Familial exudative vitreoretinopathy (FEVR) is a hereditary vitreoretinal disorder that can cause various types of retinal detachments. The abnormalities in eyes with FEVR are caused by poor vascularization in the peripheral retina. The genetics of FEVR is highly heterogeneous, and mutations in the genes for Wnt signaling and a transcription factor have been reported to be responsible for FEVR. These factors have been shown to be the regulators of the pathophysiological pathways of retinal vascular development. Studies conducted to identify the causative genes of FEVR have uncovered a diverse and complex relationship between FEVR and other diseases; for example, Norrie disease, a Mendelian-inherited disease; retinopathy of prematurity, a multifactorial genetic disease; and Coats disease, a nongenetic disease, associated with pediatric retinal detachments.

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The application of digital imaging technologies including confocal scanning laser ophthalmoscopy (CSLO), optical coherence tomography (OCT), and scanning laser polarimetry (SLP) has significantly improved the detection of optic nerve head (ONH) deformation and progressive retinal nerve fiber layer (RNFL) thinning for assessment of glaucoma progression. Algorithms for change analysis such as topographic change analysis and guided progression analysis perform event analysis of serial ONH surface height topology maps and RNFL thickness/RNFL retardance maps, respectively, providing a topographical display of the location of significant change. With spectral-domain OCT, it is feasible to delineate and measure the lamina cribrosa surface depth in addition to ONH surface depth and RNFL thickness. Growing evidence from experimental and clinical studies indicates that ONH and lamina cribrosa deformation can be observed prior to detectable RNFL thinning and functional loss in glaucoma. These findings lend support to the notion that upon detection of ONH/lamina cribrosa deformation, a time window for therapeutic intervention for better outcomes may exist. The ONH and the lamina cribrosa are therefore important targets for monitoring glaucoma progression. This review summarizes the latest findings comparing the performance of OCT, CSLO, and SLP for detection of progressive ONH and RNFL damages in glaucoma patients and the clinical implication and limitations of studying the morphological alteration of the ONH, lamina cribrosa, and RNFL in the assessment of glaucoma progression.

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Purpose: To describe a case series in which corneal fluorescein staining (CFS) development occurred in short break-up time (s-BUT) dry eyes after a short period during prolonged opening of the eye. Methods: The study was designed as a clinical case series. Ocular surface evaluations were performed on 13 individuals with s-BUT dry eye. Tear function examinations included Schirmer’s test and BUT evaluation. Results: In all 13 cases, the BUT was short, but the tear quantity was not so bad. In all cases, CFS developed following a single eye opening, and the staining was observed at sites that showed as dark spots. In several cases, the CFS disappeared later. Conclusion: In this study, we demonstrated that CFS could develop following a single eye opening. Based on our findings, CFS is a dynamic phenomenon rather than a stable indicator of ocular surface abnormalities. Moreover, s-BUT dry eye has the potential to show ocular surface abnormalities.

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Purpose: To analyze visual acuity (VA) improvement, causes of low vision (LV), and quality of life (QOL) following the use of low vision aids (LVAs) in children with LV. Methods: A prospective analysis was conducted on children with LV aged between 4 years and 18 years between March 2013 and October 2013. Children were recruited from both urban schools and rural schools. LVAs were tried for visual improvement, and improved VA was noted. All children were trained to use the aid and followed up monthly for 3 consecutive months for VA improvement; QOL through a questionnaire was analyzed after the use of LVAs. Results: A total of 74 children (148 eyes; 50% male; mean age, 11.8 ± 3.2 years) were analyzed, where 34 children were recruited from rural areas and 40 from urban schools. After LVA use, 101 (68.24%) eyes of 59 (79.72%) children improved for distance with telescope and 81 (54.72%) eyes of 51 (68.91%) children improved for near with magnifiers. LV due to retinal problems, optic atrophy, congenital anomalies, and amblyopia drastically reduced after use of LVA. A statistically significantly higher proportion of children had either “excellent” or “good” QOL, and a significantly lower proportion of children had either “not satisfactory” or “poor” QOL after the use of LVA (p < 0.0001). Conclusion: LVA is essential and effective in improving VA and QOL in children with LV.

