scholarly journals Long-Term Follow-Up in Bietti Crystalline Dystrophy

2007 ◽  
Vol 17 (4) ◽  
pp. 680-682 ◽  
Author(s):  
A.M. Mansour ◽  
S.H. Uwaydat ◽  
C.-C. Chan

Purpose To present a long-term follow-up of Bietti crystalline dystrophy. Methods Two brothers are presented including the clinical findings, fluorescein angiography, electrophysiology (electroretinography [ERG], electrooculography [EOG], adaptometry), optical coherence tomography (OCT), and transmission electron microscopy of bulbar conjunctiva and peripheral blood lymphocytes. The clinical findings were documented over a period of 25 years in one brother and 5 years in the other. Results The most striking features were deposits in the retina that were formed de novo with old ones replaced by choroidal atrophy in advanced stage of the disease. The light rise (EOG), rod- and cone-driven responses (ERG), and visual fields were affected progressively during the course. These changes of the retinal pigment epithelium and choriocapillaris were observed in the second decade and worsened gradually. OCT demonstrated preferential crystal accumulation in the inner retina. Cytoplasmic lipid crystalline inclusions were found in lymphocytes and conjunctival fibroblasts by transmission electron microscopy. Conclusions Bietti crystalline retinopathy is a progressive retinal disease characterized by retinal crystals gradually replaced by atrophy of the retinal pigment epithelium and gradual constriction of visual fields.

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Martin Stattin ◽  
Stefan Hagen ◽  
Daniel Ahmed ◽  
Eva Smretschnig ◽  
Florian Frommlet ◽  
...  

Purpose. To evaluate normalized short-wavelength fundus autofluorescence (SW-FAF) imaging changes over time as a predictive parameter for the retinal pigment epithelium (RPE) function in eyes compromised by acute central serous chorioretinopathy (CSCR) after indocyanine green angiography-guided verteporfin (Visudyne®, Novartis Pharma, Basel, Switzerland) photodynamic therapy (PDT) with a half-fluence rate (25 J/cm2). Methods. Quantitative data of SW-FAF grey values (SW-FAF GV) from a 350 μm (SW-350) and 1200 μm (SW-1200) diameter circle centered on the fovea and normalized with the level of SW-FAF GV in a 30° image of 20 eyes in 11 patients initially treated for unilateral acute symptomatic CSCR were collected and retrospectively analyzed after 7 years. A 2-sided t-test was calculated to explore the differences of SW-350 and SW-1200 between one month and the long-term follow-up. Results. Mean differences (95% CI) in SW-FAF GV between 1 month and 7 years after half-fluence PDT were 0.07 ± 0.11 for SW-350 ([95% CI: −0.002; 0.14], p=0.06) and 0.11 ± 0.15 for SW-1200 ([95% CI: 0.01; 0.21], p=0.03). Mean differences in SW-FAF GV of the contralateral untreated eye were 0.06 ± 0.14 for SW-350 ([95% CI: −0.04; 0.17], p=0.22) and 0.05 ± 0.13 for SW-1200 ([95% CI: −0.04; 0.15], p=0.22). Conclusion. After 7 years, normalized SW-FAF GV were significantly lower in eyes with resolved acute CSCR treated with reduced-fluence PDT compared to the follow-up after 1 month without correlation to explicit pattern changes or structural damages. Half-fluence PDT remains a safe and considerable treatment option in acute CSCR.


2018 ◽  
Vol 9 (1) ◽  
pp. 215-220 ◽  
Author(s):  
Yuka Ito ◽  
Masahito Ohji

Background: Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare benign tumor of the retinal pigment epithelium characterized by a focal, darkly pigmented nodule in the macular lesion in healthy persons. We report a case of CSHRPE with long-term follow-up. Case: A 41-year-old Japanese woman was incidentally discovered to have a dark lesion on the fundus of the left eye. We evaluated the patient by measuring her best-corrected visual acuity (BCVA) and by slit-lamp biomicroscopy, fundus color photography, and optical coherence tomography (OCT) over a 10-year period. The BCVA gradually declined during the early follow-up period, having decreased from 1.2 to 0.8 in the left eye 3 years after the initial examination, and then has been maintained for the following 7 years. The lesion did not show a change in OCT 10 years after the first examination. Conclusion: It is important to follow a CSHRPE carefully over the long term because visual acuity might decrease.


2015 ◽  
Vol 2 (1) ◽  
pp. 37-40
Author(s):  
Yacouba Njankouo Mapoure ◽  
◽  
Namme Henry Luma ◽  
Cyrille Nkouonlack ◽  
Ariane Vanessa Pokossy ◽  
...  

Neurosurgery ◽  
2020 ◽  
Vol 88 (1) ◽  
pp. 106-112 ◽  
Author(s):  
Young Soo Chung ◽  
Minkyun Na ◽  
Jihwan Yoo ◽  
Woohyun Kim ◽  
In-Ho Jung ◽  
...  

Abstract BACKGROUND Compressive optic neuropathy is the most common indication for transsphenoidal surgery for pituitary adenomas. Optical coherence tomography (OCT) is a useful visual assessment tool for predicting postoperative visual field recovery. OBJECTIVE To analyze visual parameters and their association based on long-term follow-up. METHODS Only pituitary adenoma patients with abnormal visual field defects were selected. A total of 188 eyes from 113 patients assessed by visual field index (VFI) and 262 eyes from 155 patients assessed by mean deviation (MD) were enrolled in this study. Postoperative VFI, MD, and retinal nerve fiber layer (RNFL) thickness were evaluated and followed up. After classifying the patients into normal (>5%) and thin (<5%) RNFL groups, we investigated whether preoperative RNFL could predict visual field outcomes. We also observed how RNFL changes after surgery on a long-term basis. RESULTS Both preoperative VFI and MD had a linear proportional relationship with preoperative RNFL thickness. Sustained improvement of the visual field was observed after surgery in both groups, and the degree of improvement over time in each group was similar. RNFL thickness continued to decrease until 36 mo after surgery (80.2 ± 13.3 μm to 66.6 ± 11.9 μm) while visual field continued to improve (VFI, 61.8 ± 24.5 to 84.3 ± 15.4; MD, −12.9 ± 7.3 dB to −6.3 ± 5.9 dB). CONCLUSION Patients with thin preoperative RNFL may experience visual recovery similar to those with normal preoperative RNFL; however, the probability of normalized visual fields was not comparable. RNFL thickness showed a strong correlation with preoperative visual field defect. Long-term follow-up observation revealed a discrepancy between anatomic and functional recovery.


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