Do infants with isolated congenital sixth nerve palsy require comprehensive work-up? A retrospective cohort and review of the literature.

PURPOSE: The purpose of this study is to describe a case series of infants with isolated congenital sixth nerve palsy (ICSNP) and suggest a management algorithm based on our experience and a review of the literature. METHODS: A retrospective cohort design was used. The clinical database of a single tertiary medical center was reviewed to identify all patients diagnosed with ICSNP from January 2020 to November 2022. Data were collected as follows: demographic parameters, age at initial presentation, presenting symptoms and signs, findings on ophthalmic and neurologic examinations, findings on follow-up, and outcome. RESULTS: Six patients were included. All were born at term. The average gestational weight was 3675.7 ± 262.7 g. Three mothers had gestational diabetes. Five deliveries necessitated labor induction either by oxytocin (n = 4) or by membrane stripping followed by oxytocin (n = 1). One had also gone a forceps assisted delivery. Symptoms were noticed in all newborns by their parents within the first week of life. Ophthalmological and neurological examinations were otherwise unremarkable apart of one patient with a head turn to the side of the involved eye. Four patients underwent brain imaging that were unremarkable. All abduction deficits resolved by 1 to 3 months of age. Follow up examinations were unremarkable (mean follow up 14.3 ± 5.0 months, range 4-23). CONCLUSIONS: This case series, together with previous reports, support ICSNP's benign nature. We suggest an initial basic work-up that solely includes ophthalmological and neurological examinations which will be elaborated in case of any additional pathologic findings or if ICSNP does not fully resolve by 3 months.

Trends in Pediatric Ophthalmic Emergency Department Visits during the COVID-19 Pandemic.

BACKGROUND: Data on how the coronavirus disease 2019 (COVID-19) affected consultations in ophthalmic departments are sparse. OBJECTIVES: To examine the epidemiology of ophthalmic consultations in a large pediatric emergency medicine department (PED) during the first nationwide COVID-19 lockdown in Israel. METHODS: The database of a tertiary pediatric medical center was retrospectively reviewed for patients aged < 18 years who attended the PED from 17 March to 30 April 2020 (first COVID-19 lockdown) and the corresponding period in 2019. Background, clinical, and disease-related data were collected from the medical charts and compared between groups. RESULTS: The study included 757 PED visits. There were no significant differences in demographics between the groups. The 2020 period was characterized by a decrease in PED visits (by 52%), increase in arrivals during late afternoon and evening (P = 0.013), decrease in visits of older children (age 5-10 year), and proportional increase in younger children (age 1-5 years) (P = 0.011). The most common diagnoses overall and during each period was trauma followed by conjunctivitis and eyelid inflammation. The mechanisms of trauma differed (P = 0.002), with an increase in sharp trauma and decrease in blunt trauma in 2020 (P < 0.001 for both). In 2020, 95% of traumatic events occurred in the home compared to 54% in 2019 (P < 0.001). CONCLUSIONS: Parents need to learn appropriate preventive and treatment measures to prevent serious and long-term ophthalmic injury while minimizing their exposure to the COVID-19. PEDs and ophthalmic pediatric clinics should consider increasing use of telemedicine and the availability of more senior physicians as consultants during such times.

The effect of anti-tumor necrosis factor alpha agents on the outcome in pediatric uveitis of diverse etiologies.

