A case of WAGR syndrome in association with developmental glaucoma requiring bilateral Baerveldt glaucoma implants and subsequent tube repositioning

Glaucoma drainage device implantation is efficacious for the treatment of pediatric glaucoma patients when multiple angle surgeries fail. However, tube touching of the corneal endothelium is one of the major postoperative complications to deal with. A 15-month-old male patient with Wilms’ tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome was diagnosed with bilateral developmental glaucoma. He underwent Baerveldt glaucoma implant (BGI) surgeries in both eyes after multiple failed trabeculotomies. The tube in his right eye was touching the cornea 15 months after BGI surgery. To avoid corneal endothelium damage, BGI tube repositioning with scleral fixation was performed without serious complications. The bilateral BGI surgeries achieved successful intraocular pressure reduction for over 2 years and tube repositioning with scleral fixation of BGI tube was successful for BGI tube malposition. Although careful attention to intraocular pressure and tube malposition is essential after glaucoma drainage device implantation, especially in pediatric cases, BGI surgery is effective in the management of developmental glaucoma following unsuccessful angle surgeries

Microperimetry and multimodal imaging in polypoidal choroidal vasculopathy

Polypoidal choroidal vasculopathy (PCV) is a degenerative macular disease. The study determined the topographical concordance in the areal extent of PCV, defined by indocyanine green angiography (ICGA), and the corresponding outcomes from spectral-domain optical coherence tomography (SD-OCT) and microperimetry, in 25 individuals (25 eyes) who had undergone 3 months of anti-vascular endothelial growth factor treatment. The differential light sensitivity within 10° eccentricity was evaluated by Pattern Deviation probability analysis. The concordances and proportional areal extents of the abnormality for ICGA, SD-OCT and microperimetry were compared. The concordance in the areal extent between all three modalities was 59%. The median concordance between ICGA and microperimetry was 60%; between ICGA and SD-OCT, 70%; and between SD-OCT and microperimetry, 72%. SD-OCT and microperimetry each identified a greater areal extent (>20%) compared to ICGA in 13 and 19 eyes, respectively. A greater areal extent (>20%) was present in 9 eyes for microperimetry compared to SD-OCT and in 5 eyes for SD-OCT compared to microperimetry. SD-OCT and microperimetry each identified a greater area of abnormality than ICGA which supports the clinical utility of SD-OCT. Strong concordance was present between SD-OCT and microperimetry; however, microperimetry identified additional areas of functional abnormality

Grid photocoagulation combined with intravitreal bevacizumab for recurrent macular edema associated with retinal vein occlusion

Purpose: To report the efficacy of grid photocoagulation combined with intravitreal bevacizumab (IVB) for macular edema recurring after previous IVBs associated with retinal vein occlusion (RVO).　　Methods: This retrospective study consisted of 19 eyes with branch retinal vein occlusion (BRVO) and nine eyes with central retinal vein occlusion (CRVO), which were treated with grid photocoagulation combined with IVB for recurrent macular edema after previous IVBs. The mean duration of total follow-up was 29.3 ± 5.8 months.　　Results: After this combination therapy, foveal thickness was reduced, significant with slight improvement in visual acuity (VA). At 1 month after treatment, although 25 of the 28 eyes showed complete resolution of the cystoid space, the macular edema recurred to some extent in 19 eyes. Compared with initial values, final foveal thickness was reduced significantly in both BRVO and CRVO groups (P < 0.001), but improvement in VA was significant only for eyes with BRVO (P = 0.012). The total number of IVB was 2.8 ± 0.7 for eyes with either BRVO or CRVO.　　Conclusion: Grid photocoagulation combined with IVB has a substantial effect on reducing recurrent macular edema associated with RVO, but the effect on visual acuity is limited

Post-Cataract Surgery Visual Disturbance in a Retinitis Pigmentosa Patient with Asteroid Hyalosis

A patient with retinitis pigmentosa showed visual disturbances following successful cataract surgery. He had a dense asteroid hyalosis in the eye before cataract surgery. After the surgery he noticed that his vision became worse. The visual disturbance was explained as being caused by the progression of retinal degeneration. Although the electroretinogram was non-recordable, the degeneration of macular area appeared relatively small. We considered that dense asteroid hyalosis was responsible for his visual disturbances, and pars plana vitrectomy (PPV) was performed to remove the asteroid hyalosis. After the PPV, rapid improvement of his visual acuity was observed. Cataract surgery may affect the status of asteroid hyalosis and cause rapid visual loss. PPV should be considered for retinitis pigmentosa patients with dense asteroid hyalosis, especially when a large decrease in visual acuity is noted shortly after cataract surgery

White dots as a novel marker of diabetic retinopathy severity in ultrawide field imaging

Purpose: To characterize white dots in diabetic retinopathy (DR) and their association with disease severity using ultra-wide-field scanning laser ophthalmoscopy. Methods: We randomly selected 125 eyes of 77 patients (25 eyes from individual categories of the international classification of DR severity) for which ultrawide field photographs were obtained. We characterized white dots, which were delineated by higher signal levels on green but not red laser images, and evaluated the relationship between the number of white dots and the international severity scale of DR. Results: Most white dots were located in nonperfused areas, and the number of total white dots was significantly correlated to that of dots in nonperfused areas. White dots corresponded to microaneurysms around the boundary between nonperfused areas and perfused areas or unknown lesions in nonperfused areas. Eyes with DR had significantly more white dots than those with no apparent retinopathy. The numbers of white dots in moderate nonproliferative diabetic retinopathy (NPDR) or more severe grades were significantly higher than in mild NPDR. The area under the receiver operating characteristics curve (AROC) analyses demonstrated that the number of white dots had the significance in the diagnosis of DR (0.908-0.986) and moderate NPDR or more severe grades (0.888-0.974). Conclusions: These data suggest the clinical relevance of white dots seen on ultrawide field images in the diagnosis of the severity of DR

Outer retinal circular structures in patients with Bietti crystalline retinopathy.

