Purpose: To determine the visual field patterns obtained by the preferential hyperacuity perimetry (PHP) in patients with Best vitelliform macular dystrophy with mutations in the BEST1 gene. Methods: Consecutive patients with Best vitelliform macular dystrophy underwent a complete ophthalmologic examination, including functional assessment by best-corrected visual acuity and Foresee PHP and morphologic assessment by fundus biomicroscopy, fundus autofluorescence, and spectral-domain optical coherence tomography. The functional "PHP visual field defect index" (which is the max peak value of the metamorphopsia [maximal distortion value at the visual field] + the max peak value of the scotoma [maximal scotoma value at the visual field]) and best-corrected visual acuity were analyzed about the disease stage. Results: Thirty eyes of 15 consecutive patients (8 men and 7 women; mean age 39 6 24 years) were included for analysis. Based on fundus biomicroscopy, fundus autofluorescence, and spectral-domain optical coherence tomography, the macular lesions could be counted as follows: previtelliform lesions in 5 eyes of 3 patients (Stage 1), vitelliform lesions in 2 eyes of 2 patients (Stage 2), pseudohypopyon lesions in 6 eyes of 5 patients (Stage 3), vitelliruptive lesions in 4 eyes of 3 patients (Stage 4), atrophic lesions in 7 eyes of 5 patients (Stage 5), and fibrotic lesions in 6 eyes of 4 patients (Stage 6). Best-corrected visual acuity and PHP visual field defect index were averaged for each stage. Best-corrected visual acuity showed a good correlation (P = 0.01) with the morphologic severity (stage) of the disease (Pearson correlation = -0.88). Similarly, the PHP visual field defect index showed a good correlation (P = 0.03) with the morphologic severity (stage) of the disease (Pearson correlation = 0.78). Finally, best-corrected visual acuity showed a good correlation (P = 0.02) with the functional PHP visual field defect index (Pearson correlation = -0.83) about the morphologic stage of the disease. Conclusion: Preferential hyperacuity perimetry could be considered an adjunctive useful tool in the evaluation of functional impairment and disease progression in patients with Best vitelliform macular dystrophy. RETINA 31:959-966, 2011
PREFERENTIAL HYPERACUITY PERIMETER IN BEST VITELLIFORM MACULAR DYSTROPHY / Querques, G., Atmani, K., Bouzitou Mfoumou, R., Leveziel, N., Massamba, N., Souied, E.h.. - In: RETINA. - ISSN 0275-004X. - 31:5(2011), pp. 959-966. [10.1097/IAE.0b013e3181f441c1]
PREFERENTIAL HYPERACUITY PERIMETER IN BEST VITELLIFORM MACULAR DYSTROPHY
QUERQUES , GIUSEPPE;
2011
Abstract
Purpose: To determine the visual field patterns obtained by the preferential hyperacuity perimetry (PHP) in patients with Best vitelliform macular dystrophy with mutations in the BEST1 gene. Methods: Consecutive patients with Best vitelliform macular dystrophy underwent a complete ophthalmologic examination, including functional assessment by best-corrected visual acuity and Foresee PHP and morphologic assessment by fundus biomicroscopy, fundus autofluorescence, and spectral-domain optical coherence tomography. The functional "PHP visual field defect index" (which is the max peak value of the metamorphopsia [maximal distortion value at the visual field] + the max peak value of the scotoma [maximal scotoma value at the visual field]) and best-corrected visual acuity were analyzed about the disease stage. Results: Thirty eyes of 15 consecutive patients (8 men and 7 women; mean age 39 6 24 years) were included for analysis. Based on fundus biomicroscopy, fundus autofluorescence, and spectral-domain optical coherence tomography, the macular lesions could be counted as follows: previtelliform lesions in 5 eyes of 3 patients (Stage 1), vitelliform lesions in 2 eyes of 2 patients (Stage 2), pseudohypopyon lesions in 6 eyes of 5 patients (Stage 3), vitelliruptive lesions in 4 eyes of 3 patients (Stage 4), atrophic lesions in 7 eyes of 5 patients (Stage 5), and fibrotic lesions in 6 eyes of 4 patients (Stage 6). Best-corrected visual acuity and PHP visual field defect index were averaged for each stage. Best-corrected visual acuity showed a good correlation (P = 0.01) with the morphologic severity (stage) of the disease (Pearson correlation = -0.88). Similarly, the PHP visual field defect index showed a good correlation (P = 0.03) with the morphologic severity (stage) of the disease (Pearson correlation = 0.78). Finally, best-corrected visual acuity showed a good correlation (P = 0.02) with the functional PHP visual field defect index (Pearson correlation = -0.83) about the morphologic stage of the disease. Conclusion: Preferential hyperacuity perimetry could be considered an adjunctive useful tool in the evaluation of functional impairment and disease progression in patients with Best vitelliform macular dystrophy. RETINA 31:959-966, 2011
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