PURPOSE: This study aimed to report the clinical outcome of children with uveitis treated with anti-tumor necrosis factor alpha (TNF-α) agents. METHODS: This included a retrospective cohort study. Children with uveitis treated with infliximab or adalimumab in 2008-2014 at five dedicated uveitis clinics were identified by database search. Their medical records were reviewed for demographic data, clinical presentation, ocular complications, and visual outcome. Systemic side effects and the steroid-sparing effect of treatment were documented. RESULTS: The cohort included 24 patients (43 eyes) of whom 14 received infliximab and 10 received adalimumab after failing conventional immunosuppression therapy. Mean age was 9.3 ± 4.0 years. The most common diagnosis was juvenile idiopathic arthritis-related uveitis (n = 10), followed by Behçet's disease (n = 4), sarcoidosis (n = 1), and ankylosing spondylitis (n = 1); eight had idiopathic uveitis. Ocular manifestations included panuveitis in 20 eyes (46.5%), chronic anterior uveitis in 19 (44.2%), and intermediate uveitis in 4 (9.3%). The duration of biologic treatment ranged from 6 to 72 months. During the 12 months prior to biologic treatment, while on conventional immunosuppressive therapy, mean visual acuity deteriorated from 0.22 to 0.45 logMAR, with a trend of recovery to 0.25 at 3 months after initiation of biologic treatment, remaining stable thereafter. A full corticosteroid-sparing effect was demonstrated in 16 of the 19 patients (84.2%) for whom data were available. Treatment was well tolerated. CONCLUSIONS: Treatment of pediatric uveitis with anti-TNF-α agents may improve outcome while providing steroid-sparing effect, when conventional immunosuppression fails. The role of anti-TNF-α agents as first-line treatment should be further investigated in controlled prospective clinical trials.

Human cytomegalovirus pUL37x1-induced calcium flux activates PKCα, inducing altered cell shape and accumulation of cytoplasmic vesicles.

The human cytomegalovirus immediate-early protein pUL37x1 induces the release of Ca(2+) stores from the endoplasmic reticulum into the cytosol. This release causes reorganization of the cellular actin cytoskeleton with concomitant cell rounding. Here we demonstrate that pUL37x1 activates Ca(2+)-dependent protein kinase Cα (PKCα). Both PKCα and Rho-associated protein kinases are required for actin reorganization and cell rounding; however, only PKCα is required for the efficient production of virus progeny, arguing that HCMV depends on the kinase for a second function. PKCα activation is also needed for the production of large (1-5 µm) cytoplasmic vesicles late after infection. The production of these vesicles is blocked by inhibition of fatty acid or phosphatidylinositol-3-phosphate biosynthesis, and the failure to produce vesicles is correlated with substantially reduced production of enveloped virus capsids. These results connect earlier work identifying a requirement for lipid synthesis with specific morphological changes, and support the argument that the PKCα-induced large vesicles are either required for the efficient production of mature virus particles or serve as a marker for the process.

Dexamethasone intravitreal implant (Ozurdex®) for pediatric uveitis.

PURPOSE: The purpose of this study was to investigate the safety and effectiveness of an intravitreally injected dexamethasone-containing implant (Ozurdex(®)) in the treatment of uveitis in children. METHODS: The study group included ten patients (14 eyes) aged 6.5-15 years (mean age 12 years) with intermediate or posterior uveitis who were treated with the Ozurdex implant at two tertiary medical centers between 2009 and 2014, following an insufficient response to standard uveitis therapy. All were followed for at least 6 months (mean 12.2 ± 4.9 months). Clinical data before and after treatment were collected retrospectively from the medical files. Outcome measures were best corrected visual acuity, vitreous haze, and macular thickness. Ocular complications were documented. RESULTS: Visual acuity improved in 12 eyes (86 %) and intraocular inflammation decreased in 13 eyes (93 %) from 1 week to 3 months after the first injection. Macular edema decreased in all eyes from 1 month to 3 months after the first injection. Five patients underwent repeated injections because of an increase in macular thickness at 3-6 month follow-up. Complications included cataract progression in one patient after one injection and cataract formation in two patients after two injections, and an elevation in intraocular pressure in two patients who responded well to topical treatment. CONCLUSIONS: Both single and repeated injections of a dexamethasone-containing implant are safe and effective for the treatment of noninfectious intermediate and posterior uveitis in children. The duration of the beneficial effect may be limited. More data are required to establish the safety profile of the implant in the pediatric age group.

Anti-TNF-α agents for refractory cystoid macular edema associated with noninfectious uveitis.

