Acute acquired comitant esotropia associated with Lhermitte-Duclos disease: a case report.

BACKGROUND: Acute acquired comitant esotropia caused by prolonged near work, such as the use of digital devices, has been frequently reported in recent years. However, intracranial examination is necessary even for patients with nonparalytic comitant esotropia. Lhermitte-Duclos disease is a rare tumor that grows in layers in the cerebellum. Among those with this disease, cases of esotropia have been reported due to abduction limitation of the eye, but there have been no reports of comitant esotropia. Here, we report the case of a young woman with acute acquired comitant esotropia who was found to have Lhermitte-Duclos disease. CASE PRESENTATION: A 16-year-old Japanese female patient, whose ethnicity was Asian, was referred to our hospital for acute acquired comitant esotropia. Fundus examination revealed papilledema in both eyes, and magnetic resonance imaging of the head revealed a cerebellar tumor in the right cerebellum with obstructive hydrocephalus. She underwent partial tumor resection, and a histopathological diagnosis of Lhermitte-Duclos disease was obtained. However, comitant esotropia status remained unchanged, and she underwent strabismus surgery. Finally, diplopia disappeared completely. CONCLUSION: Neurological and intracranial imaging examinations are essential when acute acquired comitant esotropia is observed. Acute acquired comitant esotropia by Lhermitte-Duclos disease did not improve with partial tumor resection and required strabismus surgery, but good surgical results were obtained.

Neonatal Macular Hemorrhage in a Term Healthy Infant: 18-Year Follow-up.

Unobstructed binocular vision is required during the critical period of vision development to obtain optimal visual acuity in each eye and binocular stereopsis. In this article, we report 18-year follow-up of a full-term, otherwise healthy infant noted to have dense premacular hemorrhage occluding the visual axis in the left eye on retinal imaging performed 48 hours after birth. Serial examinations by the retina service were performed weekly for 10 weeks as the hemorrhage resolved spontaneously. Shortly thereafter, visual acuity revealed fixation was present, but the mother noted intermittent left eye esodeviation. At 90 days of life, the infant was seen by pediatric ophthalmology and started on 1 to 2 hours patching of the right eye daily for the esotropia, which was maintained through 24 months. At 18 years of age, the patient had orthophoria alignment, no spectacle correction, vision of 20/20 in the right eye and 20/25 in the left eye, and normal binocularity and stereopsis. Intermittent esodeviation on the left eye was observed when the patient was fatigued. Early identification of a visual axis occlusion led to prompt referral for the esotropia and initiation of patching therapy. This patient ultimately achieved a very favorable visual functional outcome that may not have been possible without early detection and management. This case report describes the longest-term published follow-up of a neonatal macular hemorrhage. [Ophthalmic Surg Lasers Imaging Retina 2024;55:354-357.].

The incidence, clinical features, and management of essential infantile esotropia in the United Kingdom. A British Ophthalmology Surveillance Unit (BOSU) study.

BACKGROUND/OBJECTIVES: A national study was undertaken through the British ophthalmology surveillance unit (BOSU) to determine the incidence, presenting features and management of essential infantile esotropia (EIE) in the UK. METHODS: Data from a prospective national observational study of newly diagnosed EIE presenting to clinicians in the United Kingdom over a 12-month period were collected. Cases with a confirmed diagnosis by a clinician of a constant, non-accommodative esotropia ≥20 prism dioptres (PD), presenting at ≤12 months, with no neurological or ocular abnormalities were identified through BOSU. Follow-up data were collected at 12 months. RESULTS: A total of 57 cases were reported giving an incidence of EIE of 1 in 12,828 live births. The mean age of diagnosis and intervention were 7.05 ± 2.6 months (range 2-12) and 14.7 ± 4.9 months (range 6.5-28.1), respectively. Management was surgical in 59.6%, botulinum toxin alone in 22.8%, and 17.5% were observed. The preoperative angle of esotropia was smaller in the observation group (P = 0.04). The postoperative angle of esotropia was not statistically significant between botulinum toxin or surgery (P = 0.3), although the age of intervention was earlier in the botulinum group (P = 0.007). Early intervention (before 12 months of age) did not influence the post-intervention motor outcomes between 0 and 10 prism dioptres of esotropia (P = 0.78). CONCLUSIONS: The incidence of EIE in the UK is considerably lower than reported in other population-based studies. The preferred method of treatment was surgical with earlier intervention in those treated with botulinum toxin. An early age of intervention (<12 months) did not influence motor outcomes.

