Abnormal Cystatin C Levels in Two Patients with Bardet-Biedl Syndrome

Bardet-Biedl syndrome (BBS) is an autosomal recessive disorder characterized by central obesity, mental impairment, rod-cone dystrophy, polydactyly, hypogonadism in males, and renal abnormalities. The causative genes have been identified as BBS1-14. In the Western countries, the prevalence of this disease ranges from 1/13,500 to 1/160,000, while only a few Japanese patients have been reported in the English-language literature. The incidence of renal dysfunction or anomalies in previous reports varies considerably ranging from ∼20% to universal occurrence. We here report that two Japanese patients who had BBS with normal BUN and creatinine levels had elevated levels of cystatin C, a sensitive marker of glomerular filtration rate. A urine albumin level increased only in the elder patient. Thus, cystatin C may be useful for detecting renal abnormalities in patients with an apparent normal renal function. Because this disease is diagnosed by accumulation of symptoms, such a sensitive marker might help early diagnosis of BBS

CSC with and without steroids

We investigated the rates of the use of steroids in Japanese central serous chorioretinopathy (CSC) cases and differences in the characteristics of CSC with and without steroids. A total of 538 eyes of 477 patients diagnosed with CSC, with 3 months or more of follow-up between April 2013 and June 2017 at 8 institutions. Patients with CSC with more than 3 months of follow-up were identified by OCT and fluorescein angiography at 8 institutions. Data collected included patient demographics, history of corticosteroid medication and smoking, spherical errors, findings of angiography, subfoveal choroidal thickness, and changes through the follow-up period. Differences in these findings were analyzed in cases with and without corticosteroid treatment. Among the 477 patients (344 men,133 women), 74 (15.5%) (39 men, 35 women) underwent current or prior steroid treatment. Cases with steroids were higher age (p = 0.0403) and showed no male prevalence, more bilateral involvement (p < 0.0001), and the affected eyes had multiple pigment epithelial detachment (p <0.0001), more fluorescein leakage sites (p < 0.0001), greater choroidal thickness (p = 0.0287) and a higher recurrence rate (p = 0.0412). Steroids can cause severer CSC through an effect on choroidal vessels and an impairment of retinal pigment epithelium

Long-Term Visual Prognosis of Patients Following Lens-Sparing Vitrectomy for Stage 4A Retinopathy of Prematurity

This study evaluated the long-term visual outcomes of patients in whom at least one eye underwent successful lens-sparing vitrectomy (LSV) for stage 4A retinopathy of prematurity (ROP). A retrospective chart review was conducted using the data of 61 eyes of 42 patients with a minimum 4-year follow-up after successful LSV, with or without anti-vascular endothelial growth factor (VEGF) therapy, and whose best-corrected visual acuity (BCVA) was measurable using Landolt rings at the final visit. The mean age at the final follow-up was 10.1 &plusmn; 3.3 years. Before LSV, all eyes underwent laser ablation therapy. Twenty eyes (32.8%) with high vascular activity received anti-VEGF therapy before LSV. The mean decimal BCVA at the final follow-up was 0.23 &plusmn; 0.26 (range: hand motion to 1.2). Twenty-three eyes (54.1%) had a decimal BCVA of &ge;0.4. Among 49 phakic eyes at the final examination, the mean refractive error was &minus;10.1 &plusmn; 5.0 D, with 37 eyes (75.5%) having high myopia (&gt;&minus;6.0 D). No significant differences were observed in terms of decimal BCVA and refractive errors between eyes with and without anti-VEGF therapy. Approximately half of the patients had a decimal BCVA of &ge;0.4, despite myopic refraction after successful LSV for stage 4A ROP. LSV for stage 4A ROP seemed to be associated with good visual function, despite myopic refraction

Long-Term Visual Prognosis of Patients Following Lens-Sparing Vitrectomy for Stage 4A Retinopathy of Prematurity

This study evaluated the long-term visual outcomes of patients in whom at least one eye underwent successful lens-sparing vitrectomy (LSV) for stage 4A retinopathy of prematurity (ROP). A retrospective chart review was conducted using the data of 61 eyes of 42 patients with a minimum 4-year follow-up after successful LSV, with or without anti-vascular endothelial growth factor (VEGF) therapy, and whose best-corrected visual acuity (BCVA) was measurable using Landolt rings at the final visit. The mean age at the final follow-up was 10.1 ± 3.3 years. Before LSV, all eyes underwent laser ablation therapy. Twenty eyes (32.8%) with high vascular activity received anti-VEGF therapy before LSV. The mean decimal BCVA at the final follow-up was 0.23 ± 0.26 (range: hand motion to 1.2). Twenty-three eyes (54.1%) had a decimal BCVA of ≥0.4. Among 49 phakic eyes at the final examination, the mean refractive error was −10.1 ± 5.0 D, with 37 eyes (75.5%) having high myopia (>−6.0 D). No significant differences were observed in terms of decimal BCVA and refractive errors between eyes with and without anti-VEGF therapy. Approximately half of the patients had a decimal BCVA of ≥0.4, despite myopic refraction after successful LSV for stage 4A ROP. LSV for stage 4A ROP seemed to be associated with good visual function, despite myopic refraction

