Relationship between strabismus associated with Angelman syndrome and orbital anomaly.

PURPOSE: To investigate the relationship between the details of strabismus and orbital abnormalities determined by ocular motility tests and orbital imaging examinations in 9 cases with Angelman syndrome (AS). STUDY DESIGN: A retrospective, clinical report. METHODS: The 9 AS cases (mean age at initial visit: 4.6 ± 8.0 years) were confirmed by genetic diagnosis of the chromosome 15q11-13 region. In all cases, axial imaging of the orbit in the transverse plane of the horizontal extraocular muscles was obtained. The opening angle between both lateral walls of the orbit (greater wing of sphenoid) was measured as the biorbital angle, and compared with the 95% confidence interval of the orbital angle in normal children. RESULTS: All cases had exotropia with means of the distance and near of angle 32.2 prism diopters (Δ) ± 9.7Δ and 32.8Δ ± 8.3Δ. The mean of the biorbital angle was 107.7° ± 7.6°, greater than the biorbital angle of 94.3° ± 5.1° previously reported in 129 normal children (P < 0.0001, t-test). Except for one biorbital angle of 93° in the 25-year-old patient, all the biorbital angles in the 8 children were larger than the upper 95% confidence interval in normal children. Astigmatic and hyperopic ametropic amblyopia were detected in 3 cases and 1 case, respectively. CONCLUSIONS: The frequency of exotropia in AS is higher than previously reported, with our results strongly suggesting that the enlarged biorbital angle is related to the pathogenesis of exotropia in AS.

Two Pediatric Patients with Acute Acquired Comitant Esotropia as the First Symptom of Brainstem Tumor: A Case Report.

Introduction: Acute acquired comitant esotropia (AACE) is an acquired strabismus with uncrossed sudden-onset diplopia due to esodeviation, comitant esotropia without accommodation factor, or paretic eye movement. The diagnosis of AACE entails differentiation from incomitant esotropia caused by abnormalities in the central nervous system. We present 2 pediatric patients with AACE as the first symptom of brainstem tumor. Case Presentation: The 2 patients were aware of their diplopia and had no other neurological abnormalities. There were no special findings in the anterior segment, ocular media, or fundus. Esotropia with a difference of no more than 10Δ between distant and near fixations was observed. Eye movements were normal, and Hess red-green test under prism neutralization did not reveal abduction restriction. The presumed cause of AACE in both patients was excessive use of digital displays, and brain magnetic resonance imaging (MRI) was performed to confirm the absence of neurological abnormality. Using MRI, a definitive diagnosis of AACE was made based on comitant esotropia associated with diffuse median glioma and medullary pilocytic astrocytoma without abducens nerve palsy. Conclusion: Although the incidence of AACE caused by brainstem tumors may be low, it is necessary to perform head imaging to confirm etiology. Furthermore, Hess red-green test under prism neutralization is considered important for the differentiation of abducens nerve palsy.

Relationship Between Suppression Scotomas and Stereoacuity in Anisometropic Amblyopia With Successfully Treated Visual Acuity.

Purpose: The purpose of this study was to investigate the relationship among suppression scotoma size, stereoacuity, and four-prism base-out test (4ΔBOT) results in anisometropic amblyopia with successfully treated visual acuity. Methods: We included 103 cases of anisometropic amblyopia successfully treated for visual acuity without strabismus. Stereoacuity was measured using a Randot Stereotest. The size of the suppression scotomas was measured using a new device, the polarized four dot (P4D) test. This is a modification of the Worth 4 dot test (W4D) device. The patients were divided into three groups based on the 4ΔBOT results: normal (group A = 29 cases), subnormal (group B = 48 cases), and abnormal (group C = 26 cases) response groups. The horizontal diameter of the suppression scotomas and stereoacuity in logarithmic values with a base of 20 seconds of arc (″) were compared among the 3 groups. Results: The mean age at P4D testing was 8.4 ± 2.1 years. The average horizontal diameters of the suppression scotomas were 0.35 ± 0.79Δ, 2.01 ± 0.82Δ, and 5.50 ± 2.72Δ in groups A, B, and C, respectively, showing significant differences (A versus B: P < 0.0001, A versus C: P < 0.0001, and B versus C: P < 0.0001; 1-way ANOVA). The average logarithmic stereoacuity were 1.07 (24.95″), 1.22 (38.84″), and 1.47 (82.79″) in groups A, B, and C, respectively, thereby showing significant differences between the groups (A versus B: P < 0.0001, A versus C: P < 0.0001, and B versus C: P < 0.0001; 1-way ANOVA). Stereoacuity and horizontal diameter of the suppression scotoma were strongly correlated (r = 0.732, P < 0.0001). Conclusions: The suppression scotoma size measured using P4D correlated significantly with stereoacuity and the 4ΔBOT results.

Surgical Outcomes with and without Prism Adaptation of Cases with Acute Acquired Comitant Esotropia Related to Prolonged Digital Device Use.