12†The incidence, clinical features, and management of essential infantile esotropia in the United Kingdom. A British Ophthalmology Surveillance Unit (BOSU) study - Final Report.

Studies from the UK have reported declining rates of surgery for childhood esotropia. It is not known if this equates to a reduced incidence of essential infantile esotropia (EIE). A national study was undertaken through the British ophthalmology surveillance unit (BOSU) to determine the incidence presenting features and management of EIE in the UKData from a prospective national observational cohort of newly diagnosed EIE presenting to clinicians in the United Kingdom over a 12-month period was collected. Cases with a confirmed diagnosis by a clinician of a constant, non-accommodative esotropia ≥ 20 prism dioptres (PD), presenting at ≤ 12months, with no neurological or ocular abnormalities were identified through BOSU. Follow up data was collected at 12 months. Data was collected on the age, gender, ethnicity, birth history, age at diagnosis, age at intervention, angle of esotropia, refraction, associated features of amblyopia, overelevation in adduction (OEIA), latent nystagmus and dissociated vertical deviation (DVD), method of management and outcomes.During the period of observation between October 2017 to October 2018 a total of 57 cases were reported giving an incidence of EIE of 1 in 12,828 live births with a corrected incidence of 1 in 9027 live births allowing for estimated under reporting. The mean age of diagnosis and intervention were 7.05± 2.6 months (range 2 to 12 months) and 14.7± 4.9 (range 6.5-28.1 months) respectively. The majority were Caucasians 86.5% and 52.7% were female. Management was surgical in 59.6%, and botulinum toxin alone in 22.8%, 17.5% were observed. There was no significant difference in the age of presentation (P=0.6), gender (P=0.8), prematurity (P=0.5), deprivation indices (P=0.68), refraction (P=0.7), OEIA (P=0.6), DVD (P=0.7) or follow up (P=0.3) between the three groups. The preoperative angle of esotropia was smaller in the observation group (P=0.04). The post-operative angle of esotropia was not statistically significant between botulinum toxin or surgery (P=0.3) though the age of intervention was earlier in the botulinum group (P=0.007). Early intervention did not influence the motor post intervention outcomes between 0-10 prism dioptres of esotropia (P=0.78). Amblyopia (P=0.02) and latent nystagmus (P=0.009) was more common in the observation group.The incidence of EIE in the UK is considerably lower than reported in other population-based studies. The preferred method of treatment was surgical with earlier intervention in those treated with botulinum toxin. An early age of intervention did not influence motor outcomes. Parental choice and amblyopia treatment were reasons cited for conservative management in the observational group.

Race, Ethnicity, Insurance, and Population Density Associations with Pediatric Strabismus and Strabismic Amblyopia in the IRIS® Registry.

PURPOSE: To evaluate the associations of sociodemographic factors with pediatric strabismus diagnosis and outcomes. DESIGN: Retrospective cohort study. PARTICIPANTS: American Academy of Ophthalmology IRIS® Registry (Intelligent Research in Sight) patients with strabismus diagnosed before the age of 10 years. METHODS: Multivariable regression models evaluated the associations of race and ethnicity, insurance, population density, and ophthalmologist ratio with age at strabismus diagnosis, diagnosis of amblyopia, residual amblyopia, and strabismus surgery. Survival analysis evaluated the same predictors of interest with the outcome of time to strabismus surgery. MAIN OUTCOME MEASURES: Age at strabismus diagnosis, rate of amblyopia and residual amblyopia, and rate of and time to strabismus surgery. RESULTS: The median age at diagnosis was 5 years (interquartile range, 3-7) for 106 723 children with esotropia (ET) and 54 454 children with exotropia (XT). Amblyopia diagnosis was more likely with Medicaid insurance than commercial insurance (odds ratio [OR], 1.05 for ET; 1.25 for XT; P < 0.01), as was residual amblyopia (OR, 1.70 for ET; 1.53 for XT; P < 0.01). For XT, Black children were more likely to develop residual amblyopia than White children (OR, 1.34; P < 0.01). Children with Medicaid were more likely to undergo surgery and did so sooner after diagnosis (hazard ratio [HR], 1.23 for ET; 1.21 for XT; P < 0.01) than those with commercial insurance. Compared with White children, Black, Hispanic, and Asian children were less likely to undergo ET surgery and received surgery later (all HRs < 0.87; P < 0.01), and Hispanic and Asian children were less likely to undergo XT surgery and received surgery later (all HRs < 0.85; P < 0.01). Increasing population density and clinician ratio were associated with lower HR for ET surgery (P < 0.01). CONCLUSIONS: Children with strabismus covered by Medicaid insurance had increased odds of amblyopia and underwent strabismus surgery sooner after diagnosis compared with children covered by commercial insurance. After adjusting for insurance status, Black, Hispanic, and Asian children were less likely to receive strabismus surgery with a longer delay between diagnosis and surgery compared with White children. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Demographic and clinical characteristics of age-related distance esotropia.