BACKGROUND: The use of anti-tumor necrosis factor (TNF)-α agents for uveitis has been increasing since the first efficacy studies in 2004. However, information on their specific role in cystic macular edema remains sparse. The aim of this study was to evaluate the efficacy of anti-TNF-α agents for the treatment of uveitis-related refractory cystoid macular edema. METHODS: The files of 23 consecutive patients treated for cystoid macular edema at the uveitis services of two tertiary medical centers in 2006-2011 were reviewed for demographic data, visual acuity, and optical coherence tomography measurements at baseline and 3, 6, and 12 months after treatment. Changes in mean visual acuity and macular thickness were analyzed. Findings were compared between patients treated with a conventional immunosuppressive regimen only (n = 18, 27 eyes) and patients treated with an anti-TNF-α agent after proving refractory to conventional treatment (n = 9, 15 eyes). RESULTS: Mean duration of cystoid macular edema before initiation of anti-TNF-α therapy was 12 ± 8 months. The two groups had similar baseline values of mean central macular thickness and visual acuity. Significant improvement in macular thickness was noted at 3 months in both groups, with a maximal effect at 6 months for the anti-TNF-α group (p = 0.002). Maximal improvement in visual acuity was achieved at 3 months in both groups, with a reduced effect towards 12 months. Mean visual acuity at 12 months was similar in both groups. CONCLUSIONS: Anti-TNF-α agents may serve as an effective lasting treatment for long-standing refractory uveitis-related cystoid macular edema. Their role as first-line therapy in this setting warrants further investigation.

Synaptic vesicle-like lipidome of human cytomegalovirus virions reveals a role for SNARE machinery in virion egress.

Human cytomegalovirus induces and requires fatty acid synthesis. This suggests an essential role for lipidome remodeling in viral replication. We used mass spectrometry to quantify glycerophospholipids in mock-infected and virus-infected fibroblasts, as well as in virions. Although the lipid composition of mock-infected and virus-infected fibroblasts was similar, virions were markedly different. The virion envelope contained twofold more phosphatidylethanolamines and threefold less phosphatidylserines than the host cell. This indicates that the virus buds from a membrane with a different lipid composition from the host cell as a whole. Compared with published datasets, the virion envelope showed the greatest similarity to the synaptic vesicle lipidome. Synaptosome-associated protein of 25 kDa (SNAP-25) is a component of the complex that mediates exocytosis of synaptic vesicles in neurons; and its homolog, SNAP-23, functions in exocytosis in many other cell types. Infection induced the relocation of SNAP-23 to the cytoplasmic viral assembly zone, and knockdown of SNAP-23 inhibited the production of virus. We propose that cytomegalovirus capsids acquire their envelope by budding into vesicles with a lipid composition similar to that of synaptic vesicles, which subsequently fuse with the plasma membrane to release virions from the cell.

Medical And Surgical Management Of Orbital Cellulitis In Children.

OBJECTIVE: The purpose of this study was to identify features of orbital cellulitis that predict response to conservative treatment without surgical intervention and factors associated with a decision for surgery. PATIENTS AND METHODS: The medical files of patients diagnosed with orbital cellulitis at a tertiary medical center in central Israel between 1995 and 2010 were reviewed for clinical data, diagnosis, complications, and type of treatment. Comparison was made between patients treated with antibiotics and patients treated with antibiotics and surgery. RESULTS: Fifty-one patients (35 male) with a mean age of 6.1 years were identified. Main clinical signs included fever (mean 38.5°C), proptosis (82.3%), extraocular motility restriction (74.5%), and ocular pain (41.1%). Forty-one patients were successfully treated with antibiotics and 10 required endoscopic sinus surgery. On between-group comparison, the surgery group had severe eye pain (p = 0.009), severe proptosis (P = 0.02), longer intravenous antibiotic treatment (13.2 vs. 9.2 days, p = 0.04), and several imaging findings. Additional factors associated with surgical intervention included older children, subperiorbital abscess, larger dimension of the abscess (mean 15 mm), involvement of frontal sinuses and findings of intraorbital air bubbles. There was no visual deterioration in either group and no late sequelae. CONCLUSION: Factors associated with surgery included age older than 9 years, severe ocular pain, severe proptosis, and subperiorbital large abscess. These may be used for early identification of patients at risk of failure of only medical management.