Purpose: To analyze surgical results of 32 cases with acute acquired comitant esotropia (AACE) related to prolonged use of digital devices (DDs). Patients and Methods: Medical records of cases with AACE related to prolonged use of DDs that had undergone surgery were retrospectively revised. Complete medical history and full ophthalmological examinations before and surgery were obtained for all cases. All cases also underwent neurological examinations using brain and orbital imaging. Motor success was considered as alignment within 10 prism diopters (Δ) at both near and distance. Sensory success was defined as stereopsis ≤60 arcsec. Nineteen cases (Group S) underwent surgery alone and 13 cases were treated with surgery and prism adaptation (Group S+P). Motor and sensory outcomes were compared between groups. Results: Mean age at first visit was 22.0 ± 9.0 years and mean daily use of DDs use was 6.3 ± 3.4 hours. Mean angle of preoperative alignment for distance and near were 26.5 ± 13.0Δ and 24.6 ± 16.4Δ, respectively. Surgery was performed at a mean of 23.3 ±3.5 years old and mean angle of alignment at final examination for distance and near were 5.8 ± 7.7Δ and 3.5 ± 5.7Δ, respectively. Final motor and sensory success rate were 84% and 53%, respectively. No significant differences in motor or sensory outcomes were evident between groups at initial or last visit. Conclusion: Favorable motor and sensory outcomes were achieved with surgical intervention with and without prism adaptation for AACT related to prolonged use of DDs.

Effects of Bilateral Medial Rectus Resection on Motor Outcomes in Infantile Exotropia.

Purpose: To evaluate the effects of bilateral medial rectus resection (BMRres) on motor outcomes in infantile exotropia. Methods: We evaluated 19 cases of infantile exotropia surgery. The mean age at surgical alignment was 4.8±3.4 years (range, 1.5-11.8 years). The surgical procedures included BMRres (5 cases), BMRres with unilateral lateral rectus recession (ULRR) (3 cases), bilateral lateral rectus recession (BLRR) (8 cases), unilateral lateral rectus recession and medial rectus resection (uniRandR) with contralateral lateral rectus recession (2 cases), and uniRandR (1 case). After dividing the cases into two groups (BMRres group, n=8; other group, n=11), the outcomes at 1 day and at 1, 3, and 6 months after surgery were compared. Surgical outcomes were defined as (1) success: distant esotropia ≤5 prism diopters (Δ) or exotropia ≤10Δ, (2) recurrence: exotropia >10Δ, or (3) overcorrection: esotropia >5Δ. Results: Although postoperative distant deviations at 1 day were not different between the two groups, the BMRres group showed smaller distant deviations at 1, 3, and 6 months than the other group (p=0.015, 0.019, and 0.006, respectively). Success rates of the BMRres and other groups were 88% and 73% at 1 day, 100% and 36% at 1 month, 88% and 27% at 3 months, 88% and 18% at 6 months, respectively. Although there were no significant differences between the two groups within 3 months after surgery, surgical outcomes in the BMRres group 6 months after surgery were significantly better than those in the other group (p=0.003). Conclusion: BMRres is a better procedure than others for infantile exotropia to achieve desirable motor outcomes after surgery.

Intraocular penetration of liposomal amphotericin B after intravenous injection in inflamed human eyes.

PURPOSE: To determine the intraocular penetration of amphotericin B (AMPH-B) after an intravenously injection of liposomal amphotericin B (L-AMB) in inflamed human eyes. METHODS: Seven eyes of 5 patients with fungal eye diseases (endophthalmitis in 6 eyes and keratitis in 1 eye) were treated with intravenous injections of 100-250 mg/day of L-AMB. Samples of blood, corneal button, aqueous humor, and vitreous humor were collected and assessed for AMPH-B. RESULTS: The AMPH-B level in the cornea (604.0 µg/g) of the case with fungal keratitis exceeded the minimum inhibitory concentration. However, the levels in the aqueous and vitreous humors of the cases with fungal endophthalmitis were lower, e.g., 0.02 ± 0.01 µg/ml (0.09% of serum level) in the aqueous humor and 0.05 ± 0.08 µg/ml (0.17% of serum level) in the vitreous humor. CONCLUSIONS: The AMPH-B levels administered intravenously were very low in the aqueous and vitreous humors. Our findings indicate that intravenous L-AMB can be considered only for patients with mild endogenous fungal endophthalmitis, e.g., isolated chorioretinitis without vitreous extensions.

Clinical features of superior segmental optic hypoplasia: hospital-based study.

PURPOSE: To determine the clinical features of patients diagnosed with superior segmental optic hypoplasia (SSOH) and to quantitatively compare retinal nerve fiber layer (RNFL) thickness in SSOH eyes, to that in normal subjects. STUDY DESIGN: Retrospective comparative case series. METHODS: We examined the medical charts of 106 eyes of 59 patients with SSOH and 35 eyes of 35 normal subjects as controls. Forty-four of 59 patients had been examined by spectral-domain optical coherence tomography (SD-OCT). Eyes with SSOH were classified into a definite and a suspect type determined by standard automated perimetry. The definite type had inferior visual field (VF) defects, while the suspect type did not have inferior VF defects. The findings of the SD-OCT images of 35 eyes with SSOH were compared to those of the 35 normal eyes. RESULTS: Of the 106 eyes with SSOH, 56 (52.8%) were classified as the definite type and 50 (47.2%) as the suspect type. OCT showed that the average of the total RNFL thickness was significantly thinner in the SSOH group than in the normal group (P < 0.001; Mann-Whitney U test). Sectorial analysis demonstrated that the RNFL was thinner than controls in all quadrants (all P < 0.001; Mann-Whitney U test). The comparison of the hourly sectors showed that the RNFL was thinner at 10, 11, 12, 1, 2, 3, 5, and 6 o'clock sectors in the SSOH group than controls. CONCLUSIONS: Approximately one-half of eyes with SSOH had a detectable VF defect. OCT showed that eyes with SSOH have a thinner RNFL than controls except in 4 o`clock and from 7 o'clock to 9 o'clock.