) areas. Compliance with occlusion therapy was measured electronically. Their parents completed an oral questionnaire, based on the “Social Position & Use of Social Services by Migrants and Natives” questionnaire that included demographics and questions on issues like education, employment, religion and social contacts. Parental fluency in Dutch was rated on a five-point scale. Regression analysis was used to describe the relationship between the level of compliance and sociocultural and psychological determinants. Results Data from 45 children and their parents were analyzed. Mean electronically measured compliance was 56 ± 44 percent. Children whose parents had close contact with their neighbors or who were highly dependent on their family demonstrated low levels of compliance. Children of parents who were members of a club and who had positive conceptualizations of Dutch society showed high levels of compliance. Poor compliance was also associated with low income, depression, and when patching interfered with the child’s outdoor activity. Religion was not associated with compliance. Conclusions Poor compliance with occlusion therapy seems correlated with indicators of social cohesion. High social cohesion at micro level, i.e., family, neighbors and friends, and low social cohesion on macro level, i.e., Dutch society, are associated with noncompliance. However, such parents tend to speak Dutch poorly, so it is difficult to determine its actual cause. Electronic supplementary material The online version of this article (doi:10.1007/s00417-011-1637-5) contains supplementary material, which is available to authorized users.

Aim: To analyse psychological causes for low compliance with occlusion therapy for amblyopia. Method: In a randomised trial, the effect of an educational programme on electronically measured compliance had been assessed. 149 families who participated in this trial completed a questionnaire based on the Protection Motivation Theory after 8 months of treatment. Families with compliance less than 20% of prescribed occlusion hours were interviewed to better understand their cause for non-compliance. Results: Poor compliance was most strongly associated with a high degree of distress (p < 0.001), followed by low perception of vulnerability (p = 0.014), increased stigma (p = 0.017) and logistical problems with treatment (p = 0.044). Of 44 families with electronically measured compliance less than 20%, 28 could be interviewed. The interviews confirmed that lack of knowledge, distress and logistical problems resulted in non-compliance. Conclusion: Poor parental knowledge, distress and difficulties implementing treatment seemed to be associated with non-compliance. For the same domains, the scores were more favourable for families who had received the educational programme than for those who had not.

Purpose To investigate omission of population-based eye screening at age 6–9 months in the Netherlands. Methods Prospective population-based consecutive birth cohort study was used. In two consecutive birth cohorts, children were eye screened at 1–2 and 3–4 months, but at general-health screening at 6–9 months, the second cohort was not eye screened, unless anything conspicuous was noted or in case of positive family history. Data were collected from screening records and anonymous questionnaires. Semi-structured daylong observations were made of physicians examining children aged 0–4 years, including children from the cohorts, by two orthoptic students. Results 58 of 6059 children (0.96%), in the screened, and 48 of 5482 children (0.88%) in the unscreened group were referred to orthoptist or ophthalmologist, mostly for observed strabismus. Amblyopia, all combined with strabismus, was diagnosed in ten screened (0.17%) versus six unscreened children (0.11%). Most physicians found preverbal examinations and decisions to refer difficult. The observations by orthoptic students revealed that cover test, pupillary reflexes, pursuit movements and eye motility were frequently performed inadequately, contrary to the Hirschberg test, at this age. Conclusion The screened and unscreened group differed little regarding the number of children referred and found to have amblyopia. Referral was mostly based on observed strabismus.

Electronic Occlusion Dose Monitors (ODMs) are a fairly recent development. As part of the international Electronic Recording of Patching for Amblyopia Group (ERPAG), we are the first center in Germany to apply these devices. The aim of our independent study was a further assessment of their potential, including technical features and the practicability of long-term use under near-clinical conditions. The ODMs, taped to the occlusion patch, measure the temperature difference between the surface of the eye and the surroundings. Investigators and patients' families kept occlusion diaries while using ODMs. Measurements were performed on the eye (patch tight or detached), forehead, arm and in trouser pockets, and while varying the room temperature. Patients' occlusion was monitored for several months together with acuity development. ODM acceptance was assessed by questionnaire. Written and monitored occlusion times corresponded excellently. Proper measurements on the eye could be distinguished more readily from those with the patch detached or in the pocket than from measurements on the arm and forehead. Very high temperatures (33-37 degrees C) prevented reliable measurements. Using ODMs was convenient for parents but time-consuming for researchers. Despite occasional data loss (incomplete diaries, ODM failure), acuity development could be followed as a function of monitored occlusion dose. This study contributes to the specification of the ODM features and their refinement for clinical use.