BACKGROUND: Age-related distance esotropia (ARDE), is an acquired, small, comitant esodeviation that is greater at distance than at near. It occurs in older adult patients without a history of neurological event or prior strabismus. It has been observed more frequently in White adults than in other racial groups. The purpose of this study was to assess the demographic and clinical characteristics of patients with ARDE presenting at a tertiary neuro-ophthalmology clinic. METHODS: In this retrospective study, ICD-9/10 (ICD-9 378.85 and ICD-10 H51.8) codes were used to identify all patients with ARDE from 2005 to 2020 seen in a single tertiary neuro-ophthalmology clinic. ARDE was defined as esotropia greater at distance than near with associated clinical signs of adnexal tissue laxity. Patients with history or findings compatible with other etiologies of strabismus, such as thyroid eye disease, neuromuscular disorders, sensory deviations, sudden onset of diplopia, and high myopia, as well as those with prior strabismus surgery, were excluded. RESULTS: A total of 89 patients (59 females [66%]) met inclusion criteria. Mean patient age was 76.6 years. All patients were White except for a single patient of African descent. Mean follow-up time was 25.2 months. Mean esodeviation at distance on presentation was 6.6Δ. Of the 87 patients electing nonsurgical treatment, 80 achieved remission of diplopia symptoms with prism therapy alone. Of the 89 patients, 59 had no neuroimaging. CONCLUSIONS: ARDE in our neuro-ophthalmology clinic population was diagnosed almost exclusively in older White adults. Prism therapy was effective for a majority of our patients.

Evaluation of Strabocheck® as an objective measurement method of comitant horizontal strabismus in children undergoing surgery.

The gold standard of angle measurement is the prism alternating cover test (PCT). This method requires cooperation from the child, experience, and presents a significant inter-observer variability. Strabocheck®(SK) is a new simple tool for objective and semi-automated angle measurement. Our purpose is to evaluate Strabocheck® in children undergoing surgery for comitant horizontal strabismus. The study population was divided into 3 groups: infantile esotropia, partially accommodative esotropia and intermittent exotropia. The primary endpoint was the agreement between Strabocheck®and the PCT. A total of 44 children were included prospectively. The correlation between the angle measured by the PCT and the angle measured by SK was strong (R = 0.87). The mean absolute difference in the angle measured by the two methods was Δ = 11.9+/- 9.8 diopters. The Bland-Altman plot shows a 95% interval limit between -30.0 [-34.4; -25.6] and 31.0 [26.7; 35.4] diopters. SK is an interesting tool to evaluate the angle of strabismus in children. However, the residual discordance between PCT and SK leads us to question the real value of the angle, which can only be approximated. A better clinical experience of this new tool in relation to the clinical condition and the PCT will allow to have a more accurate idea on the true angle and will probably help the surgeon to adapt this procedure.

Management of acute acquired comitant esotropia in children.

