RATE OF AND TIME TO COMPLETE RETINAL VASCULARIZATION IN PREMATURE INFANTS AND ASSOCIATED FACTORS.

PURPOSE: To evaluate the rate of and time to complete vascularization in premature infants and to explore associated factors. METHODS: A monocentric, retrospective cohort study including 541 premature infants who underwent screening for retinopathy of prematurity (ROP) between July 2016 and June 2019. Patients underwent regular dilated fundus examinations with indirect ophthalmoscopy until complete vascularization. The worse eye of each patient was included for analyses. The proportion of infants with complete retinal vascularization at the last visit and the time to full vascularization was analyzed. RESULTS: Among all infants (average gestational age 31.29 ± 3.12 weeks), 490 (90.57%) had complete records of retinal vascularization outcomes, of whom 439 (89.59%) achieved complete vascularization. The average postmenstrual age for complete vascularization was 45.39 ± 11.04 weeks, and 95.22% achieved completion before 64 weeks of postmenstrual age. Retinopathy of prematurity developed in 118 (22.56%) infants; 33 (6.10%) received antivascular endothelial growth factor treatment. For all infants screened for ROP, lower birth weight, presence of ROP, and antivascular endothelial growth factor therapy predicted delayed complete vascularization; for infants diagnosed with ROP, only lower birth weight predicted delayed complete vascularization. Subgroup analysis showed significant differences between patients without ROP, with untreated ROP, and with treated ROP in time to complete vascularization and its rate (99.7%, 66.2%, and 16.7%, respectively). CONCLUSION: Lower birth weight predicted delayed complete vascularization. Antivascular endothelial growth factor therapy and the presence of ROP, including ROP severity, may also affect time to complete vascularization. These findings should help improve the understanding and management of persistent avascular retina in preterm infants.

CLINICAL CHARACTERISTICS AND PROGNOSTIC FACTORS AFFECTING CLINICAL OUTCOMES IN CYTOMEGALOVIRUS RETINITIS WITH OR WITHOUT HIV INFECTION.

PURPOSE: To explore the clinical features and outcomes of cytomegalovirus retinitis (CMVR) in patients with HIV and non-HIV. METHODS: This retrospective cohort study included all patients with CMVR in National Taiwan University Hospital from 2013 to 2018. Demographic data, clinical characteristics, CMVR recurrence, and overall survival were compared between the HIV and non-HIV groups. Generalized estimating equation models were implemented to analyze the risk factors of poor visual prognosis. The Kaplan-Meier survival analysis was performed to investigate recurrence and survival. RESULTS: A total of 66 patients (95 eyes) with CMVR were enrolled, with no significant differences between the HIV (41 patients; 61 eyes) and non-HIV (25 patients; 34 eyes) groups in initial/final visual acuity, lesion area, or viral loads. Poor visual outcome was associated with poor initial visual acuity, retinal detachment, and a higher plasma cytomegalovirus titer. The HIV group had significantly longer survival rate ( P = 0.033) and lower recurrence rate ( P = 0.01) than the non-HIV group, and it also presented with better prognosis in recurrence-free survival analysis ( P = 0.01). CONCLUSION: Patients with CMVR without HIV had higher mortality and recurrence rates than the HIV group. Risk factors of poor visual outcome included poor initial visual acuity, retinal detachment, and a high plasma cytomegalovirus titer.

Clinical manifestations and risk factors for poor visual outcomes of ocular sarcoidosis in Taiwan.

