Search

To investigate the association between clinical features of chronic central serous chorioretinopathy (CSC) and subsequent development of polypoidal choroidal vasculopathy (PCV). Characteristics and treatment response of PCV secondary to CSC were described. This retrospective observational study included 18 patients with chronic CSC (18 eyes) with subsequent PCV and 36 controls (36 eyes) with chronic CSC without PCV development during follow-up. Clinical features were compared between the two groups. A logistic regression model was used to evaluate the risk factor of PCV formation. Treatments for PCV included anti-vascular endothelial growth factor (VEGF) monotherapy, photodynamic therapy (PDT), or PDT and anti-VEGF combination treatment. Subretinal fluid on optical coherence tomography images were assessed after treatments. Significant between-group differences were observed in best-corrected visual acuity after disease resolution and presence of pachyvessels (P = .001 and P = .003, respectively). The presence of pachyvessels in chronic CSC was associated with subsequent PCV (odds ratio = 6.00; 95% CI, 1.74–20.68; P = .005). CSC recurrence and subfoveal choroidal thickness (SFCT) were not significantly associated with subsequent PCV development (P = .393 and P = .911, respectively). The mean age of PCV diagnosis was 51 years, and the mean time from CSC diagnosis to PCV confirmation was 77.8 months. The mean (range) SFCT of PCV was 327.7 (134–599) μm. Nine patients received anti-VEGF monotherapy and 5 had disease remission. Four patients received PDT and anti-VEGF combination treatment and all of the 4 had disease remission. In chronic CSC, pachyvessel characteristics are associated with subsequent PCV development. This result will assist clinicians to evaluate CSC in clinical practice and provide insights into the pathogenesis of PCV.

To evaluate a 10-year visual outcome of endogenous endophthalmitis (EE) patients. A 10-year retrospective chart review of EE patients. Thirty-eight patients (40 eyes) were diagnosed with EE at the mean age of 42. Among the identifiable pathogens (71.1% culture positive), the causative agents were predominantly gram-negative bacteria (48.1%). The most common specie was Klebsiella pneumoniae (25.9%). About a quarter of the patients required surgical eye removal, and the remaining 45.7% had visual acuity (VA) worse than hand motion at one month after the infectious episode. The most common complication was ocular hypertension (52.5%). Poor initial VA was significantly associated with a worse visual outcome in the early post-treatment period (p 0.12, adjusted OR 10.20, 95% CI 1.65–62.96). Five patients continued to visit the clinic for at least ten years. One patient had gained his vision from hand motion to 6/7.5. Two patients had visual deterioration, one from corneal decompensation, and the other from chronic retinal re-detachment. Two patients developed phthisis bulbi, with either some VA perception of light or no light perception. Poor initial VA is the only prognostic factor of a poor early post-treatment visual outcome of EE.

Uveal effusion is a rare disease that is characterized by exudative detachment of the ciliary body and choroid. Herein, we report a rare case of uveal effusion associated with viral encephalitis, which resolved following the treatment of the viral encephalitis and administration of corticosteroids.A 67-year-old man who was hospitalized for viral encephalitis was referred to our clinic. He had been treated for herpes zoster ophthalmicus in his left eye 3 weeks previously. Choroidal detachment and uveal effusion between the ciliary body and sclera were observed. He was prescribed oral and topical steroids and cycloplegics to treat uveal effusion, and an antiviral agent (Acyclovir) to treat viral encephalitis. After 4 weeks, the choroidal detachment resolved completely.Uveal effusion syndrome can develop in association with viral encephalitis and be treated successfully with oral and topical steroids; we suggest that medical treatment should be attempted prior to surgery.ADEM: Acute disseminated encephalomyelitis; BCVA: Best corrected visual acuity; CSF: Cerebrospinal fluid; CT: Computed tomography; MRI: Magnetic resonance imaging; WBC: White blood cell.

The aim of this review was to identify the imaging methods at our disposal to optimally manage posterior uveitis at the present time. The focus was put on methods that have become available since the 1990s, some 30 years after fluorescein angiography had revolutionised imaging of posterior uveitis in particular imaging of the retinal vascular structures in the 1960s. We have focussed our review on precise imaging methods that have been standardised and validated and can be used universally thanks to commercially produced and available instruments for the diagnosis and follow-up of posterior uveitis. The second part of this imaging review will deal with invasive imaging methods and in particular ocular angiography.摘要: 本文旨在确认当前可行的影像学方法, 以优化后葡萄膜炎的治疗。聚焦于自20世纪90年代以来可行的方法, 30年前荧光素血管造影术对后葡萄膜炎的成像进行了变革, 尤其是20世纪60年代针对视网膜血管结构的成像。本综述着重阐述已标准化及被验证的精确的成像方法, 商业化生产它们得以普遍使用, 且可用于后葡萄膜炎的诊断及随访。本文的第二部分将讨论侵入性影像学方法, 尤其是眼血管造影术。.

This study reports the use of cell-type-specific in vivo bioluminescence to measure intraocular immune cell population dynamics during the course of inflammation in a mouse model of uveitis. Transgenic lines expressing luciferase in inflammatory cell subsets (myeloid cells, T cells, and B cells) were generated and ocular bioluminescence was measured serially for 35 days following uveitis induction. Ocular leukocyte populations were identified using flow cytometry and compared to the ocular bioluminescence profile. Acute inflammation is neutrophilic (75% of ocular CD45 + cells) which is reflected by a significant increase in ocular bioluminescence in one myeloid reporter line on day 2. By day 7, the ocular T cell population increases to 50% of CD45 + cells, leading to a significant increase in ocular bioluminescence in the T cell reporter line. While initially negligible ( 4% by day 35. This change is reflected by a significant increase in the ocular bioluminescence of the B cell reporter line starting on day 28. Our data demonstrates that cell-type-specific in vivo bioluminescence accurately detects changes in multiple intraocular immune cell populations over time in experimental uveitis. This assay could also be useful in other inflammatory disease models.