Aim: This report aims to discuss and review the diagnosis and management of acute acquired comitant esotropia (AACE) in children and to add several cases to the limited literature available on this unusual condition in the pediatric population. Materials and methods: We present two cases of AACE with large-angle deviations that were investigated and followed-up according to current recommendations. Both cases required strabismus surgery for AACE, but different procedures were chosen, with good postoperative results. Results: Unilateral recession of the medial rectus and resection of the lateral rectus (R&R) were performed in one case and bilateral medial rectus (MR) recession in the other, with resolution of the diplopia and full recovery of binocular vision. Discussion: Although isolated AACE is usually benign, studies have reported the presence of intracranial disease in up to 10% of cases, making it a potential first sign of an underlying serious pathology. Therefore, AACE should be investigated as a medical emergency and neuroimaging should be performed in all patients with unclear onset of AACE, as well as in those with associated neurological symptoms, such as headache, cerebellar imbalance, weakness, or nystagmus. Conclusion: Acute acquired comitant esotropia (AACE) is an infrequent type of esotropia that usually appears in older children. It is characterized by esotropia and diplopia with acute onset. Neurological examinations and neuroimaging should be performed to exclude any potential intracranial disease. Treatment of AACE without underlying neurological disease is focused on managing the diplopia and resolving the esotropia. Strabismus surgery has good motor and sensory results and can successfully restore good binocular function. Abbreviations: AACE = Acute acquired comitant esotropia, LR = lateral rectus, MR = medial rectus, PD = prism diopters, R&R = recession and resection, BSV = binocular single vision, PAT = prism adaptation test.

Clinical features, etiological reasons, and treatment results in patients who developed acute acquired nonaccomodative esotropia.

PURPOSE: To evaluate the clinical features, possible etiological reasons, and treatment results in children who developed acute acquired comitant esotropia (AACE) without strabismus in previous years. METHODS: Medical records of the patients who were diagnosed with AACE between July 2017 and June 2021 were retrospectively reviewed. The children with ocular and orbital pathology, hypermetropia > 2.00 diopters, and anisometropia > 1.00 diopters were not included in the study. Possible etiological factors that could cause esotropia, treatment results, motor, and sensory functions were investigated. RESULTS: The mean age at first admission, and the onset of AACE, was 8.8 ± 2.9 (4-13) years of three female (23.1%) and 10 male (76.9%) cases. The causes of AACE were determined to be occlusion of the eye due to corneal foreign body removal in one (7.7%), emotional stress in one case (7.7%), and excessive close work, on computer and smartphone screens in the other 11 cases (84.6%). Orthotropia was achieved in cases who underwent strabismus surgery (n = 10) and in cases using the prism (n = 2); except for one case, all (92.3%) achieved binocular single vision (100 s/arc stereopsis and fusion) after treatment, while there was no binocular single vision in any of the cases before treatment. CONCLUSIONS: Acute acquired comitant esotropia is a rare clinical entity. Successful motor and sensory outcomes can be achieved by strabismus surgery or by prism therapy. It is critical to investigate the patients with AACE in terms of intracranial pathologies, although rarely seen.

Clinical Profile, Neuroimaging Characteristics, and Surgical Outcomes of Patients With Acute Acquired Non-accommodative Comitant Esotropia.

PURPOSE: To report the clinical presentations, neuroimaging findings, and surgical outcomes in patients with acute acquired non-accommodative comitant esotropia (AACE). METHODS: A retrospective review of records of all patients diagnosed as having AACE between January 2011 and December 2019 across three tertiary eye care centers was done. Cases with AACE onset after age 1 year were included. Patients were divided into two groups based on age of onset of esotropia: childhood (16 years or younger) and adult (older than 16 years). Surgical success was defined as postoperative horizontal deviation of 8 prism diopters (PD) or less at the last follow-up visit. RESULTS: A total of 338 patients (220 males and 119 females; mean age at presentation: 12.60 ± 9.8 years) met the study criteria. The mean age at onset of esotropia in the childhood and adult onset groups was 3.61 ± 1.1 and 26.6 ± 8.7 years, respectively. There were significantly more individuals with myopia (30%) in the adult onset group compared to the childhood onset group (6%) (P = .004). Thirty-seven (16%) had positive neuroimaging findings (16.4% adult onset vs 9.4% childhood onset). A total of 148 (44%) patients underwent surgery for esotropia, and the overall success rate was 73%. Mean preoperative esotropia was comparable in either group (P = .20), but surgical success was better in the adult onset group (75.6% vs 66.3% in the childhood onset group). Mean duration of follow-up postoperatively was 13.6 ± 12 months. CONCLUSIONS: Two-thirds of the patients had childhood onset of AACE. Intracranial pathology was found in 1 of 6 patients. Surgical success was better in the adult onset group, which was not influenced by preoperative esotropia, neuroimaging findings, or refractive status, but was dependent on age at onset of esotropia and duration between onset and intervention. [J Pediatr Ophthalmol Strabismus. 2023;60(3):218-225.].