PURPOSE: To assess ocular involvement in sarcoidosis and to analyze the manifestations of ocular sarcoidosis (OS) in Taiwan. METHODS: A retrospective review was performed in 364 patients diagnosed as sarcoidosis at National Taiwan University Hospital from 2015 to 2019. The OS diagnosis was based on the revised criteria of International Workshop on Ocular Sarcoidosis. Demographics, clinical manifestations, treatment and effects, complications, visual outcomes and risk factors of poor vision (<20/200) were analyzed. RESULTS: A total of 122 eyes of 66 patients (13 males and 53 females) with OS were identified. Bilateral involvement accounted for 84.8%. Thirty-five patients were diagnosed with definite OS, 14 with presumed OS and 17 with probable OS. The average age was 51.9 ± 14.1 years, with no significant difference in sex. Most patients presented with panuveitis (61 eyes, 50.0%) and posterior uveitis (52 eyes, 42.6%). Systemic steroid was the mainstay treatment, and immunosuppressants or anti-metabolic agents were supplemented. Common complications included cataract (50.8%), ocular hypertension/glaucoma (25.4%) and posterior synechiae (20.5%). Forty-three eyes (36.1%) and 74 eyes (62.2%) had a final vision of 20/20 and 20/40 or better, respectively. The causes of poor visual outcome were diverse, and generalized estimating equations analysis indicated that female and poor initial vision were risk factors. CONCLUSION: Uveitis is an early sign of sarcoidosis and it might result in several complications. The overall visual outcomes were good if patients received proper treatment. Both poor initial vision and females that tended to have more severe complications were associated with poor outcome.

Clinical manifestations and genetic characteristics in the Taiwan thoracic aortic aneurysm and dissection cohort - a prospective cohort study.

BACKGROUND: Thoracic aortic aneurysm and dissection (TAAD) is a devastating but treatable disease if detected early. The clinical manifestations and genetic characteristics underlying TAAD patients in Taiwan, however, remain unclear. METHODS: We consecutively recruited patients referred for TAAD screening and/or management at a tertiary medical center in Taiwan. All patients received a comprehensive survey of the clinical manifestations and a genetic testing with a 29-gene next-generation sequencing (NGS) panel. RESULTS: Patients (n = 107) were referred for different reasons, and could be grouped into 4 categories: known aortic aneurysm or dissection (AoAD) (n = 57), Marfanoid features (n = 36), having family members of suspected AoAD (n = 11), and ectopic lens (n = 3). AoAD were confirmed in 73 (68.2%) of the entire cohort. Among all the clinical manifestations, skin striae distensae was the only physical sign that showed significant association with AoAD (p = 0.007 after adjusted). Disease-causing genes/variants were identified in 46 patients (43.0%); FBN1 was the most prevalent disease-causing gene, followed by TGFBR1, TGFBR2 and FBN2. A positive genetic testing was not only an independent predictor of AoAD (hazard ratio (HR) 3.468, 95% confidence interval (CI) [1.541-7.807], p = 0.003), but also had a higher chance of dissection among the patients with known dilated aorta (HR 4.552, 95% CI [1.578-13.135], p = 0.005). CONCLUSION: The presence of skin striae distensae may serve as a clinical cue for physicians to search for AoAD in subjects who are at risk. The NGS panel test not only helps confirm the diagnosis, but also stratify the risk of dissection among patients with dilated aorta.

Clinical outcomes of intravitreal methotrexate injection protocol with a reduced initial frequency for intraocular lymphoma.

BACKGROUND/PURPOSE: To investigate the clinical characteristics of intraocular lymphoma and to evaluate two protocols of intravitreal methotrexate injection. METHODS: A retrospective chart review was conducted of newly-diagnosed intraocular lymphoma patients between January 2013 and January 2018 at National Taiwan University Hospital. Patients were divided into two groups. In Group A, intravitreal methotrexate was administered weekly for the initial 8 weeks, every 2 weeks for the following 12 weeks, and then monthly for 7 months. In Group B, intravitreal methotrexate was administered twice a week for the initial 2 weeks, weekly for the subsequent 2 weeks, once every 2 weeks for the next 1 month, and monthly for the last 10 months. RESULTS: A total of 12 patients were analyzed in the study; seven of these patients were allocated to Group A. Differences in the overall survival and progression-free survival between the two groups did not yield statistical significance. The median visual acuity was improved from LogMAR 0.46 to LogMAR 0.30 with borderline significance in Group A (p = 0.053). Two of seven patients in Group A and five of five patients in Group B developed punctate keratitis during intravitreal methotrexate injection treatment. CONCLUSION: Intravitreal methotrexate is an effective and repeatable treatment for intraocular lymphoma. A new protocol with reduced frequency of intravitreal injections as shown in this study could potentially produce similar results without a worse prognosis, along with a decrease in the incidence of keratitis.

