Exudative Retinal Detachment Research Articles

Ocular Toxocariasis in Adult Caucasian Patients: Clinical Presentations and Treatment Outcomes

ABSTRACT Purpose To report the clinical features and treatment outcomes in adult Caucasians with ocular toxocariasis (OT) and investigate their prognosis depending on their serological status. Methods Retrospective observational cohort study (2016–2021) including consecutive adults with uveitis and positive western blot (WB) in the aqueous humor or vitreous. The presence of serum antibodies was not necessary for inclusion, allowing to compare the outcomes depending on the serological status. Results Seventeen eyes of 15 patients were included. Mean age at diagnosis was 51.9 years. Vitreous inflammation was the most frequent sign (100%). Vitreoretinal tractions (41.2%) and chorioretinal granulomas (58.8%) were less prevalent. Atypical features were: spontaneous intravitreal hemorrhage (23.5%), exudative retinal detachment (11.8%), isolated macular edema (17.6%), papillitis (29.4%) and vasculitis (47.1%). Twenty percent of patients had a positive serum serology. Baseline clinical features did not differ statistically depending on the serological status; however, the degree of inflammation was numerically higher in patients with negative serology. Overall, macular thickness, anterior and posterior segment inflammation improved significantly after treatment with oral albendazole, systemic ± local corticosteroids. Vitrectomy (47.1%) was performed in case of persistent vitritis (62.5%), retinal detachment (12.5%) and intravitreous hemorrhage (25%). Conclusion OT has no pathognomonic sign and atypical presentations were not infrequent in this adult Caucasian cohort. Serum antibodies were rarely positive, stressing on the importance of ocular sample analysis, especially in case of atypical features. Serum antibodies may prove useful in forecasting the rapidity of inflammation clearance. Antiparasitic and anti-inflammatory treatment was safe and efficient in most cases.

Prognostic value of pretreatment indocyanine green angiography in the acute uveitic phase of Vogt-Koyanagi-Harada disease.

To investigate the prognostic value of pretreatment indocyanine green angiographic (ICGA) features in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease. Retrospective analysis of 84 patients (168 eyes). Main outcome measures were final visual acuity, development of 'sunset glow fundus' (SGF) and progression to chronic recurrent evolution. Thirty-eight patients (76 eyes) presented in the phase preceding anterior segment (AS) inflammation (early presentation) and 46 patients (92 eyes) had AS inflammation at presentation (late presentation). The mean number of hypofluorescent dark dots (HDDs) and frequency of disc hyperfluorescence were more in the late presentation group (p < 0.001 for both comparisons), whereas the early presentation group showed higher frequencies of peripapillary punctate choroidal hyperfluorescence (p < 0.001) and hypofluorescent patches involving macula corresponding to the areas of exudative retinal detachment (p = 0.012). The mean number of HDDs and the frequency of disc hyperfluorescence were higher among eyes that developed SGF (p < 0.001 for both comparisons) and eyes that progressed to chronic recurrent evolution (p < 0.001; p = 0.001, respectively). The frequencies of peripapillary punctate choroidal hyperfluorescence and hypofluorescent patches corresponding to the areas of exudative retinal detachment were less in the eyes that developed SGF (p = 0.019; p = 0.003, respectively). Punctate choroidal hyperfluorescence elsewhere was less frequent in the eyes that developed SGF (p < 0.001) and eyes that progressed to chronic recurrent evolution (p = 0.002). Pretreatment ICGA has a prognostic value in initial-onset acute uveitis associated with VKH disease.

Safety and Efficacy of Photodynamic Therapy in the Treatment of Circumscribed Choroidal Hemangioma: A Systematic Review.