A rare case of intramuscular orbital amyloidosis presenting as progressive esotropia in an adult.

Orbital amyloidosis is an uncommon entity. Lacrimal gland, orbital soft tissue and extraocular muscles are the usual sites of involvement. Deposition of amyloid in the extraocular muscle can be either isolated or associated with systemic amyloidosis. We describe a case of a 43-year-old male with isolated amyloid infiltration of the right lateral rectus muscle presenting as a progressive esotropia. The case was initially misdiagnosed as lateral rectus palsy and myositis due to non-specific radiological features of the entity. The diagnosis was confirmed by a biopsy. Intramuscular Botulinum toxin injection, was administered to the right medial rectus to alleviate diplopia temporarily, and strabismus surgery was planned at a later stage. Amyloid deposition is a rare cause of extraocular muscle enlargement. Histopathology is confirmatory. Screening for systemic amyloidosis is mandatory in all cases.

Four cases of acute comitant esotropia associated with diffuse intrinsic pontine glioma in children.

BACKGROUND: Acute acquired concomitant esotropia (AACE) is usually a benign form of strabismus that infrequently is associated with intracranial pathology. Clinicians have noted an increase in its incidence and theorize that it may be related to public health "lockdown" measures taken in response to the COVID-19 pandemic. With an increased incidence of AACE clinicians must firstly differentiate AACE from common accommodative esotropia and secondly recognize AACE as a possible sign of serious neuropathology.Diffuse Intrinsic Pontine Glioma (DIPG) is a devastating diagnosis for affected families. Children typically present at age 6-7 years with cranial nerve palsies, long tract signs, and/or cerebellar signs. Diagnosis is made from characteristic findings on magnetic resonance brain imaging (MRI brain) and treatment includes radiotherapy and palliative care. Two years from diagnosis, 90% of affected children will have died from their disease. CASE SERIES: We present four cases that attended our pediatric ophthalmology clinic with AACE either as a presenting sign of DIPG or as a clinical finding following a DIPG diagnosis. Patient A (age 5 years) presented to the emergency eye clinic with sudden onset diplopia and intermittent esotropia. Suppression later developed, they had 0.00 logMAR visual acuity either eye, and bilateral physiological hypermetropia. MRI brain imaging requested as a result of the unusual presentation led to the DIPG diagnosis. The other 3 cases (ages 11, 5 & 5 years) were assessed post DIPG diagnosis and found to have an esotropia measuring bigger on 1/3-meter fixation than 6-meter fixation, full ocular motility, physiological hypermetropia or emmetropia, and visual acuity normal for age. Other than patient B (age 11 years), who had papilledema and gaze evoked nystagmus when they were assessed 2 weeks prior to death, no patient had any other clinical eye findings. CONCLUSIONS: This small series of 4 patients attending our clinic within a 12-month period supports the notion that children presenting with AACE should routinely be offered brain MRI. Not all children with DIPG-associated AACE have significant ophthalmic findings indicative of intracranial pathology. With the potential for increased incidence of AACE related to lockdowns, clinicians should be reminded of the infrequent possibility their patient has a more serious condition.

Profile of infantile strabismus at a tertiary eye care center in India.