Clinical Manifestations and Outcomes of Tubercular Uveitis in Taiwan-A Ten-Year Multicenter Retrospective Study.

Background andObjectives: This 10-year multicenter retrospective study reviewed the clinical manifestations, diagnostic tests, and treatment modalities of tubercular uveitis (TBU), including direct infection and indirect immune-mediated hypersensitivity to mycobacterial antigens in Taiwan. Materials and Methods: This retrospective chart review of patients with TBU was conducted at 11 centers from 1 January 2008 to 31 December 2017. We used a multiple regression model to analyze which factors influenced best-corrected visual acuity (BCVA) improvement. Results: A total of 79 eyes from 51 patients were included in the study. The mean age was 48.9 ± 16.4 years. The mean change of LogMAR BCVA at last visit was -0.21 ± 0.45. Diagnostic tools used include chest X-ray, chest computed tomography, Mantoux test, interferon gamma release test (QuantiFERON-TB Gold test), intraocular fluid tuberculosis polymerase chain reaction, and bronchial alveolar lavage. The clinical manifestations included 48% posterior uveitis and 37% panuveitis. In the sample, 55% of the cases were bilateral and 45% unilateral. There was 60.76% retinal vasculitis, 35.44% choroiditis, 21.52% serpiginous-like choroiditis, 17.72% vitreous hemorrhage, 12.66% posterior synechiae, 6.33% retinal detachment, and 3.80% choroidal granuloma. Treatment modalities included rifampicin, isoniazid, pyrazinamide, ethambutol, oral steroid, posterior triamcinolone, non-steroidal anti-inflammatory drugs, vitrectomy, and immunosuppressants. BCVA improved in 53.2% of eyes and remained stable in 32.9% of eyes. In the final model of multiple regression, worse initial BCVA, pyrazinamide, and receiving vitrectomy predicted better BCVA improvement. Ethambutol was associated with worse visual outcomes. Seven eyes experienced recurrence. Conclusions: This is the largest 10-year multicenter retrospective study of TBU in Taiwan to date, demonstrating the distribution of clinical manifestations and clinical associations with better treatment outcomes. The study provides a comprehensive description of TBU phenotypes in Taiwan and highlights considerations for the design of further prospective studies to reliably assess the role of ATT and vitrectomy in patients with TBU.

Neurodevelopmental Outcomes after Bevacizumab Treatment for Retinopathy of Prematurity: A Meta-analysis.

PURPOSE: To evaluate neurodevelopmental outcomes after intravitreal bevacizumab (IVB) therapy in retinopathy of prematurity (ROP) infants compared with those not exposed to IVB. CLINICAL RELEVANCE: The primary concern regarding IVB treatment of ROP is the potential systemic side effects, especially the risk of causing severe neurodevelopmental impairment (sNDI). Results regarding neurodevelopmental outcomes after IVB therapy are conflicting. METHODS: We conducted a meta-analysis and searched PubMed, Embase, and Web of Science for related publications from inception through March 12, 2020. The eligibility criteria were as follows: comparative studies of ROP patients that (1) included IVB as a treatment arm, (2) included a control group without bevacizumab treatment, and (3) reported on at least 1 neurodevelopmental outcome, such as sNDI, Bayley Scales of Infant and Toddler Development, Third Edition (Bayley III), composition scores, or cerebral palsy (CP). The primary outcome was sNDI, with the odds ratio (OR) calculated. Secondary outcomes were mean differences (MDs) for cognitive, language, and motor scores (Bayley III) and OR for CP. The quality of evidence was assessed using the Grades of Recommendation, Assessment, Development, and Evaluation approach. RESULTS: Eight studies, 6 including laser-controlled ROP infants and 2 including ROP infants not requiring treatment, were included. The weighted OR for sNDI in the IVB group was 1.39 (95% confidence interval [CI], 0.98-1.97). The weighted MDs were -1.92 (95% CI, -4.73 to 0.88), -1.32 (95% CI, -4.65 to 1.99), and -3.66 (95% CI, -6.79 to -0.54) for cognitive, language, and motor scores in Bayley III, respectively. The OR for CP was 1.20 (95% CI, 0.56-2.55). No differences were observed between the preset subgroups comprising laser-controlled ROP infants and ROP infants not requiring treatment. The current quality of evidence was rated as low (sNDI and all Bayley III scores) to very low (CP). CONCLUSIONS: Risk of sNDI was not increased in ROP patients after IVB treatment. Bayley III scores were similar in the IVB and control groups, except for a minor difference in motor performance. These findings suggest that the risk of additional sNDI after IVB treatment is low. Randomized trials are warranted to provide a higher quality of evidence.

