Prevalence of Color Vision Deficiency and its Correlation with Amblyopia and Refractive Errors among Primary School Children.

PURPOSE: To determine the prevalence of color vision deficiency (CVD) and its correlation with amblyopia and refractive errors among primary school children. METHODS: In this population-based cross-sectional study, 2160 children were selected from 36 primary schools; 60 students were from each school (10 students in each grade), with equal sex distribution. A complete eye examination including refraction using a photorefractometer, determination of visual acuity (VA) and color vision using a Yang vision tester, and evaluation of ocular media opacity using a direct ophthalmoscope was performed. Children who could not answer at least 4 plates of the Ishihara color test were considered as color vision deficient subjects. Amblyopia was determined if pinhole VA was worse than 0.3 LogMAR (equal to 20/40). RESULTS: The prevalence of CVD was 2.2% (95% CI: 1.5% to 3%) which was higher in male subjects (37 [3.5%] boys vs. 11 [1.0%] girls, P < 0.001). Mean VA was lower among students with CVD as compared to normal color vision children (P = 0.035) and amblyopia was observed in 8.3% (95% CI: 0.2% to 16.4%) of patients with CVD versus 2.1% (95% CI: 1.5% to 2.08%) of children with normal color vision perception (P = 0.005). A statistically significant correlation between lower VA and CVD was observed (P = 0.023). CONCLUSION: Although CVD was correlated with lower VA and amblyopia, there was no relationship between CVD and the type of amblyopia, refractive error, anisometropia or strabismus.

Accuracy and Repeatability of Refractive Error Measurements by Photorefractometry.

PURPOSE: To determine the accuracy of photorefraction and autorefraction as compared to cycloautorefraction and to detect the repeatability of photorefraction. METHODS: This diagnostic study included the right eyes of 86 children aged 7-12 years. Refractive status was measured using photorefraction (PlusoptiX SO4, GmbH, Nürnberg, Germany) and autorefraction (Topcon RM800, USA) with and without cycloplegia. Photorefraction for each eye was performed three times to assess repeatability. RESULTS: The overall agreement between photorefraction and cycloautorefraction was over 81% for all refractive errors. Photorefractometry had acceptable sensitivity and specificity for myopia and astigmatism. There was no statistically significant difference considering myopia and astigmatism in all comparisons, while the difference was significant for hyperopia using both amblyogenic (P = 0.006) and nonamblyogenic criteria (P = 0.001). A myopic shift of 1.21 diopter (D) and 1.58 D occurred with photorefraction in nonamblyogenic and amblyogenic hyperopia, respectively. Using revised cut-off points of + 1.12 D and + 2.6 D instead of + 2.00 D and + 3.50 D improved the sensitivity of photorefractometry to 84.62% and 69.23%, respectively. The repeatability of photorefraction for measurement of myopia, astigmatism and hyperopia was acceptable (intra-cluster correlation [ICC]: 0.98, 0.94 and 0.77, respectively). Autorefraction results were significantly different from cycloautorefraction in hyperopia (P < 0.0001), but comparable in myopia and astigmatism. Also, noncycloglegic autorefraction results were similar to photorefraction in this study. CONCLUSION: Although photorefraction was accurate for measurement of myopia and astigmatism, its sensitivity for hyperopia was low which could be improved by considering revised cut-off points. Considering cut-off points, photorefraction can be used as a screening method.

A case of concurrent proteus syndrome and hemophilia a.

BACKGROUND: Proteus syndrome is a very rare condition with less than 100 confirmed cases reported worldwide. We report a case of Proteus syndrome in a two-year-old male who has hemophilia A comorbidity. CASE PRESENTATION: A two-year-old male patient was admitted with the chief complaint of severe bleeding in mouth cavity after trauma for two weeks. At admission he was found to have petechiae on buccal mucosa and fecal discoloration due to GI bleeding. We noted multiple abnormalities in his musculoskeletal system and skin. He had lymph edema in left leg, hemihypertrophy, macrodactyly in both foots and macrocephaly. With the history of severe bleeding and recurrent blood product transfusion, we suspected a hemorrhagic disorder. The reduced level of Factor VIII activity confirmed the diagnosis of hemophilia A. Considering patient's various musculoskeletal abnormalities according to the diagnostic criteria and after ruling out similar disorders the diagnosis of Proteus syndrome was established. CONCLUSION: Because of the variability of clinical features, Proteus syndrome can be confused with other disorders of multiple tissue overgrowth. Our case of Proteus syndrome, who had hemophilia A comorbidity outlines the challenges in diagnosis of such rare combination of diseases.