textabstractPURPOSE. Noncompliance is one of the limiting factors in the success of occlusion therapy for amblyopia. Electronic monitoring was used to investigate predictors of noncompliance, and, in a prospective randomized clinical trial, determined the effectiveness of an educational program. METHODS. Compliance was measured electronically during 1 week every 3 months in 310 newly diagnosed amblyopic children. The family's demographic parameters and the child's clinical parameters were assessed for their influence on the level of compliance. In addition to standard orthoptic care, children were randomized to receive an educational cartoon story, reward stickers, and an information sheet for the parents (intervention group), or a picture to color (reference group). These and the electronic device were distributed during home visits by researchers. The primary outcome measure was the percentage of compliance (actual/prescribed occlusion time) in the two groups. The secondary outcome measure was the influence of demographic and clinical factors on compliance. RESULTS. Compliance was associated with parental fluency in the national language, country of origin, level of education, and initial visual acuity of the child. During the first 1-week measurement period children in the intervention group had better compliance than the reference group had (78% ± 32% vs. 57% ± 40%; P < 0.0001), and fewer children were not occluded at all (3 vs. 23 in the reference group; P < 0.0001). This difference remained throughout the study period. CONCLUSIONS. Poor parental fluency in the national language, a low level of education, and poor acuity at the start of treatment were predictors of low compliance. An educational program primarily aimed at the child improved compliance and reduced the number of children who did not comply with occlusion at all. Copyright

Approximately one third of all amblyopic eyes do not reach visual acuity of 20/40 in spite of occlusion therapy. One of the reasons is a lack of adherence to therapy, which, however, could not be quantified in the past. Experience with new devices (occlusion dose monitors, ODMs) for electronic recording of occlusion has recently been reported. The aim of the present study was to evaluate the potential of the ODMs developed in The Netherlands. Various features were tested, including the reliability of the ODM recordings compared to diaries, two ODMs used simultaneously on one patch, the influence of the ambient temperature, and the specificity of the recording pattern for measurements on the eye.The ODMs were taped to the outside of the standard occlusion patch and measured the temperature difference between their front and back surfaces. Members of the research group and the families of two patients kept occlusion diaries while using the ODMs. Recorded and written occlusion periods were compared. Measurements were carried out under various conditions: patch with one ODM tightly on the eye or detached (allowing peeping); ODMs taped to various parts of the body; two ODMs simultaneously on one patch; variation of room temperature.There was good correspondence between the occlusion times recorded by the ODMs and those from the diaries, as well as between the recordings of two ODMs used simultaneously on one patch. High ambient temperatures (33 degrees C to 37 degrees C) prevented reliable ODM measurements. Measurements on other parts of the body were misclassified with probabilities between P=0.099 and P=0.325 as measurements with the patch tightly on the eye.In spite of some technical limitations, the ODMs provide a chance for reliable assessment of compliance and therefore objective information on dose-response function for occlusion therapy. This will lead to a more evidence-based treatment for amblyopia.

To identify the variation within and consistency amongst orthoptists when prescribing occlusion therapy for amblyopia in an objective survey.A questionnaire was designed with five case examples of amblyopic children and distributed at annual meetings of orthoptists in The Netherlands and Germany. It was filled in simultaneously within 15 min in complete silence to avoid any exchange of opinions between orthoptists that would reduce variability. For each case the orthoptists were asked to give their prescription of hours or days of occlusion.The questionnaire was filled in by 177 Dutch orthoptists and 227 German orthoptists. Their prescriptions of occlusion therapy were classified into five main regimens: part-time; part-time not every day; full-time; also occluding the amblyopic eye; alternating; and no occlusion therapy. The variation was large: the standard deviation was half the average prescribed hours of occlusion for each regimen in each of the five cases. All orthoptists were assigned a rank number for each of the five cases depending on whether their prescription was above or below average. These five rank numbers were not consistently above or consistently below average value per case.The number of prescribed hours of occlusion varied widely per regimen per case. Orthoptists were not consistently strict or lenient in their prescription of occlusion therapy.