M1 ipRGCs Influence Visual Function through Retrograde Signaling in the Retina.

UNLABELLED: Melanopsin-expressing intrinsically photosensitive retinal ganglion cells (ipRGCs, with five subtypes named M1-M5) are a unique subclass of RGCs with axons that project directly to many brain nuclei involved in non-image-forming functions such as circadian photoentrainment and the pupillary light reflex. Recent evidence suggests that melanopsin-based signals also influence image-forming visual function, including light adaptation, but the mechanisms involved are unclear. Intriguingly, a small population of M1 ipRGCs have intraretinal axon collaterals that project toward the outer retina. Using genetic mouse models, we provide three lines of evidence showing that these axon collaterals make connections with upstream dopaminergic amacrine cells (DACs): (1) ipRGC signaling to DACs is blocked by tetrodotoxin both in vitro and in vivo, indicating that ipRGC-to-DAC transmission requires voltage-gated Na(+) channels; (2) this transmission is partly dependent on N-type Ca(2+) channels, which are possibly expressed in the axon collateral terminals of ipRGCs; and (3) fluorescence microscopy reveals that ipRGC axon collaterals make putative presynaptic contact with DACs. We further demonstrate that elimination of M1 ipRGCs attenuates light adaptation, as evidenced by an impaired electroretinogram b-wave from cones, whereas a dopamine receptor agonist can potentiate the cone-driven b-wave of retinas lacking M1 ipRGCs. Together, the results strongly suggest that ipRGCs transmit luminance signals retrogradely to the outer retina through the dopaminergic system and in turn influence retinal light adaptation. SIGNIFICANCE STATEMENT: Melanopsin-expressing intrinsically photosensitive retinal ganglion cells (ipRGCs) comprise a third class of retinal photoreceptors that are known to mediate physiological responses such as circadian photoentrainment. However, investigation into whether and how ipRGCs contribute to vision has just begun. Here, we provide convergent anatomical and physiological evidence that axon collaterals of ipRGCs constitute a centrifugal pathway to DACs, conveying melanopsin-based signals from the innermost retina to the outer retina. We further demonstrate that retrograde signals likely influence visual processing because elimination of axon collateral-bearing ipRGCs impairs light adaptation by limiting dopamine-dependent facilitation of the cone pathway. Our findings strongly support the hypothesis that retrograde melanopsin-based signaling influences visual function locally within the retina, a notion that refutes the dogma that RGCs only provide physiological signals to the brain.

Surgical result of pterygium extended removal followed by fibrin glue-assisted amniotic membrane transplantation.