A Case of Eosinophilic Chronic Rhinosinusitis Associated with Choroidal Folds and Ocular Motility Disorder

We report a case of eosinophilic chronic rhinosinusitis (ECRS) associated with choroidal folds and ocular motility disorder. A 50-year-old male with rhinosinusitis and bronchial asthma presented with anorthopia of the lower visual field and ocular motility disorder of the left eye. Dilated fundus examination and optical coherence tomography (OCT) revealed wavy choroidal folds in the upper retina. An emergent computed tomography (CT) showed sinusitis, a partial defect of the superior wall of the orbit on the left side, and deformation of the left eye. Based on the clinical findings, the patient was diagnosed with sinusitis complicated by ocular motility disorder. Endoscopic sinus surgery (ESS) was performed. A histopathological examination of the excised polyps showed extensive eosinophil invasion. According to the clinical findings of the nasal polyps, CT images, and peripheral blood tests, he was diagnosed as ECRS. One month after ESS, both ocular movement and anorthopia were improved. The choroidal folds observed using OCT disappeared 2 months after ESS. Although ECRS is rarely associated with ocular complications, bone involvement in sinusitis may result in deformation of the eyeball leading to choroidal folds and ocular motility disorder

En Face Optical Coherence Tomography Imaging of the Choroid in a Case with Central Serous Chorioretinopathy during the Course of Vogt-Koyanagi-Harada Disease: A Case Report

Vogt-Koyanagi-Harada (VKH) disease and central serous chorioretinopathy (CSC) develop serous retinal detachment; however, the treatment of each disease is totally different. Steroids treat VKH but worsen CSC; therefore, it is important to distinguish these diseases. Here, we report a case with CSC which was diagnosed by en face optical coherence tomography (OCT) imaging during the course of VKH disease. A 50-year-old man was referred with blurring of vision in his right eye. Fundus examination showed bilateral optic disc swelling and macular fluid in the right eye. OCT showed thick choroid, and en face OCT images depicted blurry choroid without clear delineation of choroidal vessels. Combined with angiography findings, this patient was diagnosed with VKH disease and treated with steroids. Promptly, fundus abnormalities resolved with the reduction of the choroidal thickness and the choroidal vessels became visible on the en face images. During the tapering of the steroid, serous macular detachment in the right eye recurred several times. Steroid treatment was effective at first; however, at the fourth appearance of submacular fluid, the patient did not respond. At that time, the choroidal vessels on the en face OCT images were clear, which significantly differed from the images at the time of recurrence of VKH. Angiography also suggested CSC-like leakage. The tapering of the steroids was effective in resolving the fluid. Secondary CSC may develop in the eye with VKH after steroid treatment. En face OCT observation of the choroid may be helpful to distinguish each condition

Drought Damage Assessment for Crop Insurance Based on Vegetation Index by Unmanned Aerial Vehicle (UAV) Multispectral Images of Paddy Fields in Indonesia

Drought is increasingly threatening smallholder farmers in Southeast Asia. The crop insurance system is one of the promising countermeasures that was implemented in Indonesia in 2015. Because the damage assessment in the present system is conducted through direct investigations based on appearance, it is not objective and needs a long time to cover large areas. In this study, we investigated a rapid assessment method for paddy fields using a vegetation index (VI) taken by an unmanned aerial vehicle (UAV) with a multispectral camera in 2019 and 2021. Then, two ways of assessment for drought damage were tested: linear regression (LR) based on a visually assessed drought level (DL), and k-means clustering without an assessed DL. As a result, EVI2 could represent the damage level, showing the tendency of the decrease in the value along with the increasing DL. The estimated DL by both methods mostly coincided with the assessed DL, but the concordance rates varied depending on the locations and the number of assessed fields. Differences in the growth stage and rice cultivars also affected the results. This study revealed the feasibility of the UAV-based rapid and objective assessment method. Further data collection and analysis would be required for implementation in the future

Central serous chorioretinopathy with and without steroids: A multicenter survey.

We investigated the rates of the use of steroids in Japanese central serous chorioretinopathy (CSC) cases and differences in the characteristics of CSC with and without steroids. A total of 538 eyes of 477 patients diagnosed with CSC, with 3 months or more of follow-up between April 2013 and June 2017 at 8 institutions. Patients with CSC with more than 3 months of follow-up were identified by OCT and fluorescein angiography at 8 institutions. Data collected included patient demographics, history of corticosteroid medication and smoking, spherical errors, findings of angiography, subfoveal choroidal thickness, and changes through the follow-up period. Differences in these findings were analyzed in cases with and without corticosteroid treatment. Among the 477 patients (344 men,133 women), 74 (15.5%) (39 men, 35 women) underwent current or prior steroid treatment. Cases with steroids were higher age (p = 0.0403) and showed no male prevalence, more bilateral involvement (p < 0.0001), and the affected eyes had multiple pigment epithelial detachment (p <0.0001), more fluorescein leakage sites (p < 0.0001), greater choroidal thickness (p = 0.0287) and a higher recurrence rate (p = 0.0412). Steroids can cause severer CSC through an effect on choroidal vessels and an impairment of retinal pigment epithelium