BILATERAL MACULAR CHOROIDAL ABNORMALITIES WITH DRUSENOID DEPOSITS IN PATIENTS WITH UNILATERAL PERIPHERAL EXUDATIVE HEMORRHAGIC CHORIORETINOPATHY.

PURPOSE: To investigate bilateral macular features on optical coherence tomography in patients with unilateral peripheral exudative hemorrhagic chorioretinopathy (PEHCR). METHODS: In this cross-sectional study, optical coherence tomography features of affected eyes (PEHCR group, n = 30) and unaffected contralateral eyes (contralateral group, n = 30) were investigated. Age-matched and sex-matched patients with polypoidal choroidal vasculopathy (PCV group, n = 51) and healthy controls (normal group, n = 50) were included to compare choroidal thickness, measured at six points apart from the fovea, with the PEHCR group. RESULTS: Subretinal drusenoid deposits were the most common feature in the PEHCR (20%) and contralateral (23%) groups, followed by soft drusen. Although the macular choroid was comparably thin in both the PEHCR and contralateral groups, pachyvessels were also observed. The choroids of the PEHCR group were significantly thinner than those of the normal group at the subfovea and 1-mm temporal to the fovea and considerably thinner than those of the polypoidal choroidal vasculopathy group from 3-mm nasal to 3-mm temporal to the fovea. CONCLUSION: In patients with unilateral PEHCR, bilateral choroidal thinning and drusenoid deposit accumulation were noted in the macula. The pathophysiology of PEHCR may be a rare peripheral complication of age-related macular degeneration with pathologic choroid.

Short-Term Outcomes of the First in Vivo Gene Therapy for RPE65-Mediated Retinitis Pigmentosa.

Here, we report early treatment outcomes of gene therapy for early onset retinitis pigmentosa (RP) (Leber congenital amaurosis) associated with biallelic RPE65 mutation in a 30-year-old female patient. Initially, her visual acuity (VA) was 20/200, and her visual field (VF) was severely constricted to the center in the left eye. Her electroretinography showed nearly extinct signals. Full-field stimulus threshold test (FST) revealed diminished dark-adapted light sensitivity. Voretigene neparvovec-rzyl (VN) is the first in vivo viral gene therapy agent to be approved. At 3 months after subretinal injection of VN in the left eye, VA, VF, and FST showed sustained improvement. She did not exhibit any signs of adverse effects from the treatment. Gene therapy for RP proved to be an effective and safe treatment in an advanced case of RPE65-associatied early onset RP.

Comparison of contrast sensitivity based on the surgical results for intermittent exotropia.

AIM: To compare contrast sensitivity (CS) based on the surgical results for intermittent exotropia (IXT) and to examine the relationship between CS and photophobia. METHODS: Medical records of the patients who underwent bilateral lateral rectus muscle recession for IXT between 4 and 12 years old were reviewed retrospectively. They were categorized based on the surgical results; successful correction group (n=36) and overcorrection group (esotropia ≥10 PD at 3mo postoperatively, n=18). Using CGT-2000 test for CS was performed binocularly, and subjective reports of photophobia was investigated preoperatively and at 3mo postoperatively. Objective photophobia was defined as a significant decrease in CS in the presence of glare. RESULTS: Preoperatively, there was no difference in CS between the groups. Postoperatively, under mesopic conditions, significant improvement of CS was observed at 6.3°, 4°, and 2.5° in the successful correction group and at 6.3° and 4° in the overcorrection group, regardless of glare. Under photopic conditions, at all visual angles except 0.64°, improvement in CS was noted in both groups while CS worsened significantly at 0.64° in the overcorrection group postoperatively. At all visual angles under photopic conditions postoperatively, regardless of glare, CS in the overcorrected group was significantly worse than that in the successful correction group, and CS was significantly decreased by addition of glare in both groups. All patients except one (96.4%) in the successful correction group and 8 patients (61.5%) in overcorrection group showed improvement of photophobia postoperatively, which correlated with CS under photopic conditions (P=0.001, 0.03). CONCLUSION: After surgery for IXT, CS under photopic conditions improve at all visual angles except 0.64°, while CS is significantly worse in the overcorrection group postoperatively at 0.64°. Subjective photophobia have significant correlation with CS under photopic conditions, and may be used as an objective indicator of photophobia.

New Method of Quantitative Analysis of Hard Exudate Using Optical Coherence Tomography: Application in Diabetic Macular Edema.

PURPOSE: In the present study, the volume of hard exudates (HEs) was quantitatively measured using optical coherence tomography (OCT) and the agreement and correlation with area of HEs in fundus photography were analyzed. METHODS: The medical records of patients with diabetic macular edema who underwent focal laser treatment and were followed up more than 3 months were retrospectively evaluated. An automated customized program designed for measuring HE volume was used. The HEs in each OCT B-scan binary image were measured using 512 × 128 pixels, 6 mm × 6 mm OCT cube scans. The volume was measured by summing the segmented HEs in each 128 B-scan image. The area was measured in 6 mm x 6 mm fundus photography. The volume and area were measured before and 3 months after the treatment. The agreement of increase and decrease in HEs, and the correlation of volume and area of HEs were analyzed. RESULTS: A total of 35 patients (39 eyes) were included in the study. The volume was significantly reduced from 0.07978 to 0.02565 mm³ at 3 months (p < 0.001). The area was also significantly reduced from 15.35 to 8.60 mm² at 3 months (p < 0.001). The volume was decreased in 34 eyes and increased in 5 eyes. The area was decreased in 37 eyes and increased in 2 eyes. A significant correlation between volume and area was found (p < 0.001) as well as agreement between increase and decrease in volume and area. CONCLUSIONS: In the 3-dimensional quantitative volumetric analysis, the volume and area of HEs were correlated and the direction of increase and decrease was concordant. Considering the distribution of HEs in multiple layers of the retina, volumetric analysis could be considered a substitute for the analysis of HE area.

Characteristics of Patients Who Are Not Responsive to Alternate Patching for Overcorrected Intermittent Exotropia.

PURPOSE: To compare the characteristics of patients with surgically overcorrected intermittent exotropia treated with alternate patching. METHODS: The medical records of 51 patients who underwent bilateral lateral rectus muscle recession for intermittent exotropia and required alternate patching to correct postoperative overcorrection were retrospectively reviewed. Patients with postoperative esodeviation ≥18 prism diopters (PD) were started on alternate patching on postoperative day 1, whereas those with postoperative esodeviation of 10 to 17 PD were started after 2 weeks. Postoperative esodeviation <10 PD was considered as slight intentional overcorrection after exotropia surgery. Patients not responsive to alternate patching treatment were defined as those with postoperative esodeviation ≥10 PD after 3 months of treatment. Sex, family history, age, refractive error, amblyopia, stereopsis, suppression, type of exotropia, surgical method, preoperative and postoperative angle of deviation, and start time of alternate patching were compared. RESULTS: Among 51 patients, 29 patients responded to alternate patching and 22 patients did not respond. Female sex (p = 0.04), larger preoperative exodeviation at distance (p = 0.04), late onset of postoperative maximal esodeviation (p < 0.01), larger postoperative maximal esodeviation at near (p = 0.02), and late initiation of alternate patching (p = 0.01) were associated with patients in the non-responsive group. Although postoperative angle of deviation was similar for 2 weeks, the angle of postoperative esodeviation was significantly larger in the non-responsive group than in the responsive group, beginning at 1 month postoperatively. CONCLUSIONS: Female sex, large preoperative exodeviation, late initiation of alternate patching, and large esodeviation 1-month postoperative predisposed patients to be resistant to alternate patching for postoperative overcorrection.