This is a retrospective study in consecutive cases with cultured-proven endogenous endophthalmitis (EE) treated at the largest tertiary medical center in middle Taiwan in the past 10 years. 83 eyes of 70 patients were enrolled. The mean interval between systemic diseases to the diagnosis of EE was 8.84 ± 6.94 days. The mean initial visual acuity (VA) in the logarithm of minimal angle of resolution (logMAR) was 1.63 ± 0.87. Type 2 diabetes mellitus was the most common predisposing medical illness (N = 53, 63.86%). The most common infectious sources were intra-abdominal abscess (N = 36, 43.37%), and the second most reason was urinary tract infection. The causative pathogen was Gram-negative predominant (N = 64, 77.11%). After aggressive treatment, 34.94% of eyes regain useful vision, and only six eyes underwent enucleation or evisceration. The binary multivariate logistic regression model revealed that female gender (95% CI 1.002–19.036, p = 0.05, OR 4.37), initial VA logMAR (95% CI 0.089–0.550, p = 0.01, OR 0.22), and more intravitreal injections (95% CI 0.368–0.927, p = 0.023, OR 0.58) were independent risk factors influencing final outcomes. Based on the results mentioned above, early diagnosis is recommended to gain better outcomes. The mean interval between systemic diseases to the diagnosis of EE was 8.84 ± 6.94 days in our sample population, clinicians should maintain a higher index of suspicion during this period when encountering patients with bacteremia or fungemia.

Purpose: The purpose of this study was to report 2 cases experiencing a transient decrease in their vision due to the development of acute myopia after an uneventful prolene gonioscopy-assisted transluminal trabeculotomy (GATT) surgery. Methods: A 52-year-old woman with primary open-angle glaucoma and an 8-year-old boy with juvenile glaucoma underwent uneventful GATT surgery. Results: On the postoperative first day, both patients had mild shallow anterior chamber and intraocular pressures measured as 19 and 16 mm Hg, respectively. Both patients had myopia measured as -4.5 and -6.0 D, respectively. Fundus examination was unremarkable in the first patient, whereas it showed bilateral optic disc cupping and retinal nerve fiber layer atrophy in the second patient. In the first patient, supraciliary effusion was detected by using ultrasound biomicroscopy. Ultrasound biomicroscopy could not be performed in the second patient, but ocular ultrasonography revealed a thin layer of suprachoroidal fluid. Myopia was completely regressed, and visual acuity increased within 1 week of the follow-up in both patients. At the sixth month of the follow-up, intraocular pressures in both patients were under control without any antiglaucoma medications. Conclusion: Transient decrease in vision after the GATT surgery might occur secondary to supraciliary effusion leading to acute, transient myopia.

Background Prognostic factors for TB-associated uveitis (TBU) remain mostly unknown, due to the challenges in interpretation of patient data. We present consensus list of prognostic factors for resolution of inflammation in TBU, by experts across the Asia-Pacific region. Methods We applied a modified Delphi technique to generate consensus on prognostic factors influencing the resolution of inflammation in TB-associated: anterior uveitis (AU), retinal vasculitis (RV), and multifocal serpiginoid choroiditis (MSC). The initial questionnaire was developed through a face-to-face meeting. Sixteen uveitis experts from eleven Asia-Pacific countries were included. A single investigator circulated the questionnaire electronically and received the responses. Participants scored each item on 4-point Likert scale, in three successive rounds. After each round, a number of items were reduced based on response, and summary of responses was provided to participants. At the end of Round 3, items were considered significant if they: (1) achieved a median ≥2, and interquartile range ≤1, and (2) ≥75% of the respondents agreed on whether the item was a positive or negative prognostic factor. Results Forty-two putative questionnaire items were considered. At the end of Rounds 3, 4, 9, and 8 items in AU, RV, and MSC, respectively, met significance criteria. These included duration of disease, previous corticosteroid/immunosuppressive therapy, co-existent HIV, disease-specific imaging features, multidrug resistant TB, and duration of anti-TB therapy. Conclusions Consensus were achieved on multiple ocular and systemic factors that influence resolution of inflammation in TBU. These will form the groundwork for validation in prospective clinical trials.

Objectives To assess the incidence of ocular manifestations of Kawasaki disease (KD) in children and to evaluate the relationship between ocular manifestations and the other clinical manifestations, laboratory findings, and echocardiographic findings. Methods Complete ophthalmologic examination and echocardiography were performed in 36 patients with KD during the acute phase before starting the treatment. Clinical manifestations and laboratory data including white blood cell (WBC) count, neutrophil-to-lymphocyte ratio, platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST) were obtained from all the patients. All the clinical and laboratory findings were compared between the group with ocular involvement and the one without ocular involvement. Results The incidence of bilateral non-exudative conjunctivitis was 63.9%. It was significantly higher in patients with skin rashes (P 0.05). Conclusion In children with Kawasaki disease, uveitis is associated with coronary artery dilatation, higher neutrophil count, and higher CRP level.

