Amaç: Albinizmli hastalarda klinik bulguları değerlendirmek ve görme keskinliği ile iris translüminasyonu, makula saydamlığı ve foveal hipoplazi arasındaki ilişkiyi saptamak. Gereç ve Yöntemler: Albinizm tanılı 23 hastanın kayıtları retrospektif olarak değerlendirildi. Bulgular: Hastaların ortalama yaşı 23,5 (20; 5-45) yıl olup, 6 (%26,08)'sı kadın, 17 (%73,91)'si erkek idi. Her bir gözün ayrı ayrı görme düzeyleri 0,7 ile 3 m'den parmak sayma arasında değişmekteydi. Otuz altı gözde miyopik, 4 gözde hipermetropik olmak üzere 40 gözde 0,75-9 D arasında astigmatizma saptandı. İki gözde grade I, 4 gözde grade II, 36 gözde grade III, 4 gözde grade IV düzeyinde iris translüminasyon defekti mevcuttu. Sekiz gözde grade I, 38 gözde grade II düzeyinde makula saydamlığı izlendi. Hastaların sağ gözlerinin (n=23 göz) ortalama foveal kalınlığı 261 μ (264, 193-325 μ), sol gözlerinin (n=23 göz) ortalama foveal kalınlığı 262 μ (265, 195-330 μ) olarak bulundu. Görme keskinliği ile iris translüminasyonu, makula saydamlığı ve foveal hipoplazi dereceleri arasında istatistiksel olarak anlamlı korelasyon saptandı. Sonuç: Albinizmli hastalarda görme kaybının mekanizmaları multifaktöriyeldir. Bu mekanizmalardan sadece biri olan refraksiyon kusurları düzeltilmelidir. İris translüminasyonu, makula saydamlığı ve fovea hipoplazisi gibi yapısal değişiklikler görme prognozunun tahmini açısından yardımcı bulgulardır.
Anahtar Kelimeler: Albinizm; foveal hipoplazi; az görme; refraksiyon kusuru; strabismus; ambliyopi
Objective: To evaluate clinical findings in patients with albinism and to determinate the relationship between visual acuity and iris transillumination, macular transparency, foveal hypoplasia. Material and Methods: The records of 23 patients with albinism were evaluated retrospectively. Results: The mean age of the patients was 23.5 (20; 5-45) years. Six (26.08%) of the patients were female and 17 (73.91%) were male. Visual acuity of each eye ranged from 0.7 to counting finger from 3 meter. Astigmatism (min: 0.75 D max: 9 D) was detected in 40 of the eye including myopic in 36 eyes and hypermetropic in 4 eyes. There were grade I iris translumination defect in 2 eyes, grade II iris translumination defect in 4 eyes, grade III iris translumination defect in 36 eyes, and grade IV iris translumination defect in 4 eyes. Eight eyes had grade I and 38 eyes had grade II macular transparency. The average foveal thickness of right eyes (n=23 eyes) was 261 μ (264, 193-325 μ), average foveal thickness of left eyes (n=23 eyes) was 262 μ (265, 195-330 μ). There was a statistically significant correlation between visual acuity and iris translumination, macular transparency foveal hypoplasia. Conclusion: The mechanism of loss of vision in albinism is multifactorial. One of these mechanisms, refraction defects should be corrected. Structural changes such as iris translumination, macular transparency and foveal hypoplasia are helpful findings for predicting visual prognosis of patients.
Keywords: Albinism; foveal hypoplasia; low vision; refractive error; strabismus; amblyopia
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