BACKGROUND/PURPOSE: To report the recurrence rate and cosmetic results of conjunctival wound edge and caruncle, and complications after pterygium extended removal followed by fibrin glue-assisted amniotic membrane transplantation. METHODS: A prospective interventional cohort study enrolled 57 (58 eyes) patients undergoing pterygium extended removal followed by fibrin glue-assisted amniotic membrane transplantation. All patients received postoperative follow-up for at least 12 months. Recurrence rate was graded from 1 to 4, and cosmetic results of conjunctival edge and caruncle were graded from 1 to 5. RESULTS: The cohort included 48 eyes with nasal pterygium, 5 eyes with temporal pterygium, and 5 eyes with double pterygium. There were 81.0% (n=47), 0% (n=0), 12% (n=7), and 7% (n=4) of eyes with Grades 1-4 recurrence, respectively. The cosmetic results of conjunctival wound edge and caruncle in cases with nasal pterygium showed 59.3% (n=32), 14.8% (n=8), 9.3% (n=5), 16.6% (n=9), and 0% (n=0) of eyes with Grades 1-5 morphology, respectively. Overall, 5.1% (n=3), 3.4% (n=2), 3.4% (n=2), 3.4% (n=2), 1.7% (n=1), 6.9% (n=4), and 1.7% (n=1) of patients suffered from postoperative pyogenic granuloma, transient diplopia, permanent motility restriction, steroid glaucoma, fat prolapse, subamniotic membrane hemorrhage, and early detachment of amniotic membrane, respectively. CONCLUSION: Pterygium extended removal followed by fibrin glue-assisted amniotic membrane transplantation results in low recurrence, satisfactory cosmetic results and a low incidence of additional complications.

Clinical outcomes of primary intraocular lymphoma patients treated with front-line systemic high-dose methotrexate and intravitreal methotrexate injection.

A standard treatment for patients with primary intraocular lymphoma (PIOL) remains unclear. This study retrospectively analyzed the clinical features and outcomes of 19 patients with PIOL who were treated with a first-line therapy comprising combined intravenous high-dose methotrexate and intravitreal methotrexate between January 2003 and December 2013. Thirteen (68.4 %) patients were female, and the median age at diagnosis was 57 (39-77 years). Diagnoses were based on the identification of abnormal lymphoid cells in vitreous fluid. Ten (52.6 %) patients had bilateral eye involvement, and six had concurrent central nervous system (CNS) involvement. All 19 patients achieved complete remission (CR) as confirmed by cytological examination of vitreous and cerebrospinal fluid and brain imaging if CNS was involved. Patients with concurrent brain involvement required a longer time to achieve CR. However, the duration of complete remission did not differ between patients with and without CNS involvement. The 5-year overall survival rate was 55.8 % for the total cohort and was higher (68.8 %) in patients with isolated PIOL than in those with concurrent CNS involvement. In all patients, methotrexate treatment was well tolerated, with manageable side effects. We conclude that combined intravitreal methotrexate and systemic high-dose methotrexate treatment is effective in patients with PIOL.

Clinical and histological features of epiretinal membrane after diabetic vitrectomy.

PURPOSE: To investigate the clinical, histopathological, and surgical features of the epiretinal membrane (ERM) after diabetic vitrectomy (DV). METHODS: From August 2007 to January 2010, clinical charts of consecutive proliferative diabetic retinopathy (PDR) cases with significant post-DV ERM, defined as thickened membrane causing macular distortion and vision decrease, were enrolled as the study group; PDR cases without post-DV ERM in 24 months follow-up served as the control group. Factors associated with post-DV ERM formation, morphological and visual changes before and after ERM surgery, and histopathological features were analyzed. RESULTS: Sixteen eyes were in the ERM group, while 60 eyes were in the control group. Active PDR (p < 0.001), fibrovascular proliferation (FVP) grade (p = 0.001), post-DV hemorrhage (p = 0.012), and residual fibrovascular stump (p = 0.002) were factors significantly associated with post-DV ERM. Most membranes (87.5 %) developed within 12 months, were widespread beyond the arcade (81.3 %), and connected with retinal vessels (87.5 %). After surgery, significant VA improvement was achieved. ERM recurrence was noted in six eyes (37.5 %). Histopathological examinations of ERMs in six cases showed abundant collagen fibers with epithelial cells. Immunohistochemical staining with CD 34 demonstrated the presence of vascular endothelium in two of the six specimens. CONCLUSION: The post-DV ERM is a complex tissue with variable vascularity that often presents with widespread distribution, rapid progression, and causes macular distortion. Associated risk factors include active PDR, high FVP grade, post-DV hemorrhage, and residual fibrovascular stumps. Membrane removal surgery may be beneficial in selected cases, but recurrence is not uncommon.