Circumscribed choroidal hemangioma (CCH) is a sort of non-malignant hamartomatous tumor that occurs in the choroidal layer of the eye. It is a rare condition that affects people between their second and fourth decades of life, leading to significant deterioration of vision. One of the most catastrophic consequences of CCH is exudative retinal detachment (ERD), which has a severe impact on vision. This review aims to comprehensively assess the safety and efficacy of photodynamic therapy (PDT) using verteporfin as a therapeutic approach. Using the eligibility criteria, we analyzed the findings of 18 published articles from PubMed, Web of Science, Scopus, and Cochrane. The standard PDT protocol was used in all included studies, except two (one used half-dose, the other one used the double-dose) with an average of 1-2 sessions. PDT induced substantial tumor regression, with a mean thickness range from 0 to 2.3 mm. However, this contrasted with a previous study that reported a thickness of 3.46 mm as an indication of PDT failure. The mean tumor diameter varied from 4.8 mm to total tumor flattening. A suboptimal effect with a mean diameter ranging from 6mm to 8mm was found in two clinical studies. Significant improvement in vision was observed during the last follow-up, ranging from a normalization of Best Corrected Visual Acuity (BCVA) 20/20 to 20/80; counting finger vision persisted in two patients even after treatment. PDT successfully achieved complete subretinal fluid (SRF) resolution in 14 studies and resolved ERD in nine articles. Most studies did not report serious adverse events, but some reported macular atrophy, microcystic degeneration of the retina, transient visual disturbances, Retinal pigmented epithelium (RPE) metaplasia, and cystic degeneration of the retina. This systemic review demonstrated PDT's effectiveness and safety as a first-line management modality for CCH. Photodynamic therapy efficiently induced tumor regression, resulting in a notable reduction in both tumor diameter and thickness, with optimal efficacy to improve vision and resolution of the consequences of CCH, such as SRF and ERD.

Протонотерапия увеальной меланомы (предварительные результаты)

Purpose. To present preliminary results of treatment of uveal melanoma with proton therapy using the scanning pencil beam technique. Material and methods. In this study we included 7 patients (female) with uveal melanoma aged 23 to 51 years. The tumor was localized in the choroid (4), ciliochoroidal (2) and iridociliary (1) zones. All patients received proton therapy in the hypofractionation mode at a total dose of 49 Gy in 7 fractions (BED0,6=620 Gy; EQD2=143 Gy). The follow-up period for patients ranged from 6 to 33 months. Results. In the early post-radiation period, a radiation reaction was observed in the form of skin erythema (2 patients), exudative retinal detachment (3 patients). Late complications were observed as neovascular glaucoma, which developed 6–11 months after the start of treatment in 4 patients, maculopathy (1 patient), dry eye syndrome with madarosis (3 patients). All treated patients had a slow rate of tumor regression, as a rule, the tumor began to regress after 6–8 months from the start of treatment. Complete regression was observed in 2 patients with T3aN0M0 stages of the process for a period of 24 months, the rest showed partial regression of the tumor. Conclusion. The obtained preliminary results of proton therapy using the scanning pencil beam technique are very encouraging in the treatment of large uveal melanomas and open a new perspective in their successful treatment. The use of photodynamic therapy with chlorin drugs should probably be considered as an adjuvant therapy, the effect of which will accelerate the rate of regression and reduce the incidence of complications. Key words: choroidal melanoma, proton therapy, brachytherapy, uveal melanoma, ophthalmo-oncology, choroidal melanoma treatment

Bullous Variant of Central Serous Chorioretinopathy in Goodpasture's Disease - A Case Report and Review of Literature.

To describe a case of bullous variant of central serous chorioretinopathy (CSR) in Goodpasture's disease (GD) compared to an identical twin without GD, and summarize the literature on ocular manifestations of GD. Interventional/observational case report and literature review. A 46-year-old white female with a history of GD presented with decreased vision. She demonstrated bilateral multifocal pigment epithelial detachments and a large inferior exudative retinal detachment in the left eye consistent with bilateral CSR with bullous variant CSR (bvCSR) in the left eye. Despite treatment, her disease remained refractory, with final VA of 20/200 in the left eye. The patient's identical twin sister did not have GD and demonstrated milder CSR on presentation with a more typical, self-limited disease course. Her final VA was 20/20 bilaterally. GD is associated with severe manifestations of CSR (exudative RD). Additional studies focusing on the association between GD and CSR severity may be of interest.

