Postoperative outcomes for unilateral congenital trochlear nerve palsy: A retrospective cohort study.

PURPOSE: Congenital trochlear nerve palsy is the most common cause of vertical strabismus. The goal of this study was to investigate surgical outcomes after superior oblique tendon plication with or without inferior oblique recession in children and adults with unilateral congenital trochlear nerve palsy. METHODS: Data and outcomes were collected in patients with a diagnosis of unilateral congenital superior oblique palsy during a retrospective single-center study conducted at the University Hospital of Tours. A reproducible, standard ophthalmological and oculomotor examination was performed pre- and postoperatively at 1 year, including presence or absence of diplopia, vertical and horizontal deviations, and compensatory head posture. Surgical success, defined as an endpoint including absence of diplopia in primary position, absence of head tilt, and vertical deviation at distance fixation<5 prism diopters (PD), was analyzed. RESULTS: A total of fifty-seven patients (median [IQR] age of 11 years [5-42]) were analyzed. Patients experienced a significant reduction in vertical distance and near deviations (p<0.001), compensatory head tilt (p < 0.001), and diplopia after surgery (p < 0.001). Surgical success was higher in adults (17/24, 70.8%) than in children (15/33, 45.5%), although this did not reach statistical significance (p=0.0657). CONCLUSION: This study suggests that plication of the superior oblique muscle tendon, with or without recession of the inferior oblique muscle, can be effective in treating unilateral congenital trochlear nerve palsy. Further studies are necessary to compare surgical procedures and investigate their efficacy in adults compared to children in the short and long term.

Congenital Fibrosis of Extraocular Muscles: A Systematic Review and Meta-Analysis.

PURPOSE: To quantify typical disease characteristics for patients with congenital fibrosis of the extraocular muscles (CFEOM) including pre-and post-surgical ocular alignment and head positioning as well as average improvement with surgical intervention. PATIENTS AND METHODS: This investigation is a systematic review and meta-analysis. All studies detailing surgical intervention in cohorts of individuals with CFEOM were included. Ocular alignment and head positioning before and after surgery was recorded. Meta-analyses were performed to quantify these findings along with other patient characteristics across all included studies. RESULTS: The estimated average improvement in vertical alignment for patients with unilateral and bilateral CFEOM was 34.8 pd (prism diopters) and 21.27 pd, respectively. For horizontal alignment, patients with unilateral and bilateral CFEOM improved an average of 25.39 pd and 10.99 pd after surgery, respectively. There was no significant difference between the two most common surgeries used to address hypotropia and blepharoptosis in this patient population. The estimated proportion of patients with post-surgical head alignment within 5-9° was 60.64% and was 28.25% for patients with<5° of alignment. CONCLUSION: This study was able to illuminate many characteristic features of patients with CFEOM. Surgical intervention for the ocular alignment and head positioning of these patients demonstrates promising utility.

A Rare Case of Congenitally Acquired Ocular Toxocariasis in A Five-Week-Old Infant.

PURPOSE: To report a case of strabismus in a five-week-old infant, likely secondary to a rare occurrence of congenitally acquired ocular toxocariasis. METHODS: Retrospective case report. RESULTS: A five-week-old male infant with left exotropia was referred to pediatric ophthalmology and to a vitreoretinal specialist. Fundoscopic examination revealed a granuloma with associated retinal folds and tractional retinal detachment typical for ocular toxocariasis. Serology revealed positivity for Toxocara antibodies, consistent with the clinical diagnosis of ocular toxocariasis. CONCLUSION: Ocular toxocariasis is typically thought to be secondary to acquired Toxocara infection secondary to fecal-oral transmission. In this case of early-onset strabismus secondary to ocular toxocariasis, it is hypothesized that this is a presentation of congenitally acquired toxocariasis.

Horizontal Gaze Palsy, Scoliosis, and Split Pons Sign in a 6-Year-Old Girl.

ABSTRACT: A 6-year-old girl presented with complaints of absent horizontal eye movements since birth. There was also associated progressive scoliosis for past 1 year. Neuroimaging revealed split pons sign, butterfly-shaped medulla, and prominent inferior olivary nuclei. The presence of congenital horizontal gaze palsy, childhood onset progressive scoliosis, and abnormal neuroimaging findings confirmed the diagnosis of horizontal gaze palsy with progressive scoliosis. This case highlights the importance of neuroimaging in a child presenting with horizontal gaze palsy and scoliosis that helped for starting early rehabilitation of the child, prevention of permanent vision loss, and parental counseling for future pregnancies.