Non-infectious uveitis (NIU) is an intractable, recurrent, and painful disease that is a common cause of vision loss. Available treatments of NIU, such as the use of topical corticosteroids, are non-specific and have serious side effects which limits them to short-term use; however, NIU requires long-term treatment to prevent vision loss. Therefore, a single dose therapeutic that mediates long-term immunosuppression with minimal side effects is desirable. In order to develop an effective long-term therapy for NIU, an adeno-associated virus (AAV) gene therapy approach was used to exploit a natural immune tolerance mechanism induced by the human leukocyte antigen G (HLA-G). To mimic the prevention of NIU, naïve Lewis rats received a single intravitreal injection of AAV particles harboring codon-optimized cDNAs encoding HLA-G1 and HLA-G5 isoforms one week prior to the induction of experimental autoimmune uveitis (EAU). AAV-mediated expression of the HLA-G-1 and -5 transgenes in the targeted ocular tissues following a single intravitreal injection of AAV-HLA-G1/5 significantly decreased clinical and histopathological inflammation scores compared to untreated EAU eyes (p

Phakic intraocular lenses are used to correct refractive errors. The procedure is predictable and potentially reversible. The procedure is not free of complications though. The occurrence of iris cyst after implantation of a phakic intraocular lens was not described previously in the literature. We describe two cases of iris cysts in the presence of a phakic intraocular lens; the first case describes a cyst which was not present prior to the anterior chamber phakic intraocular lens implantation. The second case describes a hidden iris cyst that affected the posterior chamber phakic intraocular lens position and lead to glaucoma.

The authors report a case of bilateral diffuse uveal melanocytic proliferation associated with transitional cell carcinoma of the bladder. A novel imaging finding on indocyanine green angiography of a “string of sausages” pattern in the large choroidal vessels is described. This occurs in areas of alternating retinal pigment epithelial hypertrophy and destruction, which is likely to be its cause. To the authors' knowledge, it has not been previously described. [ Ophthalmic Surg Lasers Imaging Retina . 2019;50:525–528.]

We report the case of congenital ectropion uveae in a 10-year-old boy with intractable unilateral glaucoma but no systemic association. Glaucoma in congenital ectropion uveae is often poorly responsive to medial therapy and requires surgical intervention. Satisfactory results are possible if diagnosis is established early and timely surgery performed, as was the case in our patient.

This study aimed to clarify the association between the retinal leakage site on fluorescein angiography (FA) and subfoveal choroidal thickness (SCT) measured using enhanced depth imaging optical coherence tomography (EDI-OCT). Twenty-two patients with Behçet’s uveitis were retrospectively selected in this study. They underwent EDI-OCT and FA in both the active and convalescent phases. The associations of the changes between the active and convalescent phases in SCT and in FA leakage in various retinal areas (total retina, peripheral retina, macula, and optic disc) were examined. The changing rates of SCT between the two investigated phases were significantly associated with the changes in total FA leakage scores (y = 1.79X+ 11.7, r2 = 0.210, p 2 = 0.219, p

Uveal melanoma (UM) is the most common primary intraocular malignancy in adults and leads to deadly metastases for which there is no approved treatment. Genetic events driving early tumor development are well-described, but those occurring later during metastatic progression remain poorly understood. We performed multiregional genomic sequencing on 22 tumors collected from two patients with widely metastatic UM who underwent rapid autopsy. We observed multiple seeding events from the primary tumors, metastasis-to-metastasis seeding, polyclonal seeding, and late driver variants in ATM, KRAS, and other genes previously unreported in UM. These findings reveal previously unrecognized temporal and anatomic complexity in the genetic evolution of metastatic uveal melanoma, and they highlight the distinction between early and late phases of UM genetic evolution with implications for novel therapeutic approaches.

To study the correlation of OCT parameters including central subfield macular thickness (CSMT), peripapillary retinal thickness (PRT), and peripapillary retinal nerve fiber layer thickness (PNFLT) with fluorescein angiography (FA) in evaluation of inflammatory activity in Behcet’s retinal vasculitis. In this case-series, concurrent FA and OCT were performed. A scoring system was devised for FA. PNFLT in 3.4-mm-diameter circle as well as PRT in doughnut shaped regions between the 1-mm- and 2.2-mm-diameter and between the 2.2-mm- and 3.45-mm-diameter circles was measured. The correlation of FA and OCT parameters was analyzed. A total of 105 sets of FA from 28 eyes (15 patients) were reviewed. Four (26.6%) were female and mean age was 31.6 ± 8.49 years. Each micron increase in CSMT, PRT2.2, PRT3.45, and PNFLT, caused a rise of 0.018 (95% CI 0.008–0.027, P

Acute retinal necrosis (ARN) is a form of infectious uveitis caused by alpha herpesviruses, including herpes simplex virus type 1 (HSV-1). We previously found that the long non-coding RNA (lncRNA) U90926 is upregulated in murine retinal photoreceptor cells following HSV-1 infection, leading to host cell death. However, to date, an orthologous transcript has not been identified in humans. We investigated U90926 orthologous transcript in humans and examined its utility as a prognostic marker for visual acuity in patients with ARN. We identified two human orthologous transcripts (1955 and 592 bases) of lncRNA U90926. The amount of the longer human U90926 transcript was approximately 30- and 40-fold higher in the vitreous fluid of patients with ARN than in those with sarcoidosis and intraocular lymphoma, respectively. Furthermore, the expression of the longer human U90926 transcript in the vitreous fluid was highly correlated with the final best-corrected logarithm of the minimum angle of resolution visual acuity in patients with ARN (r = 0.7671, p = 0.0079). This suggests higher expression of the longer human U90926 transcript in the vitreous fluid results in worse visual prognosis; therefore, expression of the longer human U90926 transcript is a potential negative prognostic marker for visual acuity in patients with ARN.