Incidental Unilateral Macular Lesions in Children.

BACKGROUND: This study aims to examine the rare entity of unilateral macular lesions in the pediatric population and describe the distinct diagnoses and characterizations related to these findings. METHODS: A retrospective cohort design. The database of the ophthalmology clinic in a tertiary medical center was reviewed to identify all children with incidental unilateral macular findings, examined during 2016 through 2021. RESULTS: Twenty children were included. Mean age was 7.8 ± 3.4 years, 50% were girls. The most common macular lesion was torpedo maculopathy (50%), followed by pigmentary changes (25%), discoid maculopathy (15%), macular scar and combined hamartoma of the retina and retinal pigment epithelium (RPE) (5% each). None of the lesions changed after a mean follow-up duration of 2.3 ± 1.5 years. Visual acuity in the involved eye was equal to that in the contralateral eye in 90% of patients and did not change from initial to final visit. CONCLUSION: Incidental unilateral macular lesions in the pediatric population are usually benign, stable, and do not affect vision. Long-term follow-up is advised, as vision-threatening alterations may appear. [Ophthalmic Surg Lasers Imaging Retina 2023;54:346-352.].

Risk factors for the development of macular edema in children with uveitis.

Aim: To determine the risk factors for macular edema (ME) in children with uveitis. Methods: A retrospective study was conducted of 150 pediatric patients (264 eyes) with uveitis attending 2 tertiary medical centers. Data were collected from the medical files on demographics, type of uveitis, etiology, clinical findings, treatment, and time to development of ME. Risk factors for the development of ME were identified. Results: ME developed in 63 eyes (23.9%) over a mean period of 15.3 ± 2.95 months from diagnosis of uveitis, at a rate of 0.08 eyes per eye-year. On univariate analysis, risk factors for the development of ME were the non-anterior location of the inflammation (p=0.002), band keratopathy (p <0.0001), posterior synechiae (p=0.003), cataract (p=0.002), and vision impairment at presentation (p <0.0001). On multivariate analysis, non-anterior uveitis, which includes intermediate, pan, and posterior-uveitis, and vision impairment retained significance as independent risk factors of ME. Conclusion: Within the pediatric population with uveitis, non-anterior location is associated with the highest risk of ME, followed by the presence of complications, such as band keratopathy and posterior synechiae. These findings indicate a need for close follow-up in children with uveitis for early detection of ME.

A targeted spatial-temporal proteomics approach implicates multiple cellular trafficking pathways in human cytomegalovirus virion maturation.

The assembly of infectious virus particles is a complex event. For human cytomegalovirus (HCMV) this process requires the coordinated expression and localization of at least 60 viral proteins that comprise the infectious virion. To gain insight into the mechanisms controlling this process, we identified protein binding partners for two viral proteins, pUL99 (also termed pp28) and pUL32 (pp150), which are essential for HCMV virion assembly. We utilized HCMV strains expressing pUL99 or pUL32 carboxyl-terminal green fluorescent protein fusion proteins from their native location in the HCMV genome. Based on the presence of ubiquitin in the pUL99 immunoisolation, we discovered that this viral protein colocalizes with components of the cellular endosomal sorting complex required for transport (ESCRT) pathway during the initial stages of virion assembly. We identified the nucleocapsid and a large number of tegument proteins as pUL32 binding partners, suggesting that events controlling trafficking of this viral protein in the cytoplasm regulate nucleocapsid/tegument maturation. The finding that pUL32, but not pUL99, associates with clathrin led to the discovery that the two viral proteins traffic via distinct pathways during the early stages of virion assembly. Additional investigation revealed that the majority of the major viral glycoprotein gB initially resides in a third compartment. Analysis of the trafficking of these three viral proteins throughout a time course of virion assembly allowed us to visualize their merger into a single large cytoplasmic structure during the late stages of viral assembly. We propose a model of HCMV virion maturation in which multiple components of the virion traffic independently of one another before merging.