Long-term outcome of foveolar internal limiting membrane nonpeeling for myopic traction maculopathy.

PURPOSE: To investigate the long-term results of a novel technique to preserve the foveolar cone without peeling off the foveolar internal limiting membrane (ILM) during myopic traction maculopathy surgery. METHODS: Nineteen patients (19 eyes) were retrospectively studied and divided into 2 groups by the extent of ILM peeled and followed for more than 3 years. Group 1: foveolar ILM nonpeeling group (FN) (12 eyes) and Group 2: total peeling of foveal ILM group (TP) (7 eyes). A donut-shaped ILM was peeled off, leaving a 400-µm diameter ILM over foveola with a sharp margin in FN group. RESULTS: Macular hole was developed in 2 of the 7 eyes (28.6%) in the TP group and none in the FN group. Long-term central fovea thickness thinning and decrease of vision were found in the TP group, but not in the FN group (P < 0.05). Inner segment/outer segment line recovered in 75% of the 12 eyes in the FN group, but in only 14.3% of the 7 eyes in the TP group. CONCLUSION: Preservation of the foveolar cone by foveola nonpeeling surgery correlates with better anatomical and visual results than total peel, prevents long-term foveolar retinal thinning, and successfully saves the fovea from macular hole formation.

Macular tractional retinoschisis in proliferative diabetic retinopathy: clinical characteristics and surgical outcome.

PURPOSE: To evaluate the clinical characteristics and surgical outcome of macular tractional retinoschisis (TRS) as compared with tractional retinal detachment (TRD) in proliferative diabetic retinopathy (PDR). METHODS: This retrospective, longitudinal study collected consecutive cases with optical coherence tomography (OCT)-confirmed TRS and TRD between January 2007 and June 2013. All cases had surgical treatment. Their preoperative findings and follow-up data were subsequently compared. RESULTS: Thirty-two eyes (32 patients) with TRS and 32 eyes (32 patients) with TRD were included. The TRS group had more clinically inactive fibrosis and less extensive fibrovascular proliferation than the TRD group. The involved area and height of the schisis varied among cases; associated macular abnormalities included inner macular cyst (43.75%), lamellar hole (12.5%) and foveal detachment (9.38%); sequential OCT in some cases showed a progression of macular changes. Both groups had significant visual improvement after surgery. Of the patients with TRS, 40.62% had residual retinoschisis. Preoperative best-corrected visual acuity (BCVA), postoperative BCVA and improvement in visual acuity showed no significant difference between TRS and TRD. CONCLUSIONS: Unique features exist for macular TRS in PDR. Visual function may benefit from vitreous surgery despite residual macular abnormalities in some cases.

Cataract extraction surgery in patients with uveitis in Taiwan: risk factors and outcomes.

BACKGROUND/PURPOSE: To analyze the preoperative conditions and postoperative outcomes of phacoemulsification and intraocular lens implantation in Taiwanese patients with uveitis. METHODS: This retrospective, consecutive case series study included 121 eyes of 84 patients with uveitis who underwent phacoemulsification and intraocular lens implantation from July 1996 to May 2006. The demographic data, postoperative outcomes, complications, and risk factors with regard to visual prognosis were analyzed. Visual acuity was converted from the Snellen equivalent to logMAR values. RESULTS: This study included 51 men and 70 women with a mean ± SD age of 44.6 ± 18.4 years. The three most common diseases were idiopathic uveitis, Behcet's disease, and Vogt-Koyanagi-Harada disease. The best-corrected visual acuity improved from 1.52 ± 0.83 logMAR units preoperatively to 0.37 ± 0.59 logMAR units postoperative (p < 0.001). The most frequent postoperative complications were posterior capsular opacity (24 eyes) and cystoid macular edema (10 eyes). Anterior uveitis related to HLA-B27 had the best visual prognosis, whereas Behcet's disease had the poorest visual outcome (p = 0.029). Logistic regression analysis indicated that disease etiology (p = 0.011) and preoperative visual acuity (p = 0.020) were related to the postoperative visual prognosis. CONCLUSION: Cataract extraction can improve visual function for most patients with uveitis. Postoperative complications were not uncommon and Behcet's disease had the poorest postoperative visual prognosis.