To identify triggering factors for the next inflammatory episode of recurrent acute anterior uveitis (RAAU), a 1:1 case–control study was conducted. We interviewed RAAU patients with recent acute anterior uveitis attack and quiescent controls for their information during a previous month using Srithanya Stress Test (ST-5) and questionnaires about potential triggering factors. Asymptomatic controls were matched for age (± 5 years), sex, and HLA-B27. There were 39 pairs of cases and controls. Patients who recently experienced a uveitis attack demonstrated higher mean ST-5 scores (3.7 ± 2.9 vs 0.7 ± 1.1) and shorter sleep time (6.3 ± 1.4 vs 7.4 ± 0.7 h per day) compared with their controls. In the multivariate conditional logistic regression analysis, ST-5 score ≥ 3 (OR 9.07, 95% CI 1.14–72.16, p = 0.037) and sleep time

The development of a retinal vein occlusion (RVO) is multifactorial. This study investigates pachychoroid as a risk factor for RVO or as an entity sharing common pathophysiology with RVO. A database screening at the University Eye Hospital, Ludwig-Maximilian University Munich, Germany was performed for patients diagnosed with central or branch RVO (CRVO/BRVO). In every patient a complete ophthalmologic examination was performed, including posterior segment enhanced depth spectral domain optical coherence tomography (EDI-SD-OCT). The SD-OCT scans of respective partner eyes without history of RVO were compared to an age- and refraction-matched, randomly recruited normal control group. In total, 312 eyes of 312 patients were included in this study, with 162 eyes in the RVO and 150 eyes in the control group. A significantly higher subfoveal choroidal thickness (SFCT) was found in the RVO (310.3 ± 72.5 (94 to 583) µm) as compared to the control group (237.0 ± 99.0 (62 to 498); p

Purpose: To determine the incidence and demographic profile of uveal effusion syndrome (UES), and to describe the visual and anatomic outcome following deep sclerectomy or vortex vein decompression. Methods: The British Ophthalmological Surveillance Unit (BOSU) mails reporting cards monthly to 1149 senior UK ophthalmologists, who are requested to report incident cases of specified rare diseases. UES was included in the reporting system from October 2009 to October 2011. If UES was identified, ophthalmologists were mailed a questionnaire to collect anonymized clinical data at baseline, and 12 months after. Results: Over 2 years, 29 cases were reported. Two cases were duplicates and 12 failed to meet the eligibility criteria. Of the 15 eligible cases, age ranged from 11 to 91 years (mean 62) and nine were males (60%). Ten patients were hypermetropic; three had an axial length of 19.0 mm or less. Estimated annual incidence was 1.2 per 10 million population. Seven cases were managed nonsurgically, including observation (one case), topical steroids (two cases), systemic steroids (three cases), and cyclodiode laser (one case). Eight cases (11 eyes) underwent full-thickness sclerectomy; the elevated flap was retained in four. The sclera was noted to be thick and rigid during surgery in five cases. Median preoperative visual acuity was 6/18, changing slightly to 6/21.5 at final review, with three eyes showing complete anatomic response, five showing some improvement, and three failing to respond. Conclusions: UES is extremely rare. It occurs in a range of ages, but is most common in middle-aged, hypermetropic men. Visual acuity can be materially reduced. The most commonly used surgical treatment in the UK is deep sclerectomy.

Optical coherence tomography (OCT) is a noninvasive method that can quickly and accurately examine the eye at the cellular level. Several studies have used OCT for analysis of anterior chamber cells. However, these studies have several limitations. This study was performed to supplement existing reports of automated analysis of anterior chamber cell images using spectral domain OCT (SD-OCT) and to compare this method with the Standardization of Uveitis Nomenclature (SUN) grading system. We analyzed 2398 anterior segment SD-OCT images from 34 patients using code written in Python. Cell density, size, and eccentricity were measured automatically. Increases in SUN grade were associated with significant cell density increases at all stages (p

Infliximab (IFX) was the first biologic introduced for refractory uveitis treatment in Behçet’s syndrome (BS). However, there have been few reports on the safety and efficacy of IFX monotherapy over follow-up periods of more than 10 years. This retrospective study evaluated the 10-year safety and efficacy of IFX monotherapy compared to IFX combination therapies with colchicine or corticosteroid for refractory uveitis in BS patients. Monotherapy was performed in 30 eyes of 16 patients while combination therapies were performed in 20 eyes of 11 patients. Continuation of IFX occurred in 70.3% of enrolled patients for 10 years without any significant difference noted in the retention rate between the monotherapy and combination therapies (p = 0.86). Reduction of ocular inflammatory attacks and improvement of best corrected visual acuity occurred in the monotherapy group after 10 years, which was equivalent to that for the combination therapies. Although adverse events (AEs) or therapy discontinuation occurred during the initial 5 years in both therapies, no AEs were observed for either therapy after 6 years. Our results suggested that IFX monotherapy proved to be effective and not inferior to combination therapies over a 10-year follow-up. Although loss of response and AEs may be noticed during the initial 5-year period, a safe and effective continuation can be expected thereafter.