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This article reports the clinical course and treatment of ocular adnexal lymphoma based on a retrospective review of five cases with a histologically approved ocular adnexal lymphoma at Kaohsiung Veterans General Hospital over 10 years. Extranodal B-cell lymphoma in the orbit, lacrimal gland, eyelid, or conjunctiva was found in these patients. Four of them were female, and they were aged 45–64 years. All patients were also consulted with hematologists for possible systemic involvement and therapeutic plan. The patient with retrobulbar and orbital apex involvement received systemic chemotherapy. The patient with lacrimal gland involvement experienced tumor recurrence after local excision, and therefore received adjuvant radiotherapy. The remaining three patients had localized lymphoma on the eyelid or bulbar conjunctiva, and they all showed no recurrence after surgical excision. The incidence of ocular adnexal lymphoma has risen worldwide over the last few decades. Although most cases are confined to ocular adnexal, some may also be associated with disseminated lymphoma. Accurate diagnosis and staging is mandatory for appropriate treatment. Generally speaking, localized and low-grade ocular adnexal lymphoma involved eyelid or conjunctiva seem to have good outcome after surgical excision only. Systemic chemotherapy should be considered in patients with advanced disease or systemic manifestations, and radiotherapy also offers a good choice for lacrimal gland lymphoma.

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Posterior subtenon injections of steroidal drugs are commonly used to treat various chorioretinal inflammatory diseases. Subsequent strabismus is rare, but it is associated with severe visual disturbance when present. We report a case of simultaneous bilateral inferior oblique muscle paresis in a 60-year-old man who developed it after receiving intravitreal injections of bevacizumab and posterior subtenon injections of triamcinolone acetonide for the treatment of diabetic macular edema. The patient complained of excessive pain during the injections, which were performed at the 6 o’clock position in both eyes. According to the literature, inferior oblique muscle paresis after posterior subtenon injections is rare. This complication may be prevented by proper selection of the injection site and paying attention to any unusual patient complaints during the procedure.

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A case of Stevens–Johnson syndrome in a healthy 58-year-old woman who underwent cataract surgery under topical anesthesia is reported. General skin erosions developed 2 hours after surgery. The patient’s family doctor diagnosed that she was allergic to seafood. One month later, she underwent phacoemul-sification surgery in the other eye. After surgery, she developed Stevens–Johnson syndrome with general skin lesions, erythema nodosa, genital mucosa erosion, oral ulcers, gastritis, and conjunctiva edema. The symptoms subsided 2 weeks later after immunotherapy. Although nonpreserved anesthesia (2% lido-caine) has seldom been reported to cause allergic reactions via the intravenous or the intramuscular route, it is possible that an intracameral injection of 0.2% lidocaine during cataract surgery can induce Stevens–Johnson syndrome. Careful evaluation of the patient’s history and proper treatment were recommended to prevent additional complications.

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Purpose: To investigate the distribution of corneal and ocular spherical aberrations (SAs) in eyes with cataract in the Taiwanese population. Methods: Corneal and ocular SAs were measured in the central 6-mm optical zone using wavefront aberrometry. Axial length (AL) and keratometry (K) were also evaluated in each eye. Results: A total of 413 eyes in 234 patients were analyzed. The mean age of the patients was 66.8 ± 10.64 years. The mean AL and K values were 24.32 mm and 44.08 D, respectively. The mean corneal SA was 0.307 ± 0.135 μm and ocular SA was −0.042 ± 0.487 μm. Ocular and corneal SAs were significantly correlated (r² = 0.04, p < 0.001). Corneal and ocular SAs were not significantly correlated with K (p = 0.096 and p = 0.634, respectively), but were significantly correlated with AL (p < 0.001). Multilinear regression showed that corneal SAs and age were the dependent variables that predicted ocular SAs (r² = 0.143, F = 13.65, p < 0.01), especially in patients who were aged > 50 years, for whom a strongly significant positive correlation was found (r² = 0.102, F = 11.10, p < 0.001). Conclusion: Corneal and ocular SAs varied among cataract patients and correlated with AL. After 50 years of age, ocular SAs increased significantly because of an increase in internal (lenticular) SAs. Corneal SAs in Taiwanese patients were larger than those in Japanese patients and similar to those in Chinese and Malaysian populations. Preoperative measurement of wavefront aberrations is necessary to select which aspherical intraocular lenses are most suitable for achieving better postoperative visual quality.