Diagnosis and treatment of syphilitic uveitis: Taiwan experts consensus.

A recent resurgence in the incidence of syphilis has sparked a new interest in this old disease. Syphilitic uveitis remains a challenging disease, among the variable syphilis infections, due to the diagnostic complexity and the wide clinical manifestations. Here, we provide recommendations regarding clinical manifestations, diagnosis, and treatment for patients with syphilitic uveitis in Taiwan based on an expert meeting and consensus from experienced uveitis specialists.

Relationship between genotype, phenotype, and refractive status in patients of inherited retinal degeneration.

BACKGROUND: To elucidate the relationship between inherited retinal disease, visual acuity and refractive error development in Asian patients. SUBJECTS: Five hundred phakic eyes with refractive data were analysed in this retrospective cohort. Diseases were categorized by clinical phenotypes, and the prevalent genotypes identified in the Taiwan Inherited Retinal Degeneration Project were analysed. Consecutive surveys in Taiwan have provided the rates of myopia in the general population. RESULTS: No differences were observed among the disease phenotypes with respect to myopia (P = 0.098) and high myopia rates (P = 0.037). The comparison of refractive error between retinitis pigmentosa and diseases mainly affecting the central retina showed no difference, and the refraction analyses in diseases of different onset ages yielded no significance. Moreover, there was no difference in the myopia rate between the diseases and general population. Among the genotypes, a higher spherical equivalent was seen in RPGR and PROM1-related patients and emmetropic trends were observed in patients with CRB1 and PRPF31 mutations. Furthermore, significantly poorer visual acuity was found in ABCA4, CRB1 and PROM1-related patients, and more preserved visual acuity was seen in patients with EYS, USH2A, and RDH12 mutations. CONCLUSIONS: No significant differences were observed in visual acuity, refractive state and myopia rate between patients with inherited retinal disease and the general population, and different subtypes of inherited retinal disease shared similar refractive state, except for higher cylindrical dioptres found in patients with Leber's congenital amaurosis. The heterogeneity of disease-causing genes in Asian patients may lead to variable refractive state.

Surgical management and immunohistochemical study of corneal plaques in vernal keratoconjunctivitis.

Two children with shield ulcer in vernal keratoconjunctivitis unresponsive to steroid therapy received plaque removal by superficial keratectomy, followed by amniotic membrane transplantation (AMT). Hematoxylin and eosin staining of the excised corneal specimen revealed a thick layer of eosinophilic material attached to the Bowman's layer. These deposits were positive for eosinophil granule major basic protein, as confirmed by an immunohistochemical study. The shield ulcer healed after the amniotic membrane was removed. No recurrent corneal plaque developed, although corneal opacity complicated in both cases. Lamellar keratectomy with AMT offers an effective management by removing the cytotoxic plaques and protecting the denuded stroma from deposition of inflammatory debris.

Pupil centroid shift and cyclotorsion in bilateral wavefront-guided laser refractive surgery and the correlation between both eyes.