BackgroundTo report undescribed characteristics of patients with bilateral diffuse uveal melanocytic proliferation (BDUMP) on ultrasound biomicroscopy (UBM) and high-frequency B-scan ultrasonography.Case presentationTwo of four participants presented with worsening bilateral vision after previously diagnosed primary pulmonary or ovarian carcinoma. The other two patients were diagnosed with lung carcinoma after presentation with BDUMP. All patients had ciliary body nevi-like lesion in combination with iris or ciliary body cysts, and uveal thickening on UBM. Focally elevated choroidal nevi-like lesion and exudative retinal detachment with choroidal thickening were detected with B-scan ultrasonography.ConclusionsOur case series demonstrates the uveal characteristics of patients with BDUMP based on high-frequency B-scan ultrasonography and UBM. Ultrasonographic findings are crucial in the diagnosis of BDUMP because it is occult in nature.

Aim of the study is to report the clinical characteristics and prognostic factors in hypertensive anterior uveitis (AU) diagnosed with multiplex polymerase chain reaction (PCR). Eighty-eight eyes of 88 patients with hypertensive AU were enrolled from 2015 to 2019 in a tertiary center in South Korea. All patients underwent multiplex PCR that was performed using aqueous humor samples collected at first visit to detect the DNA of six herpesviruses. Twenty-eight (31.8%) eyes were PCR positive. Herpes simplex virus was found in 6 (6.8%) eyes, varicella-zoster virus in 7 (8.0%) eyes, cytomegalovirus in 14 (15.9%) eyes, and Epstein–Barr virus in 1 (1.1%) eye. On multivariate regression analysis, PCR positivity was significantly associated with coin-shaped keratic precipitates (odds ratio (OR) = 6.01, P = 0.044). Recurrence and final visual acuity were significantly associated with a presumed diagnosis of viral endotheliitis (OR = 21.69, P = 0.04 and OR = 6.3, P = 0.004, respectively). This study showed the importance of PCR positivity, suggesting that identification of the virus through active PCR testing could affect the course, treatment, and prognosis of hypertensive AU.

Background To report an unusual case of non-nanophthalmic uveal effusion syndrome (UES) with histologically normal sclera but responsive to scleral resection. Case presentation A73-year-old man presented with a bullous retinal detachment without ciliochoroidal detachment on funduscopic examination of the right eye. The axial length of both eyes was normal. Extensive investigations for possible causes of exudative retinal detachment were performed with unremarkable results except for choroidal hyperpermeability on indocyanine green angiography (ICGA). Ultrasound biomicroscopy (UBM) revealed scleral thickening with peripheral choroidal elevation leading to the diagnosis of UES. Partial thickness sclerectomy and sclerotomy was performed resulting in complete retinal reattachment, reduction of choroidal hyperpermeability on ICGA and improvement of visual acuity. However, histological studies of the excised sclera revealed no scleral architectural changes or abnormal deposits. Conclusions The diagnosis of UES in non-nanophthalmic eyes is challenging. Thorough systemic and ocular investigations are critical to rule out other etiologies. UBM can be helpful to evaluate scleral thickness and anterior choroid in equivocal cases. Our case was unique in that, although the sclera was thick, no abnormal microscopic scleral architecture could be identified. Misdiagnosis may lead to different surgical procedures such as vitrectomy resulting in unfavorable outcomes.

Purpose Outcomes and reoperation rates in infants with unilateral persistent fetal vasculature (PFV) with elongated or stretched ciliary processes have not been extensively studied and were excluded from the Infant Aphakia Treatment Study (IATS). The purpose of this study is to analyze the preoperative measurements, reoperation rates, and complications after surgery in unilateral anterior PFV with stretched processes. Design Retrospective case series. Methods Inclusion criteria consisted of unilateral cataract surgery prior to 7 months of age and anterior PFV with elongated ciliary processes. Eyes with posterior retinal involvement and less than 6 months of follow-up were excluded. All patients underwent lensectomy, posterior capsulectomy, and vitrectomy. Results Eleven eyes of 11 patients were included. Patients had a mean age at surgery of 2.4 ± 1.4 months. Average follow-up was 4.5 ± 3.7years. Globe axial lengths were 18.6 ± 1.9 mm. Ten patients (91%) were initially left aphakic. Three patients (27%) later received a secondary intraocular lens (IOL), and 1 patient underwent an IOL exchange. Six out of 10 (60%) aphakic patients developed visual axis opacification. One aphakic patient required topical therapy for glaucoma. One additional patient developed neovascular glaucoma and retinal detachment. The eye was subsequently enucleated. Three patients underwent strabismus surgery. Two patients underwent pupilloplasty. Two patients (18.2%) had a final visual acuity better than 20/200. Conclusion Eyes operated for PFV with elongated ciliary processes are unlikely to have a final visual acuity greater than 20/200 and many will need additional surgery. Postoperative visual axis opacification occurred in 60% and glaucoma developed in 18%.

Purpose To report two cases of idiopathic intraocular cilia presenting as sectoral scleritis with progressive intraocular inflammation. Methods Both patients were treated with intravitreal antibiotics and underwent pars plana vitrectomy where the cilia were removed and identified on histopathology. Results One patient developed a retinal detachment while being treated for presumed endophthalmitis. The intraocular cilium was discovered during pars plana vitrectomy. In the second case, the cilium was detected on dilated fundus exam and was believed to be the cause of the patient's scleritis and vitritis. Therapeutic vitrectomy was performed. In both cases, the cilia were positively identified on histopathology. Conclusion Idiopathic intraocular penetration of cilia should be considered in the differential diagnosis of sectoral scleritis with progressive intraocular inflammation.

We report a case of trifocal choroidal melanoma (three separate tumors) in a 48-year-old Caucasian female who had been followed for oculodermal melanocytosis since childhood. At presentation, no tumor was present and annual examination was advised. Seventeen years later, three choroidal melanocytic lesions were detected in the right eye. Growth of each was documented, enucleation was performed, and histopathology revealed three independent choroidal melanomas. The patient developed extensive liver and bone metastases and subsequently died. Oculodermal melanocytosis is a risk factor for the development of uveal melanoma and a potential marker for worse prognosis. Careful long-term follow-up is required.