Functional and Structural Findings in Infants Treated for Retinopathy of Prematurity Using Optical Coherence Tomography Angiography.

BACKGROUND AND OBJECTIVE: To assess the foveal microvascular structure of children with retinopathy of prematurity (ROP) treated with diode laser photocoagulation using optical coherence tomography angiography (OCTA). PATIENTS AND METHODS: OCTA was performed at a tertiary medical center in 17 children (27 eyes) aged 4 to 16 years with a history of diode laser photocoagulation treated ROP. OCTA parameters were compared with those of 12 healthy age-matched controls (23 eyes) attending the orthoptics clinic and correlated with clinical factors. RESULTS: Compared with controls, the ROP group had a smaller foveal avascular zone area (P < .001), lower deep vascular plexus density (P < .001), lower flow density (P = .025), and greater central macular thickness (P < .001). High intraventricular hemorrhage grade correlated with smaller foveal avascular zone area (P = .008) and greater inner macular thickness (P = .047). There was no impact of gestational age, birth weight, or refractive status. CONCLUSION: OCTA can identify significant quantifiable long-term macular microvascular and structural changes in this patient population. [Ophthalmic Surg Lasers Imaging Retina. 2022;53(4):194-201.].

Intravitreal bevacizumab as supplemental treatment or monotherapy for severe retinopathy of prematurity.

PURPOSE: To investigate the benefit of intravitreal bevacizumab as supplemental or primary treatment for retinopathy of prematurity. METHODS: The files of nine consecutive infants treated with intravitreal bevacizumab for bilateral severe posterior retinopathy of prematurity were reviewed. RESULTS: Gestational age was 24 weeks to 27 weeks, and birth weight was 660 g to 1,131 g. Indications for treatment were retinopathy of prematurity progression from Stage 3 to 4A or 2 to 3 with extraretinal neovascularization despite laser treatment; active neovascular Stage 4A disease after laser and cryo-treatment; anterior segment neovascularization and bleeding after laser treatment; and aggressive posterior disease with tunica vasculosa lentis and vitreous haze, which prevented laser treatment. One patient (two eyes) underwent lens-sparing vitrectomy after bevacizumab treatment; one eye acquired macular fold. One patient underwent bilateral scleral buckle. Bevacizumab treatment was associated with subsidence of the active vascular component in all eyes. Anatomical results were favorable in 17 eyes. There were no local or systemic complications. CONCLUSION: Intravitreal bevacizumab may serve as a supplemental therapeutic agent for severe laser-refractory retinopathy of prematurity or as monotherapy when media opacities preclude diode laser photocoagulation or the patient is too sick for lengthy laser treatment.

Uveitis Induced by Biological Agents Used in Cancer Therapy.

Purpose: To report cases of uveitis induced by biological therapy given for cancer.Methods: Retrospective review of medical charts of patients.Results: Included were six patients aged 14-81 years. Three were treated with vemurafenib and one each with nivolumab, ipilimumab, and osimertinib. The oncological diagnoses were metastatic thyroid carcinoma, pleomorphic xanthoastrocytoma, metastatic melanoma, adenocarcinoma of the lung, and metastatic breast cancer. Ocular manifestations appeared 4-82 weeks after the biological treatment was initiated. The most common ocular presentation was anterior uveitis. Onset was sudden in all cases. The median duration of uveitis was 70 weeks. Treatment included topical or systemic corticosteroids; one patient received a single intravitreal steroid injection in one eye.Conclusions: Uveitis may rarely be induced by treatment with biological agents for cancer. Both oncologists and ophthalmologists should be aware of this potential side effect. Early detection and management can prevent permanent complications and save the patient's vision.