We previously demonstrated that compliance with occlusion therapy for amblyopia was improved by the use of an educational programme, especially in children of parents of foreign origin and who spoke Dutch poorly. The programme consisted of: (i) a cartoon story for amblyopic children that explained without words why they should patch, (ii) a calendar with reward stickers, and (iii) an information leaflet for parents. In the current study, we assessed the individual effect of each component on compliance. We recruited 120 3- to 6-year-old children who lived in a low socio-economic status (SES) area in The Hague and were starting occlusion therapy for the first time. They were randomised to receive one of the components (three intervention groups), or a picture to colour (control group). The randomisation was blinded for treating orthoptist and researcher. Compliance was measured electronically using the Occlusion Dose Monitor (ODM). Primary outcome was percentage of compliance (actual/prescribed occlusion time). Secondary outcome was absolute occlusion hours per day. Parental fluency in Dutch was rated on a five-point scale. Compliance could be measured electronically in 88 of the 120 children; in 32 others, it failed for various reasons. Parental fluency in Dutch was moderate or worse in 36.4 % (p = 0.327). Average compliance was 55 % standard deviation (SD) 40 (n = 18) in the control group, 89 % SD 25 in the group receiving the educational cartoon (n = 25, P = 0.002 compared with control group), 67 % SD 33 (n = 24, P = 0.301) in the reward-calendar group and 73 % SD 40 (n = 21, P = 0.119) in the parent-information-leaflet group. On average, children in the control group occluded 1:46 SD1:19 hours/day, 2:33 SD 1:18 hours/day in the group receiving the educational cartoon, 1:59 SD 1:13 hours/day in the reward-calendar group and 2:18 SD 1:13 hours/day in the parent-information-leaflet group. No child who received the cartoon story occluded less than 1 hour per day, against seven in the reward-calendar group, five in the parent-information-leaflet group and five in the control group. Although all three components of the programme improved compliance with occlusion therapy in children in low-SES areas, the educational cartoon had the strongest effect, as it explained without words to a 4- to 5-year-old child why it should wear the eye patch.

OBJECTIVE To present and evaluate a new technique that allows the second-stage suture adjustment in strabismus surgery to be skipped or delayed if the immediate postoperative alignment is satisfactory. METHODS The "short tag noose" technique replaces long sutures with short tags that can be left under the conjunctiva after adjustment. Retrospectively, the medical records of all patients treated by a single surgeon with this approach between January 1, 2005, and December 31, 2008, were evaluated for success rate (< or =10 prism diopters [PD] horizontal and < or =6 PD vertical), reoperation rate, and complications. RESULTS Of 120 procedures reviewed, 27 (22.5%) were performed in children; 97 procedures (80.8%) were performed in patients with complex strabismus. Mean follow-up was 6 months. The success rate was 81.0% for horizontal strabismus and 70.7% for vertical strabismus at 2 months. The reoperation rate was 10.0% for horizontal strabismus and 19.0% for vertical strabismus. The noose slipped in 1 patient (0.8%) and was corrected by readjustment on day 4. Two patients (1.7%) required in-office excision of cysts or granulomas. CONCLUSIONS The short tag noose technique simplifies the logistics of suture adjustment and avoids the need for sedation in children who do not require adjustment. It provides the ability to defer adjustment for days after surgery.

Electronic monitoring of compliance shows that prescribing longer periods of occlusion is not always better

Currently, the most frequently prescribed treatment for amblyopia is patching of the better eye. Most people will refer to Comte de Buffon as the first to prescribe patching therapy ([De Buffon, 17...

Purpose. We set out to determine whether the children who have low compliance (measured electronically) with occlusion therapy for amblyopia are those with insufficient increase of visual acuity. Methods. In 14 newly identified amblyopic children (mean age 4.3±1.9 years), compliance was measured electronically over a period of 1 week, 6 months after the start of occlusion therapy. Compliance was measured with an Occlusion Dose Monitor (ODM). The measurements took place during planned domiciliary visits. The children were diagnosed with anisometropia (n=5), strabismus (n=4) and anisometropia and strabismus (n=5). Compliance was expressed in percentages of the electronically registered time compared with the prescribed occlusion time. Satisfactory acuity increase following 6 months of occlusion therapy was defined on reaching any of the following criteria: acuity increase expressed as a ratio between acuity of the amblyopic eye and acuity of the good eye of more than 0.75, acuity of the amblyopic eye exceeding 0.5 as measured on the E-Chart or Landolt-C, or three LogMAR lines of increase in acuity. Results. Measured compliance averaged 80% in the eight children who had a satisfactory acuity increase and 34% in the six children who had an unsatisfactory visual acuity increase. Children with low acuity increase had statistically significantly lower compliance (P=0.038). Conclusion. The general assumption among orthoptists, that compliance with occlusion therapy for amblyopia is low in children with insufficient acuity increase, has been validated by electronic, objective means.