1,2 This small but important finding can be a marker for potentially life-threatening conditions, making it of great clinical utility. A CWS appears as a white and fluffy superficial lesion 0.1mm to 1.0mm in diameter that obscures the underlying retinal detail. One of these potential retinal findings is the cotton wool spot (CWS). We believe that this case could have presented better outcomes with strict adherence to therapy.A careful retinal examination is critical in order to evaluate each patient for various pathologies, some of which yield an underlying systemic cause. Amblyopia treatment is long and requires full cooperation from the patient and the family members. Despite the initial presentation, the patient showed a refractory response to the treatment of amblyopia, which could be explained by the lack of complete adherence. In our case, there was no macular involvement and no development of strabismus, which are recognized as good prognostic factors. Our case represents an early diagnosis of Straatsma syndrome identified through a visual screening. The amount of anisometropia and the total area of myelination are important prognostic factors for visual improvement. Despite the presentation and prognosis, there are some reports with good visual results therefore, intensive visual rehabilitation should always be attempted. The presence of strabismus is frequent in Straatsma syndrome and is also correlated with worse visual outcomes, with younger patients without strabismus and with parafoveal fixation reported to respond best to amblyopia treatment .įull optical correction of myopia and treatment of amblyopia should be initiated as soon as possible. Amblyopia associated with myelinated retinal fibers portends a poor prognosis and a worse response to treatment than anisometropic amblyopia without myelinated retinal fibers. Visual deprivation in early life can induce axial elongation with resultant myopia, and dense myelinated retinal fibers may cause visual deprivation by obscuring vision and producing optical defocus. Patients with Straatsma syndrome present axial myopia instead of refractive myopia. Visual evoked potentials were normal in the right eye and showed moderate dysfunction of the left optical path, compatible with the amblyopia presented. Regardless of the prescribed occlusion, the parents still reported very irregular compliance. In the moment of the last observation, two and a half years later from diagnosis, the patient maintained a BCVA of 2/10 in the left eye (and 10/10 in the right eye), with no development of strabismus. Daily occlusion of the right eye (six hours) was advised during the entire period of follow-up. Given the lack of compliance to therapy, total right eye occlusion was prescribed for one month, but no improvement was noted after that time therefore, the total time of occlusion was reduced to six hours daily. The mother reported incomplete treatment, accomplishing only two hours of daily occlusion. Three months after starting the occlusion therapy, a new cycloplegic retinoscopy revealed an increase in myopia, with a new prescription of -4.00 and -3.00 × 70° to the left eye and a BCVA of 2/10. At this point, amblyopia treatment was started with six hours daily of right eye occlusion. Glasses with full optical correction were prescribed, and the child was evaluated three months later, presenting a best-corrected visual acuity (BCVA) of 8/10 in the right eye and 1/10 in the left eye.
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