BACKGROUND/PURPOSE: Accuracy of ocular alignments is emphasized in laser refractive surgery. We evaluate pupil centroid shift and cyclotorsion and the correlation between both eyes in bilateral wavefront-guided laser refractive surgery. METHODS: A retrospective study was performed to analyze pupil centroid shift and cyclotorsion using an iris registration system of Zyoptix 100 platform in 186 eyes of consecutive 93 patients at National Taiwan University Hospital. Pearson's correlation analysis was used. RESULTS: The mean pupil centroid shift was 0.179±0.096mm, and 42.2% of eyes had more than 0.2mm shift between wavefront measurement with dilated pupil and laser ablation with undilated pupil. When the pupil was pharmacologically dilated, pupil centers predominantly shifted to inferonasal direction (59% of eyes). The vertical shift was larger than the horizontal shift. The correlations between both eyes in horizontal and vertical shifts were statistically significant, indicating a symmetric mirror pattern. A good opposite correlation of pupil centroid shifts was observed between in wavefront measurement and in laser treatment. The mean amount of cyclotorsion between the seated and supine positions was 3.22±2.53° with a maximum of 13.51°. A total of 112 eyes (60.2%) had cyclotorsion > 2°, while 39 eyes (21.0%) had cyclotorsion > 5°. Moderate correlation was observed between cyclotorsion of both eyes and was statistically significant. CONCLUSION: Inferonasal pupil centroid shift as the pupil pharmacologically dilated and a significant amount of cyclotorsion with good correlation between both eyes was observed in refractive surgery and could be compensated by iris registration.

Long-term outcomes of combined intravitreal methotrexate and systemic high-dose methotrexate therapy in vitreoretinal lymphoma.

OBJECTIVE: The optimal treatment for vitreoretinal lymphoma (VRL) remains a challenge, as central nervous system (CNS) relapse occurs frequently, leading to the worst impact on survival. We previously proposed combined intravitreal methotrexate and systemic high-dose methotrexate therapy for this disease. This study aimed to report the long-term outcomes of patients with VRL using this combination treatment. METHODS: We conducted a retrospective cohort study on patients with VRL at a tertiary referral center between 2003 and 2018. RESULTS: Thirty-two patients were included, of whom 23 had primary VRL (PVRL) and nine had concurrent intraocular and CNS diseases. The treatment was well tolerated. Twenty-six (81.3%) patients achieved complete response (CR). After a median follow-up time of 103.5 months, the 5-year survival rate was 73.3%, whereas the 5-year progression-free survival (PFS) rate was 29.9%. Twenty-four (75%) patients relapsed, including 12 with isolated intraocular relapses at first relapse and a total of 17 with CNS/systemic relapses. The development of CNS/systemic relapse negatively affected survival, but intraocular relapse did not. The median CNS/systemic PFS was 69.5 months, but the risk of CNS/systemic relapse increased steadily with a cumulative incidence rate at 2, 5, and 10 years being 22.6%, 44.2%, and 65%, respectively. Multivariate analysis identified concurrent CNS disease at diagnosis as the only poor-risk factor for CNS/systemic relapse. CONCLUSIONS: This study confirms good efficacy and acceptable toxicities of the combination approach. However, incorporation of further intensive consolidation strategies into the treatment protocol to effectively prevent subsequent CNS/systemic relapse deserves to be considered.

Diagnosis and Treatment of Tubercular Uveitis in Taiwan - Consensus of Expert Panels.

There is currently a lack of guidelines with regard to tubercular uveitis (TBU) management in Taiwan. We therefore propose an evidence-based consensus on the management for TBU. The Taiwan Ocular Inflammation Society conducted a meeting that included nine ophthalmologist and one infection disease expert that focused on three broad areas of (1) nomenclature for TBU, (2) assessment and diagnosis for TBU, and (3) treatment of TBU. Brief literature review on TBU diagnosis and management was conducted that informed this panel meeting in order to make decisions on each consensus statements. In terms of our results, a consensus statements and recommendations for the diagnosis and management of TBU were developed. This consensus statement provides an algorithmic approach toward diagnosing and managing TBU. These statements are meant to enhance but not replace individual clinician-patient interactions and to facilitate real-world clinical practice improvement in terms of TBU patients care.