Surgical outcomes after unilateral horizontal rectus surgery in children with infantile esotropia for the correction of A or V pattern.

PURPOSE: The management of A or V pattern deviation associated with esotropia can be challenging since the horizontal deviation changes with position of gaze. This study aimed to assess the effect of unilateral horizontal rectus surgery for the correction of horizontal deviation associated with A or V pattern in children with non-comitant infantile esotropia. METHODS: Twenty-seven children with infantile esotropia and A-V pattern, more than 10 and 15 prism diopters respectively, were included in this retrospective observational single-center study. Horizontal rectus surgery was performed on the most deviated eye under general anesthesia. The patients were divided into two groups: A pattern and V pattern. The outcome measures were change in the amount of pattern and rate of regression after surgery. The amount of pattern was characterized by the difference in esodeviation between upgaze and downgaze. RESULTS: Horizontal deviation at distance and near fixation decreased significantly (P<0.0001). Vertical gaze esotropia disparity decreased significantly (P=0.01 and P=0.0002 for A and V patterns respectively). A pattern esotropia was reported in only 2 (7%) cases after surgery compared to 9 (33%) before surgery. The number of subjects with V pattern esotropia decreased from 18 (67%) to 3 (11%) after surgery. CONCLUSIONS: The mechanisms involved in the pathophysiology of A and V patterns may not always be related to oblique muscle dysfunction. These findings suggest that unilateral horizontal rectus surgery may be an effective procedure to correct both horizontal deviation and A-V pattern in non-comitant infantile esotropia.

Congenital Ocular Neuromyotonia with Partial Third Nerve Palsy.

PURPOSE: We report the first case of congenital ocular neuromyotonia (ONM) and the results of strabismus surgery for this patient's co-existing cranial nerve (CN) III palsy. PATIENTS AND METHOD: The patient presented at 18 months with strabismus that had reportedly been present since the time of birth. On exam, she had persistent exotropia (RXT) and hypertropia (RHT) with episodes of esotropia in the right eye that could be evoked by sustained left gaze. A diagnosis of ONM with partial CN III palsy was made. T1-weighted, T2-weighted, and fluid-attenuated inversion recovery magnetic resonance imaging failed to reveal intracranial pathology. RESULTS: Gaze induced intermittent esotropia resolved with carbamazepine. Surgery was performed to improve the patient's RXT and RHT. Post-operatively, the patient's RXT had improved from 12 to 15 prism diopters (∆) at near and 20∆ at a distance to 10∆ RXT at near with no horizontal deviation at distance. Her deviation has remained stable for 13 years, as has her neurological exam and good state of health. CONCLUSION: This case demonstrates that ONM may present congenitally and adds to the body of knowledge regarding surgical outcomes on concurrent CN palsies in these patients.

Clinical evidence supporting the use of donor sclera as spacer material in complicated cases of strabismus surgery - retrospective evaluation of surgical results in 117 patients with thyroid-associated ophthalmopathy or congenital strabismus.

PURPOSE: To examine the outcome of the use of donor sclera as spacer material in complicated cases of strabismus surgery. METHODS: A retrospective cohort study on patients with complicated thyroid-associated ophthalmopathy (TAO) (n = 42) or congenital strabismus (n = 75). Patients underwent strabismus surgery during 1994-2014. The surgical results were evaluated in terms of the reduction in the angle of deviation, the need for re-operation, complications and patient satisfaction. RESULTS: The majority of the TAO patients underwent surgery for vertical strabismus (n = 25). The vertical angle of deviation in the primary position was significantly reduced from a median of 23 prism dioptres (PD) to 2 PD (n = 35, p < 0.001). The horizontal angle of deviation in primary position for TAO patients with esotropia was significantly reduced, from a median of 35 PD to 2 PD (n = 17, p < 0.001). The majority of the congenital cases had horizontal strabismus (esotropia = 29, exotropia = 27). The angle of deviation in esotropia was reduced from a median of 29 PD to 8 PD (n = 36, p < 0.001) and in exotropia from 30 PD to 10 PD (n = 34, p < 0.001). Most of the patients were satisfied with the outcome of surgery, and only 12% required re-operation within 2 years. CONCLUSION: This is the first clinical study on the use of donor sclera as spacer material in complicated cases of strabismus surgery. The surgical results were good in terms of the reduction in the angle of deviation, the need for re-operation, complications and patient satisfaction, supporting the use of donor sclera for strabismus surgery.