PURPOSE: To evaluate the relationship between central corneal thickness (CCT) and horizontal corneal diameter (HCD) in eyes with uveal coloboma and microcornea. PATIENTS AND METHODS: All adult patients with uveal coloboma having microcornea in at least one eye and seen between May 2014 and July 2016 at the cataract clinic of our tertiary eye care center were evaluated. The study group comprised 26 colobomatous eyes of 15 patients who had associated microcornea. The control group comprised eyes with uveal coloboma and normal-sized cornea (n=12 eyes of 9 patients). The main outcome measure included the relationship between CCT and HCD in eyes with uveal coloboma and microcornea. RESULTS: The mean HCD of the study and control groups was 6.97±1.28 and 11.39±0.67 mm, respectively. The mean central pachymetry of the study group was 668.4±40.2 μm compared with 555.3±41.8 μm in the control group. In both the groups, CCT was negatively correlated with HCD (R=−0.62 in the study group and R=−0.72 in the control group). CONCLUSIONS: Uveal colobomatous eyes with microcornea have thicker corneas compared with uveal colobomatous eyes with normal diameter corneas that have normal central corneal thickness. In all the eyes with uveal coloboma, there is a negative correlation between horizontal corneal diameter and central corneal thickness.

Congenital ectropion uveae (CEU) is a rare anomaly characterized by ectropion uveae, iris hypoplasia, iridotrabecular dysgenesis and glaucoma. The apparent ectropion uveae results from the spread of iris pigment epithelium beyond the iris ruff and onto the anterior surface of the iris. Conclusion Open-angle glaucoma results due to angle dysgenesis, so patients should be carefully examined periodically for its early detection.

Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic intraocular disease that causes progressive visual loss in patients driven by an IgG factor associated with an underlying malignancy. Characteristic ocular findings include exudative retinal detachment, rapid cataract formation and uveal melanocytic tumours. The awareness and documentation of BDUMP has increased during the past decade, and the increasing amount of data collected demonstrates the effect of treatment with plasmapheresis and the value of diagnostic tools in BDUMP such as genetic and immunologic investigations. The literature of BDUMP has not been reviewed since 2003, and there is a growing need for an updated review on diagnosis and management of BDUMP. We review the literature and report a case of BDUMP with a white ciliary body tumour, iris rubeosis, increased iris pigmentation and cataract.

We aimed to present a novel case of sectoral ciliary body agenesis and complicated cataract as an embryogenic defect of eye development diagnosed by ultrasound biomicroscopy. A 20-year-old male patient presented with a complaint of visual impairment in his left eye since childhood. Slit-lamp examination of the left eye revealed pigment precipitation and focal lens opacities extending from the temporal quadrant through the posterior lens capsule, blocking the central optical axis. On ultrasound biomicroscopy examination, there was a hyperechoic reflection belonging to the rudimentary ciliary body structures between 2-5 o’clock in the temporal quadrant. The zonules could not be visualized in the same location. At all other quadrants of the anterior chamber angle, the ciliary body and zonules were normal. This is a very rare case of sectoral ciliary body agenesis complicated by cataract. Ultrasound biomicroscopy may be useful for detecting rare congenital anomalies of the anterior segment, anterior chamber angle, and ciliary body.

To examine whether autotaxin (ATX) in the aqueous humor causes elevated intraocular pressure (IOP) in patients with Posner-Schlossman syndrome (PSS). ATX and transforming growth factor beta (TGF-β) in the aqueous humor were quantified in PSS patients. The expression of ATX and TGF-β in cytomegalovirus (CMV)-infected-human trabecular meshwork (hTM) cells was examined. Biological changes in hTM cells and monkey Schlemm’s canal endothelial (SCE) cells cultured in the conditioned medium of CMV-infected hTM cells were analyzed. The expression of ATX and TGF-β1 was upregulated in the aqueous humor of CMV-positive PSS patients, and the level of ATX in the aqueous humor was positively correlated with IOP. CMV infection upregulated ATX and TGF-β1 in hTM cells. The conditioned medium induced fibrotic changes in hTM cells and reduced SCE permeability, which was attenuated by an ATX inhibitor, a lysophosphatidic acid receptor antagonist, and a Rho kinase inhibitor. ATX in the aqueous humor induced by CMV infection may trigger elevated IOP. Modulating ATX activity may be a novel treatment modality for PSS.

Introduction Ocular tuberculosis (TB) is an extrapulmonary manifestation of mycobacterium infection that most commonly presents as uveitis. This is the first prospective incidence study of presumed ocular tuberculosis performed in the United Kingdom (UK). Method New cases of ocular tuberculosis presenting to hospitals in the UK were prospectively ascertained between October 2016 and November 2017 with the aid of the British Ophthalmological Surveillance Unit (BOSU). Initial presentation data and 1-year follow-up data was collected using questionnaires. Results Forty-eight patients were recruited giving an overall incidence for ocular TB of 0.73 per million population per annum. The origin of birth for 71% of the patients was a non-UK country and 87.5% had their initial diagnosis of TB made by an ophthalmologist. The most common first line treatment was isoniazid, rifampicin, ethambutol and pyrazinamide which 71% of patients were treated with 60% of patients were commenced on a reducing course of oral steroids. At 1-year follow-up, 29 patients (83%) had complete resolution of active clinical signs. Mean best corrected visual acuity (BCVA) at presentation was +0.41 LogMAR(SD = 0.62), compared to +0.31 LogMAR (SD = 0.56) at 12-month follow-up. Discussion It is increasingly the responsibility of the ophthalmologist to diagnose ocular TB and although it remains a rare condition, consensus on diagnostic criteria and treatment is required. Increasing recognition and accessibility to gamma-interferon testing should enable earlier detection. Treatment with quadruple ATT treatment regimens for at least 6 months shows good clinical outcomes. However, it is still unclear whether steroid use is beneficial. Further large studies with longer follow-up would be warranted to answer these questions.