When pediatric acute acquired comitant esotropia is not caused by a neurological disease.

BACKGROUND: Acute acquired comitant esotropia (AACE) is a relatively rare type of pediatric strabismus, often described as a possible presentation of intracranial pathology. The risk of having neurological disease in isolated AACE is not clear, because many previously published cases had other neurological or ophthalmological abnormalities. The purpose of this study was to analyze the incidence of neurological abnormalities in children presenting with AACE and otherwise normal neurological and ophthalmological evaluations. METHODS: The medical records of consecutive patients >4 years of age with AACE examined by a single practitioner from 2014 to 2018 were reviewed retrospectively. The main outcome measure was the presence of neurological disease. Children with duction deficits, incomitant esodeviations, and hyperopia of >2.00 D were excluded. RESULTS: A total of 20 children (11 males; mean age, 9.8 ± 4.1 years) were included. Mean esodeviation was 29.5Δ ± 14.8Δ (range, 10Δ-55Δ). All had an otherwise normal ophthalmological and neurological evaluations. Of the 20, 19 (95%) had normal brain neuroimaging. One child that did not have neuroimaging was followed over 2 years without developing any neurological sequelae. CONCLUSIONS: In our study cohort, pediatric AACE not accompanied by other ophthalmic and neurological abnormalities was not a manifestation of intracranial pathology. In such cases, the decision to perform neuroimaging should take into account other factors, including caregivers' preferences and availability for close monitoring.

Evaluation of ocular motility deviation changes in exotropic patients after cycloplegic eye drops versus prism adaptation test.

PURPOSE: To evaluate changes in ocular motility deviation with cycloplegic eye drop examination compared to the prism adaptation test in patients with strabismus. METHODS: The medical charts were reviewed of all patients who underwent primary strabismus surgery in our center from December 2013 to July 2015. Data collected included demographics, medical history, and findings on pre-operative ophthalmic/orthoptic examination. Ocular motility deviation was measured before instillation of cycloplegic eye drops, immediately after maximal dilation (end point), and 10 and 20 min later. Prism adaptation test readings were taken at baseline, immediately after prism removal (end point), and 10 and 20 min later. RESULTS: A total of 43 patients had complete pre- and post-operative evaluations. Our analysis focused only on the exotropic patients (n = 33). On cycloplegics, there was no significant difference in ocular motility deviation between baseline and end point for distance and near (p = 0.584, p = 0.468, respectively). On prism adaptation test, comparison of ocular motility deviation between baseline and end point was statistically significant for distance and near (p = 0.002, p = 0.001, respectively). Changes remained significant 10 min after the end point for near (p = 0.011). Comparison at the end points between the tests revealed statistical significance for distance and near, favoring the prism adaptation test (p = 0.001 and p < 0.001, respectively). This significance was maintained even after 10 min for near (p = 0.036). CONCLUSION: The prism adaptation test is preferred over cycloplegic eye drops for the evaluation of maximal reserve of distance/near motility before surgical correction of exotropia.

The effect of reduced contrast sensitivity on colour vision testing.

OBJECTIVE: To assess the effect of reduced contrast sensitivity on three commonly used colour vision tests in order to establish key discrepancies that may be relevant for clinical practice. METHODS: A prospective non-interventional clinical study of colour vision testing using three commonly used devices: Ishihara and Hardy-Rand-Rittler (H-R-R) pseudoisocochromatic plate tests, and Farnsworth D-15 arrangement test performed under progressively reduced contrast sensitivity conditions achieved with a neutral density filter bar. RESULTS: The Pelli-Robson contrast sensitivity (PRCS) at which 5% of the population should first experience a 10% reduction in colour vision testing from baseline was calculated for each of the three colour vision devices: Farnsworth D-15 test: 1.81 log contrast sensitivity (CS), H-R-R test: 1.69 log CS, and Ishihara test: 1.34 log CS. Single factor repeated measures analyses, conducted separately at each contrast sensitivity level, revealed no difference between the colour vision testing devices at PRCS ≥1.80 log CS (P ≥ 0.367). However, in all PRCS ≤1.65 log CS, the differences were statistically significant (all P ≤ 0.004), demonstrating a significantly lower percentage of errors in the Ishihara test compared with both the Farnsworth D-15 (P < 0.023) and H-R-R (P < 0.035) tests. CONCLUSIONS: At high contrast sensitivities, all colour vision tests function almost equally; however, at decreased levels of contrast sensitivity, H-R-R and Farnsworth D-15 are more greatly affected.