Sutura de tração permanente para tratamento de paralisia congênita de III nervo / Permanent treatment suture for the treatment of congenital nerve palsy of III nerve

Resumo A paralisia do terceiro nervo craniano representa o estrabismo paralítico de tratamento mais complexo e desafiador. Os casos de paralisia completa III par incitam o uso de certas técnicas de cirurgia de estrabismo destinadas a manter o olho voltado para a posição primária do olhar (PPO). Entretanto, as possibilidades terapêuticas são limitadas e complexas e o tratamento cirúrgico tende a hipocorreção e recorrências frequentes a longo prazo.O envolvimento completo e congênito do terceiro nervo craniano requer cirurgias para a exotropia, hipotropia e ptose.Dentre as técnicas cirúrgicas já descritas, optou-se pela realização de uma modificação da técnica cirúrgica de recuo-ressecção, que deu-se em único tempo cirúrgico, sendo suficiente para alcançar o objetivo estético. Este trabalho relata o resultado positivoda manutenção de sutura de tração à carúncula para tratamento cirúrgico de estrabismo paralítico congênito de nervo oculomotor de longa data.

Abstract Paralysis of the third cranial nerve represents the most complex and challenging paralytic squint. The cases of complete III nerve paralysis encourages the use of certain strabismus surgery techniques in order to keep eye in primary position of gaze. However, the therapeutic possibilities are limited and complex and the surgical treatment tends to hypocorrection and frequent recurrences in the long term. Complete and congenital involvement of the third cranial nerve requires surgeries for exotropia, hypotropia and ptosis. Among the surgical techniques already described, we choose a modification of the surgical technique of recession-resection, which occurred in a single surgical time, being suffice to achieve aesthetic objective. This paper reports the positive result of the maintenance of caruncle traction suture as surgical treatment of congenital III nerve paralysis.

The visual system in infants with microcephaly related to presumed congenital Zika syndrome.

PURPOSE: To describe and analyze ocular features in infants with microcephaly due to presumed congenital Zika syndrome. METHODS: Ophthalmologic evaluation, including indirect ophthalmoscopy and eye fundus imaging, visual acuity testing with Teller Acuity Cards, and strabismus assessment were performed in infants with microcephaly at a nongovernmental organization clinic for visually disabled children. RESULTS: A total of 70 infants with microcephaly were referred to the clinic. Of these, 25 (mean age, 3 months; 14 males) had ophthalmologic changes: 18 (26%) had intraocular abnormalities, including macular chorioretinal atrophy, mottled retinal pigment epithelium and optic nerve pallor; 7 patients (10%) had strabismus or nystagmus without intraocular abnormalities. Visual acuity was below normal range in all 11 infants tested. CONCLUSIONS: Ophthalmologic abnormalities occurred in 36% of the patients. Macular circumscribed chorioretinal atrophy, focal mottled retinal pigment epithelium, optic nerve pallor, early-onset strabismus, nystagmus and low visual acuity were common ophthalmological features in infants with microcephaly due to presumed congenital Zika syndrome.

Visual impairment in children with congenital Zika syndrome.

PURPOSE: To describe the visual impairment associated with ocular and neurological abnormalities in a cohort of children with congenital Zika syndrome (CZS). METHODS: This cross-sectional study included infants with microcephaly born in Pernambuco, Brazil, from May to December 2015. Immunoglobulin M antibody capture enzyme-linked immunosorbent assay for the Zika virus on the cerebrospinal fluid samples was positive for all infants. Clinical evaluation consisted of comprehensive ophthalmologic examination including visual acuity, visual function assessment, visual developmental milestone, neurologic examination, and neuroimaging. RESULTS: A total of 32 infants (18 males [56%]) were included. Mean age at examination was 5.7 ± 0.9 months (range, 4-7 months). Visual function and visual developmental milestone could not be tested in 1 child (3%). Visual impairment was detected in 32 infants (100%). Retinal and/or optic nerve findings were observed in 14 patients (44%). There was no statistical difference between the patients with ocular findings and those without (P = 0.180). All patients (100%) demonstrated neurological and neuroimaging abnormalities; 3 (9%) presented with late-onset of microcephaly. CONCLUSIONS: Children with CZS demonstrated visual impairment regardless of retina and/or optic nerve abnormalities. This finding suggests that cortical/cerebral visual impairment may be the most common cause of blindness identified in children with CZS.

Idiopathic Unilateral Enlargement of the Extraocular Muscles in an Infant.