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Background/Purpose: The purpose of this study is to share experiences diagnosing and treating choroidal neovascularization (CNV) in young patients (age ≤ 50 years) at our hospital. Methods: The study reviewed retrospective data of patients (≤ 50 years old) with CNV who received antivascular endothelial growth factor treatment (anti-VEGF) between January 2007 and August 2012 at Shin Kong Wu Ho-Su Memorial Hospital. We recorded the total number of injections, types of drugs, preoperative and final best-corrected visual acuity (BCVA), central retinal thickness (CRT) in optical coherence tomography (OCT), and total follow-up times, and then used two-tailed paired t tests to compare mean changes in BCVA and CRT on OCT. Results: The study enrolled 59 patients ≤ 50 years of age with CNV diagnosed in 67 eyes. The mean age was 36.9 ± 10.0 years (range, 8–50 years). Twenty-one patients were male and 38 patients were female. Forty-two CNV lesions were subfoveal, 19 were juxtafoveal, and five were extrafoveal. The mean total follow-up time was 18.5 ± 19.9 months (range, 0.5–71 months). Pathologic myopia was the most common cause of CNV in this study (47.8%), followed by punctate inner choroidopathy (17.9%), idiopathic CNV (16.4%), polypoidal choroidal vasculopathy (13.4%), angioid streaks (3.0%), and choroidal rupture (1.5%). After anti-VEGF treatment, the mean BCVA improved from 0.69 ± 0.61 to 0.42 ± 0.59 (p < 0.05). CRT decreased from 257.5 ± 48.2 to 210.3 ± 35.7 (p < 0.05). The mean number of injections was 1.9 ± 1.6 (range, 1–9). Conclusion: In this study we found that pathologic myopia, punctate inner choroidopathy, and idiopathic and polypoidal choroidal vasculopathy comprised the four most common causes of CNV in patients ≤ 50 years of age in Taiwan. We also revealed that anti-VEGF treatment is highly effective in the treatment of CNV in this age group.

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A 30-year-old man presented with glaucomatocyclitic crisis, also known as Posner–Schlossman syndrome, and acute intraocular pressure elevation. Changes in the longitudinal retinal nerve fiber layer (RNFL) and the cup-to-disc (C/D) ratio of the optic disc were detected by Stratus optical coherence tomography (OCT). The average RNFL thickness in the affected eye was greater than that in the unaffected eye on Day 3 (132.99 μm, C/D ratio: 0.24 vs. 105 μm, C/D ratio: 0.26). However, the RNFL thickness continued to decrease and the C/D ratio progressively increased in the affected eye over 12 months (60 μm, C/D ratio: 0.67), although the intraocular pressure was controlled at <21 mmHg during that period. Glaucomatous visual field defects were also found. A progressive decrease in the thickness of the RNFL was demonstrated by Stratus optical coherence tomography following an elevation in the acute intraocular pressure in the patient. Prompt treatment and longitudinal monitoring are necessary to prevent and detect glaucomatous damage.

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There are yet no reports in the literature describing the mechanism of macular hole (MH) formation associated with fibrovascular proliferation in proliferative diabetic retinopathy. We report four cases of MHs in diabetic retinopathy with fibrovascular proliferation; formation of MHs were studied using sequential pre-MH optical coherence tomography (OCT). In Case 1, initial OCT revealed tractional schisis and cysts with fovea detachment. An MH with bowl-shaped detachment was noted within 6 weeks. In Case 2, initial OCT revealed thickened posterior hyaloid membrane with vitreomacular traction. Five and a half years later, OCT showed MH formation with possible vitreomacular separation. Some epiretinal membrane was also noted in the macula area. In Case 3, initial OCT revealed tractional retinal elevation from the superonasal area to the fovea with macular thinning. An MH with detachment developed 7 weeks later. In Case 4, initial OCT revealed macula-involved retinal detachment with traction. An MH was noted 4 weeks later. The analysis of sequential OCT findings in these four cases suggests that strong vitreoretinal adhesion and traction of fibrovascular proliferation may induce an MH without going through the same evolutionary phases as those characteristic of idiopathic MHs.

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