Visual and Clinical Outcome of Macular Edema Complicating Pediatric Noninfectious Uveitis.

PURPOSE: To investigate the clinical course and visual outcome of macular edema (ME) in pediatric patients with chronic noninfectious uveitis. DESIGN: Retrospective case series. METHODS: The databases of the uveitis clinics of 4 tertiary medical centers in Israel and the UK were searched for all children treated for uveitic ME in the years 2005-2015. Data were collected from the medical records as follows: demographics, diagnosis, visual acuity, clinical and imaging findings, and treatment given specifically for ME. Findings at baseline and at 3, 6, 12, and 24 months were evaluated. RESULTS: The cohort included 25 children (33 eyes) of mean age 8.5 ± 3.4 years. The most common diagnosis was intermediate uveitis, in 14 children (7 idiopathic, 7 pars planitis). Uveitis was active at ME diagnosis in 28 eyes (84.8%). Median duration of follow-up was 48 months. Median time to resolution of ME was 6 months, with complete resolution in 25 eyes (75.8%) by 24 months. Baseline visual acuity was ≥20/40 in 8 eyes (24.2%), increased to 57.6% at 3 months (P < .0001), and remained stable thereafter. Treatment regimens included corticosteroids (systemically and/or locally), immunosuppression, and biologic therapies. No correlation was found between outcome and either structural characteristics of ME or specific treatment strategy. CONCLUSIONS: The prognosis of pediatric uveitic ME is favorable despite its chronic course. Larger randomized controlled trials are needed to define differences among treatment regimens.

Horizontal Deviations in Congenital Brown Syndrome.

PURPOSE: To report the incidence of horizontal deviations requiring surgical correction in patients with congenital Brown syndrome. METHODS: In a retrospective study, the medical records of all children who underwent a surgical correction of congenital Brown syndrome at Schneider Children's Medical Center of Israel from 1998 to 2016 were reviewed, analyzing the presence of preoperative primary position horizontal misalignment. RESULTS: Overall, 19 eyes (8 right and 11 left eyes) of 16 patients (7 males, 9 females; mean age: 4.2 ± 2.6 years) were included in this study. Fourteen patients (88%) had surgery for correction of a compensatory head position, including 8 patients (50%) with a head tilt and 6 patients (38%) with a chin-up position, and 2 patients had surgery for primary position hypotropia. All of them underwent a weakening procedure of the superior oblique tendon, by either Z-tenectomy (81%, n = 13) or suture elongation of the superior oblique tendon (19%, n = 3). Fifty-six percent of patients (n = 9) had primary position horizontal deviation before surgery, including 50% (n = 8) exodeviations, ranging from exophoria of 4 prism diopters (PD) to exotropia of 30 PD, and one esotropia of 14 PD. Fifty percent of patients (n = 8) had surgery to correct the horizontal deviation by a recession of either one (31%, n = 5) or two (19%, n = 3) muscles. Mean preoperative horizontal deviation (9.3 ± 3.4 PD) decreased significantly following surgery (1.7 ± 1 PD, P = .001) (paired t test). CONCLUSIONS: Significant horizontal misalignment is often present in patients with congenital Brown syndrome and its correction should be considered at the time of surgery. [J Pediatr Ophthalmol Strabismus. 2018;55(2):113-116.].