A 6-month-old boy presented with a unilateral motility deficit of the right eye in all fields of gaze. Neuroimaging revealed unilateral enlargement of the medial, lateral, and inferior rectus muscles with sparing of the tendons. An evaluation for thyroid eye disease, idiopathic orbital inflammation, myositis, inflammatory and neoplastic infiltration of the muscle, vascular anomalies, and metastatic neuroblastoma was unrevealing. Biopsy of the muscle revealed normal architecture with an absence of inflammation, infiltration, or fibrosis. A review of the literature reveals the exceptionally rare nature of this finding. While the authors cannot rule out an atypical case of congenital euthyroid eye disease, this constellation of findings is not consistent with thyroid eye disease and may represent previously described cases of idiopathic enlargement of the extraocular muscles.

Balance in subjects with congenital or early onset strabismus: Influence of age.

Few studies have investigated the relationship between strabismus and balance, and those that do exist focused on patients within a limited age range, while no studies on possible age-related changes have yet been conducted. Therefore, the aim of our study was to investigate whether the balance strategies adopted by patients with congenital or early onset strabismus change with age. Forty strabismic patients and 36 healthy subjects were enrolled in the study. Both patients and healthy subjects were divided into three subgroups according to age (children, adolescents, and adults) and underwent a stabilometric evaluation. When we compared the whole group of strabismic patients with the group of healthy subjects, we found that the center of pressure area and the trunk oscillations in the former were significantly different from those in the latter; when we considered the three age groups separately, only values in children with strabismus were different from those in the age-matched control group of healthy subjects. Strabismus was found to affect balance in children by inducing a postural strategy characterized by a reduction in physiological trunk oscillations. Gaining a better insight into postural control in strabismic subjects and its evolution with age may be crucial to improving rehabilitation in such patients and planning tailored rehabilitation treatment.

Convergence and Accommodation Development Is Preprogrammed in Premature Infants.

PURPOSE: This study investigated whether vergence and accommodation development in preterm infants is preprogrammed or is driven by experience. METHODS: Thirty-two healthy infants, born at mean 34 weeks gestation (range, 31.2-36 weeks), were compared with 45 healthy full-term infants (mean 40.0 weeks) over a 6-month period, starting at 4 to 6 weeks postnatally. Simultaneous accommodation and convergence to a detailed target were measured using a Plusoptix PowerRefII infrared photorefractor as a target moved between 0.33 and 2 m. Stimulus/response gains and responses at 0.33 and 2 m were compared by both corrected (gestational) age and chronological (postnatal) age. RESULTS: When compared by their corrected age, preterm and full-term infants showed few significant differences in vergence and accommodation responses after 6 to 7 weeks of age. However, when compared by chronological age, preterm infants' responses were more variable, with significantly reduced vergence gains, reduced vergence response at 0.33 m, reduced accommodation gain, and increased accommodation at 2 m compared to full-term infants between 8 and 13 weeks after birth. CONCLUSIONS: When matched by corrected age, vergence and accommodation in preterm infants show few differences from full-term infants' responses. Maturation appears preprogrammed and is not advanced by visual experience. Longer periods of immature visual responses might leave preterm infants more at risk of development of oculomotor deficits such as strabismus.

Congenital cranial dysinnervation disorder in a boy with congenital mirror movements.

"Mirror movements" are an axonal guidance disorder that consists of involuntary contralateral movements that mimic unilateral intentional ones, typically involving the fingers of the hand. They can be isolated or associated with conditions such as Klippel-Feil syndrome, Kallmann syndrome, or congenital hemiplegia. Isolated congenital mirror movements are sometimes caused by autosomal dominant mutation in the genes DCC or RAD51. At least 4 previously reported cases had strabismus, 3 with Moebius syndrome and 1 with Duane retraction syndrome. We report the case of a boy with an unusual incomitant strabismus consistent with orbital dysinnervation and suggest that for some patients with congenital mirror movements the neurological miswiring extends to the orbit, causing congenital cranial dysinnervation disorder.

Manejo multidisciplinario en retinoblastoma: experiencia en 37 ojos / Multidisciplinary management of retinoblastoma: Experience in 37 eyes