[Background] : Bietti crystalline retinopathy (BCR) is a distinct retinal degenerative disease characterised by retinal degeneration with many yellow–white crystals located mainly at the posterior pole area. Using spectral domain-optical coherence tomography (SD-OCT), the structural change in retina was investigated. [Methods] : Patients diagnosed with BCR (n=12), retinitis pigmentosa (RP, n=292) and cone dystrophy (n=16) were included in this study. The authors mainly examined fundus photographs and SD-OCT, infrared and fundus autofluorescence images of these patients. [Results]: Crystalline deposits were detected in portions of the retinal pigment epithelium that lacked patchy degenerated lesions. SD-OCT revealed that most of the observed crystalline deposits were located adjacent to the inner side of retinal pigment epithelium layer. The change most frequently observed was circular hyper-refractive structures in the outer nuclear layer. Although the structures were considered to be previously reported “tubular formation” or “tubular degeneration”, we determined that many of these circular structures were slices of spherical structures and were typically noted in areas suspected of ongoing active degeneration. [Conclusion] : BCR has characteristic structures in the outer nuclear layer. Although the incidence of the structure varies, it may be characteristic of retinal degeneration and can be found in many retinal degenerative diseases

Assessment of macular choroidal thickness by optical coherence tomography and angiographic changes in central serous chorioretinopathy.

[Objective]: To investigate the relationship between macular choroidal thickness measured by highpenetrating swept-source optical coherence tomography (SS-OCT) and angiographic findings in central serous chorioretinopathy (CSC). [Design]: Prospective cross-sectional case series. [Participants and Controls]: Thirty-four patients with CSC (44 eyes) and 17 volunteer subjects (17 normal eyes). [Methods]: All subjects underwent a comprehensive ophthalmologic and SS-OCT prototype examination. All patients with CSC also underwent simultaneous fluorescein angiography (FA) and indocyanine green angiography (IA). Mean regional choroidal thickness measurements on the Early Treatment Diabetic Retinopathy Study (ETDRS) layout and squared sector grids were obtained by 3-dimensional raster scanning using SS-OCT. [Main Outcome Measures]: Macular choroidal thickness and angiographic abnormalities. [Results]: Mean whole macular choroidal thickness in eyes with CSC (total, 329.3_83.0 _m; classic CSC, 326.9_83.1 _m; chronic CSC, 325.4_93.3 _m; and multifocal posterior pigment epitheliopathy, 359.0_15.5 _m) was greater than that in normal eyes (233.0_67.0_m) (P_ 0.001). In unilateral cases, mean whole macular choroidal thickness was greater in eyes with unilateral CSC than in unaffected fellow eyes (P_0.021). There was no significant difference in choroidal thickness between active eyes and resolved eyes in any of the ETDRS sectors. Mean choroidal thickness was greater in areas with leakage on FA than in areas without leakage (P_0.001). Mean choroidal thickness was greater in areas with choroidal vascular hyperpermeability and in areas with punctate hyperfluorescent spots on IA than in unaffected areas (P_0.001 for both). [Conclusions]: Increased choroidal thickness was observed in the whole macular area of eyes with any of the CSC subtypes. Choroidal thickness was related to leakage from the retinal pigment epithelium, choroidal vascular hyperpermeability, and punctate hyperfluorescent lesions. These findings provide evidence that CSC may be caused by focally increased hydrostatic pressure in the choroid. [Financial Disclosure(s)]: Proprietary or commercial disclosure may be found after the references

Effects of age and blood pressure on the retinal arterial wall, analyzed using adaptive optics scanning laser ophthalmoscopy

The wall-to-lumen ratio (WLR) of the vasculature is a promising early marker of retinal microvascular changes. Recently, adaptive optics scanning laser ophthalmoscopy (AOSLO) enabled direct and noninvasive visualization of the arterial wall. Using AOSLO, we analyzed the correlation between age and WLR in 51 normal subjects. In addition, correlations between blood pressure and WLR were analyzed in 73 subjects (51 normal subjects and 22 hypertensive patients). WLR showed a strong correlation with age (r=0.68, P<0.0001), while outer diameter and inner diameter did not show significant correlation with age in the normal group (r=0.13, P=0.36 and r=0.12, P=0.41, respectively). In the normal and hypertensive groups, WLR showed a strong correlation with systolic and diastolic blood pressure (r=0.60, P<0.0001 and r=0.65, P<0.0001, respectively). In conclusion, AOSLO provided noninvasive and reproducible arterial measurements. WLR is an early marker of morphological changes in the retinal arteries due to age and blood pressure