A rare association of type 2 Duanes retraction syndrome with arthrogryposis multiplex congenita.

A 10-year-old boy presented with complaints of abnormal eye movements and face turn since early There was a limitation of adduction in LE with narrowing of palpebral fissure and downshoot characteristic of type 2 Duane Syndrome. He was a known case of Arthrogryposis multiplex congenita (AMC) and had undergone multiple orthopedic surgeries. The literature on Duane Retraction Syndrome (DRS) in AMC is limited. Here, we report in brief the association of DRS type 2 in AMC.

Interocular symmetry of retinal nerve fiber layer and optic nerve head parameters measured by Cirrus high-definition optical coherence tomography in a normal pediatric population.

PURPOSE: To determine interocular differences in the retinal nerve fiber layer (RNFL) and optic nerve head (ONH) parameters in a pediatric population using Cirrus high-definition optical coherence tomography (HD-OCT). METHODS: Seventy normal Indian children aged 5-17 years presenting to the Pediatric Clinic were included in this observational cross-sectional study. All subjects underwent a comprehensive ophthalmologic examination and an evaluation of the RNFL and ONH by Cirrus HD-OCT. Differences between the right and left eyes were calculated and values were compared by means of a paired t-test. Subjects were also divided into two groups based on age (under or over 10 years of age). Interocular differences in RNFL and ONH parameters together with sex and age variations for these differences were determined. RESULTS: The mean age of studied pediatric population was 11.83 ± 3.3 years (range 5-17). Average RNFL thickness was 94.46 ± 8.7 µm (± SD) (range 77-111). Differences in the average RNFL between right and left eyes were not statistically significant (P = 0.060). Superior quadrant RNFL was thicker in the left eye and temporal quadrant was thicker in the right eye. Among ONH parameters, there were no statistically significant differences in any parameters, except vertical cup-disc (CD) ratio which was significant (P = 0.007). The 2.5%-97.5% limits of asymmetry were 9 µm for average RNFL, 0.14 for average CD ratio, and 0.22 for vertical CD ratio. Mean interocular RNFL thickness differences in superior, superior nasal, and temporal superior quadrants were 10.61 (P < 0.001), 12.57 (P < 0.001), and 4.46 (P = 0.002) µm, respectively. Interocular nerve fiber layer thickness differences were not significantly correlated with sex, while only significant differences with age were observed in 12 clock hour sector analysis, mainly in nasal inferior and inferior quadrant. CONCLUSIONS: We report the degree of interocular symmetry of RNFL and ONH parameters measured by Cirrus HD-OCT in a healthy pediatric population. The normal interocular RNFL asymmetry should not exceed 9 µm and vertical CD ratio beyond 0.22 should be considered for further investigations. The physiologic asymmetry provided by this study may assist in identifying changes in RNFL thickness and ONH parameters in pediatric glaucoma and ONH disorders.

Unilateral acquired Brown's syndrome in systemic scleroderma: An unusual cause for diplopia.

Brown's syndrome can be congenital or acquired with multiple causes. It has been described as a ocular complication in various rheumatic and nonrheumatic diseases. We describe a case of 27-year-old female patient with 5 years old history of systemic scleroderma who developed vertical diplopia, a left head tilt, and restriction of left eye on elevation in adduction. The patient responded to systemic steroids with resolution of diplopia.

Retinal nerve fiber layer thickness in normal Indian pediatric population measured with optical coherence tomography.

PURPOSE: To measure the peripapillary retinal nerve fiber layer (RNFL) thickness in normal Indian pediatric population. SUBJECTS AND METHODS: 120 normal Indian children ages 5-17 years presenting to the Pediatric Clinic were included in this observational cross-sectional study. RNFL thickness was measured with stratus optical coherence tomography (OCT). Children with strabismus or amblyopia, with neurological, metabolic, vascular, or other disorders and those with abnormal optic discs were excluded. One eye of each subject was randomly selected for statistical analysis. The effect of age, refraction and gender on RNFL thickness was investigated statistically. RESULT: OCT measurements were obtained in 120 of 130 (92.3%) subjects. Mean age was 10.8 ± 3.24 years (range 5-17). Average RNFL thickness was (± SD) 106.11 ± 9.5 µm (range 82.26-146.25). The RNFL was thickest inferiorly (134.10 ± 16.16 µm) and superiorly (133.44 ± 15.50 µm), thinner nasally (84.26 ± 16.43 µm), and thinnest temporally (70.72 ± 14.80 µm). In univariate regression analysis, age had no statistical significant effect on RNFL thickness (P = 0.7249) and refraction had a significant effect on RNFL thickness (P = 0.0008). CONCLUSION: OCT can be used to measure RNFL thickness in children. Refraction had an effect on RNFL thickness. In normal children, variation in RNFL thickness is large. The normative data provided by this study may assist in identifying changes in RNFL thickness in Indian children.