OBJETIVOS: Reportar los resultados del manejo multidisciplinario de pacientes con retinoblastoma, incluyendo supervivencia global, tasa de enucleación y éxito de la quimiorreducción sistémica. MÉTODOS: Estudio retrospectivo 27 pacientes (37 ojos) con diagnóstico de retinoblastoma, tratados por un equipo multidisciplinario en el Hospital San Juan de Dios. Se incluyeron información demográfica, características clínicas, supervivencia, tratamiento local y sistémico. Se realizó además un subanálisis de tratamiento con quimioterapia intraarterial (QIA). RESULTADOS: Catorce pacientes (52%) fueron de sexo masculino. La mediana de edad al ingreso fue de 8 meses (0,16-90). La mediana ± desviación estándar de seguimiento fue de 33 ± 21 meses. Diez casos (37%) se diagnosticaron después de los 15 meses de edad, con una mediana de 35 meses (24-90). En 17 (63%) pacientes fueron retinoblastomas unilaterales, mientras 10 (37%) tuvieron retinoblastoma bilateral. La leucocoria, aislada o asociada a otros signos, fue el motivo de consulta más frecuente (63%). La tasa global de enucleación fue del 57% (n = 21), siendo el tratamiento primario en 15 (55%) pacientes. La tasa de enucleación en retinoblastoma unilateral fue del 76,5% y en retinoblastoma bilateral del 60% de un ojo y el 10% de ambos. Diecisiete (63%) pacientes recibieron quimioterapia sistémica (media de ciclos: 5,3 ± 2,1). El éxito global de la quimiorreducción sistémica y la terapia focal fue del 68%. Tres pacientes fueron tratados con QIA como terapia de rescate, logrando controlar el tumor en 2 pacientes a 6 meses de seguimiento, siendo los primeros casos en Chile. La supervivencia fue del 100%. CONCLUSIÓN: El manejo multidisciplinario del retinoblastoma permite una supervivencia y una morbilidad comparable con la literatura internacional

OBJECTIVE: To report the results of the multidisciplinary management of patients with retinoblastoma, including survival, enucleation rate, and systemic chemoreduction success. METHODS: A retrospective study was conducted on 27 patients (37 eyes) diagnosed with retinoblastoma, and treated by a multidisciplinary team in San Juan de Dios Hospital. Demographic information, clinical characterization, survival, local and systemic treatments were included in the analysis. Patients treated with intra-arterial chemotherapy (IAC) were also reviewed. RESULTS: The study included14 male patients (52%). The median of age at presentation was 8 months (0.16-90). The median follow-up time was 33 ± 21 months. The diagnosis was made in 10 (37%) cases after 15 months old, with a median of 35 months (24-90). 17 (63%) patients had unilateral retinoblastoma, and 10 (37%) bilateral retinoblastoma. Leukocoria, isolated or associated with other signs, was the most frequent reason for referral (63%). Global enucleation rate was 57% (n = 21), being the primary treatment in 15 (55%) patients. Enucleation rate in unilateral retinoblastoma was 76.5%, and for bilateral retinoblastoma, it was 60% for one eye and 10% for both. Systemic chemotherapy was prescribed in 17 (63%) patients, with a mean number of cycles of 5.3 ± 2.1. The overall success of chemoreduction and focal therapy in order to avoid external radiotherapy and/or enucleation was 68%. Three patients were treated with IAC as a salvage therapy, controlling the tumor in 2 patients at 6 months of follow-up. These are the first cases reported in Chile. Survival rate was 100%. CONCLUSION: Multidisciplinary management of retinoblastoma led to a survival rate and morbidity comparable with international reports

Congenital fibrosis of the extraocular muscles: magnetic resonance imaging findings and surgical treatment.

PURPOSE: We analyzed findings of orbital and cranial magnetic resonance imaging (MRI) in patients with congenital fibrosis of the extraocular muscles (CFEOM). We described surgery and its outcome. MATERIAL AND METHOD: Nine out of 10 patients with clinical findings of CFEOM underwent orbital and cranial MRI to perform a study of the extraocular muscles and cranial nerves. A multimodality workstation platform developed by the imaging laboratory of our hospital for PC computer allowed us to visualize and measure the cross sections of the extraocular muscles in a coronal section. Surgery was indicated to resolve strabismus. Outcome was considered favorable if the final deviation was < 10 pd in the primary position without head turn. RESULTS: In 8 cases (6 males, 5 unilateral [3 left eye]), MRI revealed atrophy of at least 1 of the extraocular muscles supplied by the third nerve. Five patients had third nerve aplasia or hypoplasia. Clinical findings were compatible with a probable diagnosis of CFEOM in all 10 patients. Four patients underwent ptosis surgery before being diagnosed with CFEOM. Four patients underwent surgery to correct strabismus and, of these, 2 required multiple interventions (1 needed 4 interventions). Outcome was successful in only 2 cases. CONCLUSION: Orbital and cranial MRI provided useful information about extraocular muscles and cranial nerves in CFEOM. Surgery must be performed on an individual basis; the number of reoperations is high. The outcome of surgery was favorable in half of the cases.