Background To report a case of non-prescription cold and flu medication-induced transient myopia with uveal effusion. Case presentation Bilateral high intraocular pressure, shallow anterior chambers, uveal effusion, and a myopic shift were encountered in a 39-year-old Chinese male 1 night after taking a non-prescription flu medicine three times than the recommended dose. Ultrasound biomicroscopy (UBM) showed bilateral ciliochoroidal effusions, disappearance of the ciliary sulcus, closure of the angle of the anterior chamber, and anterior displacement of the lens-iris diaphragm. Treatment with aqueous suppressants was given. Within a week, the uncorrected vision restored, and the myopia had disappeared. UBM revealed major resolution of the ciliochoroidal effusions in both eyes, deepening of the anterior chamber, return of the lens-iris diaphragm to a more posterior position. Conclusions Overdose of non-prescription cold and flu medication may cause bilateral uveal effusions inducing acute angle-closure glaucoma and acute myopia.

This study aimed to characterise the distribution of choroidal thickness (CT) in diabetic retinopathy eyes, inside and outside of the vascular arcade, as well as at the fovea, using spectral-domain optical coherence tomography (OCT). Forty-nine healthy eyes, 80 diabetic retinopathy (DR) eyes (59 non-proliferative diabetic retinopathy (NPDR) eyes and 21 proliferative diabetic retinopathy (PDR) eyes) were examined with OCT to obtain nine horizontal lines (far superotemporal, near superotemporal, central, near inferotemporal, far inferotemporal, far superonasal, near superonasal, near inferonasal, far inferonasal) inside and outside of the vascular arcade. Nine points were chosen in 0.5-mm intervals to calculate CT, which was measured at 81 points in each patient. In the DR group, CT decreased significantly, compared with the control group, in all nine horizontal lines except central and near inferotemporal (−29.74 to −36.97 μm, p

Purpose To investigate the association between serum amyloid A (SAA) protein and the clinical features of acute anterior uveitis (AAU), and to evaluate the disease activity and treatment effect in relation to SAA levels. Methods AAU patients and healthy individuals were recruited from October 2016 to August 2017 at the Department of Uveitis, in the Eye Hospital of Wenzhou Medical University. Related demographic, clinical characteristics, and therapeutic data were analyzed. Results One hundred and eight AAU patients and 18 healthy controls were included in this study. Serum SAA levels in AAU patients were significantly higher than those of healthy controls (p all

Purpose Patients with nanophthalmos who undergo intraocular surgery often present with abnormal ciliary zonules. In a previous study, we reported mutation in MYRF that is implicated in the pathogenesis of nanophthalmos. The aim of this study was to model the mutation in mice to explore the role of MYRF on zonule structure and its major molecular composition, including FBN1 and FBN2. Methods Human MYRF nanophthalmos frameshift mutation was generated in mouse using the CRISPR-Cas9 system. PCR and Sanger sequencing were used for genotype analysis of the mice model. Anterior chamber depth (ACD) was measured using hematoxylin and eosin-stained histology samples. Morphologic analysis of ciliary zonules was carried out using silver staining and immunofluorescence. Transcript and protein expression levels of MYRF, FBN1, and FBN2 in ciliary bodies were quantified using quantitative real-time PCR (qRT-PCR) and Western blot. Results A nanophthalmos frameshift mutation (c.789delC, p.N264fs) of MYRF in mice showed ocular phenotypes similar to those reported in patients with nanophthalmos. ACD was reduced in MYRF mutant mice (MYRFmut/+) compared with that in littermate control mice (MYRF+/+). In addition, the morphology of ciliary zonules showed reduced zonular fiber density and detectable structural dehiscence of zonular fibers. Furthermore, qRT-PCR analysis and Western blot showed a significant decrease in mRNA expression levels of MYRF, FBN1, and FBN2 in MYRFmut/+ mice. Conclusions Changes in the structure and major molecular composition of ciliary zonules accompanied with shallowing anterior chamber were detected in MYRFmut/+ mice. Therefore, MYRF mutant mice strain is a useful model for exploring pathogenesis of zonulopathy, which is almost elusive for basic researches due to lack of appropriate animal models.

BACKGROUND/PURPOSE To report a new technique for treating patients with uveal effusion syndrome by the fiberoptic-guided CO2 laser. METHODS Interventional case report. A 74-year-old man presented with exudative detachment of the choroid secondary to uveal effusion syndrome. Partial-thickness sclerotomy and full-thickness sclerotomy were performed to treat the disease using a fiberoptic-guided CO2 laser. RESULTS After the surgery, the patient's visual acuity improved and choroidal folds disappeared. CONCLUSION This technique allows concomitant coagulation and cutting, thereby reducing the risk of bleeding and providing better depth control.