Purpose: To study the profile, risk factors, and management outcomes of infantile strabismus at a tertiary eye care center. Methods: We prospectively analyzed the data of infants (children less than 1 year of age) who presented at our institute from August 2018 to December 2019. We excluded infants who did not complete a minimum follow-up of 6 months. Detailed meticulous history based on a set of standardized questionnaires was obtained and a comprehensive ophthalmological examination of the child was performed. Data were collected regarding refractive error (astigmatism; myopia; hyperopia; anisometropia [<1.0 DS or >1.0 DS]; astigmatism [<1.0 DS or >1.0 DS]) and the type of strabismus. Results: During this period, we saw 4,773 infants, out of which 123 infants were diagnosed to have infantile-onset strabismus (hospital prevalence of 2.6%). Boys and girls were equally affected. Sixty-two patients had esotropia, 37 had exotropia, 2 had hypotropia, and 22 had pseudo strabismus. Prematurity, hypermetropia, and anisometropia had increased odds of developing esotropia, whereas delivery by cesarean section, delayed cry at birth, infantile seizures, parental consanguinity, delayed development of milestones, and myopia had increased odds of developing exotropia. Twenty-nine patients underwent a surgical correction. The mean deviation at the first visit was 42.59 ± 15.40 PD and 8.25 ± 12.70 PD at the last visit. For all patients who underwent a squint surgery, the change in ocular deviation was clinically and statistically significant (P-value <0.0001, paired t-test). Conclusion: The hospital prevalence of infantile strabismus in our cohort was found to be 2.6%. Our study suggests that esotropia is two-fold more common in our cohort as compared to exotropia. Further, our study highlights risk factors for the development of strabismus in infancy, which must be kept in mind and awareness must be created among pediatricians. Surgical correction should be considered early during the infantile period, because it may lead to promote the development of good binocular vision.

Surgical outcomes of acquired acute comitant esotropia. Causes and classification.

PURPOSE: To study the results of surgical treatment of acquired concomitant esotropia and its posible causes. METHODS: Retrospective study of the patients with acute acquired esotropia that were operated on since 2017. Distance and near horizontal deviation, spherical equivalent and the excessive use of the tablets and smartphones were analyzed. A good sensorial outcome was considered when stereopsis was recovered and good motor outcome if horizontal deviation was ≤10 prism diopters at the end of the follow-up. RESULTS: A total of 15 cases were included (60% women), mean age: 29.07 years (86.66% ≥16 years). Mean time since the beginning of the symptoms to the surgery was 23.3 months. Mean horizontal preoperative deviation was 19.73 ± 7.2 pd at near and 22.93 ± 7.3 dp at distance decreasing to 3.33 ± 3.8 pd at near and 3.86 ± 3.9 pd at distance (P < .001). The 66.6% had diplopía. The 46.66% had decompensated esophoria or microesotropia, 86,66 % myopia and the 33.33% referred excesive use of the tablets and smartphones. The medial rectus recession were done in 10 cases. A good motor outcome was obtained in the 93.33% of the cases and sensorial outcome in the 53.3% without diplopía. CONCLUSION: Surgery was an excellent treatment to resolve the deviation and diplopia. The esophorias and microesotropias represented near the half of the cases and the excesive use of the tablets and smartphones were the third part of the sample. Most of the patients had myopia.

Clinical profile and management of sixth nerve palsy in pediatric patients (0-15 years) in Southern India - A hospital-based study.

PURPOSE: This study was done to evaluate the clinical profile in pediatric patients (0-16 years) presenting with acute onset esotropia due to sixth nerve palsy and its management options in a tertiary care set up of Southern India. METHODS: A total of 12 patients presenting to our OPD with acute onset esotropia due to sixth nerve palsies were included in this retrospective study. All patients were observed for 6 months and managed with prism and/or patching while waiting for spontaneous resolution and later managed surgically. Neuroimaging was done in all cases. RESULTS: The mean deviation of esotropia at presentation was 30.17 ± 5.7 Prism Diopter (range 12-50 Prism Diopter 95% CI, SD 10.11). Mean age of the patients during presentation was 8.6 ± 2.4 years (range: 1-15 years, SD 4.27). Among the common causes of sixth nerve palsy in our study population were trauma and idiopathic intracranial hypertension followed by tumor and miscellaneous causes. Only three patients underwent surgical correction of residual deviation after a waiting period of 6 months for self-resolution. Spontaneous resolution was observed in 41.6% patients, and surgical correction (unilateral resection-recession) was done in 25% of the patients with good surgical outcome. CONCLUSION: At 1-year follow up, the motor outcome was satisfactory except for one patient who had diffuse pontine glioma and had worsening neurological symptoms on follow-up.

Functional convergence spasm: an unexpected finding in a patient with focal epilepsy.