PURPOSE To report an unusual case of delayed-onset bilateral diffuse uveal melanocytic proliferation in a patient with a remote history of gastric adenocarcinoma 17 years earlier. METHODS Case report of a patient with bilateral diffuse uveal melanocytic proliferation including comprehensive systemic and ocular examinations. RESULTS A 78-year-old man presented with a history of progressive bilateral vision loss during the 4 previous years associated with fever of unknown origin. He underwent total gastrectomy 17 years earlier as a treatment for gastric adenocarcinoma. Funduscopic examination revealed multiple subretinal pigmented and nonpigmented lesions involving the posterior pole of both eyes. These lesions showed early hyperfluorescence on fluorescein angiography, producing a giraffe pattern. Spectral-domain optical coherence tomography showed intraretinal and subretinal fluid with multiple hyperreflective mounds involving the retinal pigment epithelium. Treatment with the intravitreal anti-vascular endothelial growth factor agent, ranibizumab, produced anatomical improvement in both eyes but visual improvement in just the right eye. CONCLUSION Although delayed-onset bilateral diffuse uveal melanocytic proliferation may occur, it is important to rule out a second malignancy. To the knowledge of the authors, this is the first report of delayed-onset bilateral diffuse uveal melanocytic proliferation associated with gastric adenocarcinoma. Treatment with intravitreal anti-vascular endothelial growth factor therapy warrants further evaluation.

PURPOSE To present the experience of a tertiary care ophthalmic institution in the assessment and management of uncommon causes of uveal effusion and related complications. MATERIALS AND METHODS A retrospective chart review was conducted of 12 patients diagnosed with uveal effusion and managed at our institution between 1996 and 2012. The presenting features, investigations, management, and outcomes were analyzed for each case. RESULTS The case series encompasses a variety of clinical conditions that cause uveal effusion including inflammatory, hydrostatic, and idiopathic mechanisms. Two thirds of the patients presented with secondary angle closure. Half of the patients had serous retinal detachment. Modern imaging techniques including ultrasound biomicroscopy and high-resolution magnetic resonance imaging were critical in making the diagnosis. Seven of the 12 patients responded to medical treatment and 4 required scleral surgery. Uveal effusions resolved in all patients after treatment. CONCLUSIONS Uveal effusion is a complex and poorly understood clinical entity with significant visual morbidity and is caused by a range of ocular and systemic diseases. Effective management is critically dependent on the underlying cause.

Purpose: To report a case of circumferential neuroepithelial cyst of the ciliary body presenting with pigment dispersion (PD) and ocular hypertension.Case report: 48-year-old female patient presented with a complaint of pain in the left eye. On examination, visual acuity of the left eye was 0.9, and the intraocular pressure was 48 mmHg. Biomicroscopic anterior segment examination of the left eye revealed 4+ pigmented cells in the anterior chamber. Active PD from the pupillary region at 11 o’clock was noticed at the time of the examination. Ultrasound biomicroscopy demonstrated 360o cystic lesions of the ciliary body in the left eye. The patient was diagnosed as neuroepithelial cyst of the ciliary body.Conclusion: Our case is unique as it is the first case of circumferential neuroepithelial ciliary body cyst presenting with acute PD and ocular hypertension.

PURPOSE To evaluate the relationship between endothelial cell density (ECD) and mean corneal diameter (MCD) in eyes with uveal coloboma associated with microcornea. METHODS Corneal endothelial cell imaging was performed using specular microscopy on 22 eyes of 18 subjects diagnosed with uveal coloboma associated with microcornea. The MCD was noted as the average of horizontal and vertical corneal diameters, which were measured using the ruler tool of the slit-lamp biomicroscope and Castroviejo calipers. RESULTS Mean age of the study subjects was 29.7 ± 10.4 years (range, 14-46 years). The MCD was 7.3 ± 1.1 mm (range, 4.9-9 mm). Mean ECD (3436 ± 316.2 cells/mm) of the study subjects was significantly (P < 0.0001) higher than the mean ECD (2761.8 ± 140.9 cells/mm) of age-matched (range, 10-40 years) normal eyes with no ocular pathology. The pleomorphism and polymegathism were comparable between the 2 groups. Regression analysis showed that MCD had a significant negative (R = -0.55; P = 0.02) relationship with ECD. CONCLUSIONS Eyes with congenital uveal coloboma associated with microcornea have increased ECD. Increased ECD may be partly due to a reduced posterior corneal surface area in microcornea.

Rationale: Pregnancy-induced hypertension (PIH) is a major cause of maternal and fetal mortality. Hypertensive choroidopathy is a preliminary sign of vasogenic edema in the choroid, and is associated with PIH. Here, we report a post-natal case of PIH-related chorioretinitis with bilateral severe serous retinal detachment (SRD) resembling uveal effusion syndrome. Patient concerns: A 35-year-old woman was diagnosed with severe PIH at 37 weeks of pregnancy. She underwent an emergency cesarean delivery. Four days after delivery, she perceived a sudden decrease of vision. At presentation, fundus examination demonstrated bullous SRD and multiple white mottles in the posterior poles of both eyes. Optical coherence tomography (OCT) showed macula edema and retinal pigment epithelium (RPE) folds. Indocyanine green angiography (ICGA) demonstrated delayed filling of choroidal circulation in the early phase and multiple hyperfluorescent spots in the mid phase. Diagnoses: PIH. Interventions: Antihypertension treatment alone resulted in gradual resolution of the SRD. Outcomes: At 463 days after delivery, fundus photographs of both eyes showed leopard spots corresponding to hyperautofluorescent spots with dark rim observed on fundus autofluorescence images. Lessons: Ophthalmologists should be aware of PIH-related chorioretinitis with similar clinical manifestations as uveal effusion syndrome, and should treat with antihypertensive agents in cooperation with obstetricians.