OBJECTIVE: Convergence spasm is a clinical condition characterized by transient episodes of convergence, miosis and accommodation with strabismus and diplopia and it is usually a manifestation of a functional neurological disorder. We describe a patient with a challenging diagnosis of convergence spasm in the setting of occipital lobe epilepsy. CASE REPORT: A 52-year-old woman came for the assessment of focal epilepsy due to left occipital cortical dysplasia. During ocular motility tests, she presented with episodes of short duration (~10-30 seconds) of convergent strabismus. Neuropsychological evaluation showed a severe mixed anxiety-depressive disorder with a tendency toward somatization. RESULTS: Convergence spasm was recorded during video-EEG examination and no ictal activity was present. CONCLUSIONS: To our knowledge, no other report of functional convergence spasm in the context of focal epilepsy associated with cortical dysplasia has been described in literature.

Prevalence of strabismus and its risk factors among school aged children: The Hong Kong Children Eye Study.

The study aims to determine the prevalence of strabismus and its risk factors among school children in Hong Kong. This is a cross-sectional study involving 6-8 year old children from different districts in Hong Kong. 4273 children received comprehensive ophthalmological examination, cycloplegic auto-refraction, best corrected visual acuity (BCVA), anterior segment examination, cover/uncover test, ocular motility, and fundus examination. Demographic information, pre- and post- natal background, parental smoking status, and family history of strabismus were obtained through questionnaires. Strabismus was found among 133 children (3.11%, 95% CI 2.59-3.63%), including 117 (2.74%) exotropia and 12 (0.28%) esotropia cases (exotropia-esotropia ratio: 9.75:1). There was no significant difference in prevalence across age (6-8 years) and gender. Multivariate analysis revealed associations of strabismus with myopia (≤ - 1.00D; OR 1.61; 95% CI 1.03-2.52; P = 0.037) hyperopia (≥ + 2.00D; OR 2.49; 95% CI 1.42-4.39; P = 0.002), astigmatism (≥ + 2.00D; OR 2.32; 95% CI 1.36-3.94; P = 0.002), and anisometropia (≥ 2.00D; OR 3.21; 95% CI 1.36-7.55; P = 0.008). Other risk factors for strabismus included maternal smoking during pregnancy (OR 4.21; 95% CI 1.80-9.81; P = 0.001), family history of strabismus (OR 6.36; 95% CI 2.78-14.50, P < 0.0001) and advanced maternal age at childbirth (> 35 years; OR 1.65; CI 1.09-2.49, P = 0.018). The prevalence of strabismus among children aged 6-8 years in Hong Kong is 3.11%. Refractive errors, family history of strabismus and maternal smoking history during pregnancy are risk factors. Early correction of refractive errors and avoidance of maternal smoking during pregnancy are potentially helpful in preventing strabismus.

[Outcomes of partial vertical rectus transposition in acquired sixth nerve palsy]. / Efficacité de la transposition partielle des muscles droits verticaux dans les paralysies acquises du sixième nerf crânien.

PURPOSE: This study aimed to assess the outcomes of partial vertical rectus transposition (PVRT) in acquired sixth nerve palsy (SNP) on distance ocular deviation in primary position (PP), binocular diplopia in PP, head turn, and limitation of abduction. METHODS: This retrospective, single center study was conducted at the Regional University Medical Center of Tours between 2004 and 2019. All patients underwent comprehensive orthoptic and ophthalmic examination before and after surgery. Total success was defined as horizontal distance deviation in PP≤10 prism diopters (PD), absence of permanent diplopia in PP, head turn≤5 degrees and absence of any induced vertical deviation at last examination. RESULTS: Ten patients underwent PVRT with a median delay of 3.3 years (2.3 to 7.3) following the diagnosis of SNP. The median duration of postoperative follow-up was 13 months (3.25 to 25). Seven patients (70%) were considered a total success. The mean distance horizontal deviation in PP was reduced from 25.4±11.7 PD preoperatively to 9.2±11 PD postoperatively. Preoperatively, 10 patients did not cross midline in abduction, compared to 8 patients at the last follow-up examination. Postoperatively, 3 patients had permanent binocular diplopia in PP, and 2 patients had an anomalous head turn. CONCLUSION: Although further prospective investigations are needed, PVRT can be considered a safe and effective procedure for acquired SNP.

Clinical Classification of Acquired Concomitant Esotropia.

BACKGROUND: Classification and management of acquired concomitant esotropia is controversial. We sought to establish a simple clinical classification in order to determine in which cases further investigations in search of underlying pathologies are necessary. PATIENTS AND METHODS: Observational retrospective study of the files of 175 consecutive patients examined in our unit between 2009 and 2018 for acute convergent strabismus. One hundred and nine patients were selected, after exclusion of infantile, incomitant, or mechanical esotropias, residual esotropias, and patients examined on a single occasion. All patients received a complete orthoptic and ophthalmological examination. We grouped the patients according to their common characteristics. RESULTS: We established the following categories: 1. Acute esotropia of large angle (20 to 45 prism diopters [PD]), accompanied by mild hyperopia (mean 1.2 D) in children (n = 16) and moderate myopia (mean 3.7 D) in adolescents and adults (n = 13). 2. Decompensated micro-esotropia, which is distinguished from the other categories by the presence of abnormal retinal correspondence (n = 33). 3. Decompensated esophoria, with intermittent deviations of 5 to 30 PD, esotropia being compensated part of the time (n = 25). 4. Esotropia present only at a distance in patients over 50 years of age (n = 20). 5. Small-angle esotropia (< 15 PD) greater at a distance, associated with oculomotor cerebellar syndrome (n = 2). Complementary examinations (MRI or CT scan) were performed on 21 patients, leading to the discovery of a posterior fossa astrocytoma in a 4-year-old boy. CONCLUSIONS: The recognition of decompensated micro-esotropia and esophoria, as well as distance esotropia of the elderly, avoids unnecessary additional investigations, which are indicated in any type of acute comitant strabismus if associated with any neurological sign or symptom (e.g., headaches, nausea, vertigo, imbalance, poor coordination, nystagmus, or papilledema). In the absence of neurological findings, there is no consensus about the indication of neuroimaging in large-angle acquired concomitant esotropia, but long-term follow-up of patients that do not undergo neuroimaging is strongly recommended in order to identify later occurring intracranial diseases. This is of particular importance with children.

[Acute Acquired Comitant Esotropia Type 2 - A Retrospective Analysis]. / Akut erworbene konkomitante Esotropie Typ 2 ­ eine retrospektive Analyse.

BACKGROUND: Acute acquired comitant esotropia (AACE) is an uncommon form of strabismus. The main characteristics of AACE Type 2 in childhood are: acute onset of strabismus around 3 years of age, large and comitant angle of strabismus, absence of accommodative component, hyperopia of not more than 3 dpt, and measurable stereopsis. Strabismus surgery is urgently indicated in AACE Type 2 in order to avoid maldevelopment or loss of stereopsis. Therefore, in order to better describe the characteristics of AACE Type 2, we performed a retrospective analysis of patients previously seen in our clinic. PATIENTS AND METHODS: Retrospective analysis of data from patients who underwent strabismus surgery between October 2011 and September 2019 due to suspected AACE Type 2 could be confirmed during postoperative visits by evaluating ocular alignment and stereopsis at our hospital. Analysed data included: age and symptoms at first visit, visual acuity, refractive status, correction by glasses, age at surgery, period of time between first symptoms and surgery, surgical procedure, stereopsis and angle of strabismus (before surgery and 1 day, 3 months, and 12 months after surgery). Ethical approval was obtained from the Cantonal Ethics Committee of Zurich. RESULTS: 18 patients (12 male, 6 female) with mean hyperopia of 1.4 ± 0.6 dpt were identified as meeting the inclusion criteria during the defined time period. Amblyopia was present at first assessment in two patients. Strabismus surgery was performed at 2.0 to 11.1 years of age and between 0.4 and 24.6 months after onset of symptoms. Surgery was performed within 6 months after onset of symptoms in 12/18 children. Before surgery, angle of strabismus at near was measured as 38 ± 10 prism diopters (PD) and was reduced after surgery to 3 ± 3 PD at 3 months and 2 ± 2 PD at 12 months. Stereopsis was confirmed in 5/18 patients one day after surgery and in 18/18 patients 12 months after surgery. CONCLUSION: Our analysis showed that our patients with diagnosis of AACE Type 2 had mild hyperopia and large comitant non-accommodative esotropia; prognosis for recovery of stereopsis is excellent. Preoperative amblyopia does not exclude the diagnosis of AACE Type 2. Therapeutic intervention is advisable within a short period of time after first symptoms and the diagnosis of AACE Type 2.