Intravitreal anti-vascular endothelial growth factor (bevacizumab) in nanophthalmic exudative macular detachment – A new treatment modality

Nanophthalmic exudative macular retinal detachment is a known entity, till now the established treatment modality is Scleral Resection. This brief communication suggests a successful new modality of non-surgical, less invasive treatment approach of intravitreal anti-vascular endothelial growth factor in case of exudate macular detachment related to nanophthalmos.

Endogenous endophthalmitis caused by urinary tract infection: A case report.

Endogenous endophthalmitis is a vision-threatening intraocular infection caused by hematogenous spread of infectious organisms from distant sites. A 71-year-old man with a history of fever and dysuria 5 days prior to presentation presented with sudden loss of vision in his left eye. The patient had no history of ocular surgery or trauma, and ocular examination revealed a large amount of exudative plaque covering the pupil. Therefore, fundus examination was not feasible. B-scan ultrasonography revealed a dome-shaped subretinal mass with an exudative retinal detachment. Endogenous endophthalmitis was diagnosed on the basis of these findings. The patient underwent pars plana vitrectomy and the early postoperative course was favorable. Vitreous cultures grew gram-negative bacilli, identified as Klebsiella pneumonia. Urinalysis revealed white blood cells (++) and urinary tract infection was the only identifiable risk factor for endogenous endophthalmitis. Urinary tract infection is an independent risk factor for endogenous endophthalmitis.

Orbital cellulitis with panophthalmitis and scleral necrosis – a case report

BackgroundOrbital cellulitis is common in young children and is often secondary to coexisting sinus disease. Coexisting orbital cellulitis and panophthalmitis is a rare clinical event and usually occurs secondary to trauma or from an endogenous source.Case presentationA febrile 2-year-old male presented with periorbital inflammation and exudative retinal detachment. Imaging showed acute sinusitis and extensive orbital cellulitis. Because of progressive scleral thinning, the patient underwent enucleation.ConclusionWe present a case of concurrent orbital cellulitis, panophthalmitis, and scleral necrosis in an immunocompetent pediatric patient. Timely intervention is important to prevent life threatening complications with the rare occurrence of coexistent orbital cellulitis and panophthalmitis.

INCIDENCE AND RISK FACTORS FOR EXUDATIVE RETINAL DETACHMENT FOLLOWING LASER PHOTOCOAGULATION FOR RETINOPATHY OF PREMATURITY.

Exudative retinal detachment (ERD) may result from laser photocoagulation for retinopathy of prematurity. Although risk factors have been hypothesized from case reports, comparative studies have not been reported. We sought to evaluate risk factors for ERD following laser, comparing affected and unaffected infants. Retrospective cohort study of infants undergoing retinopathy of prematurity laser at the Children's Hospital of Philadelphia over 6 years. All received near-confluent laser of avascular retina. Demographic, medical, and procedural risk factors for ERD were evaluated in univariate analysis because of the rarity of ERD. Among 149 lasered infants, 6 infants (4%, 95% confidence interval [CI] 1.5%-8.6%) developed ERD. Race was a significant risk factor ( P = 0.01). Among 71 African American or Hispanic infants, 6 (8.5%, 95% CI 3.2%-17.5%) developed ERD. Among 78 non-African American or Hispanic infants, 0 (0%, 95% CI 0%-4.6%) developed ERD. There were no significant differences in the other studied factors. Exudative retinal detachment was uncommon (4%) following retinopathy of prematurity laser. Despite so few cases, darker pigmented race with likely increased pigmented fundi was significantly associated with an increased ERD risk. Further study may reveal whether increased choroidal pigment causes greater laser tissue damage or makes it difficult to discern the ora, resulting in inadvertent lasering of the ciliary body, leading to ERD.

Exudative Retinal Detachment as a Prodromal Manifestation of Differentiation Syndrome in Acute Promyelocytic Leukemia: A Case Report and Review of Literature

The ocular manifestations of the differentiation syndrome (DS) are rarely reported in the literature with only six cases published until now. Patients with acute promyelocytic leukemia (APL) who undergo treatment with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) are especially susceptible to developing DS. This is a serious diagnosis with a life-threatening impact on some patients, however, amenable to prophylaxis and treatment by systemic corticosteroids. An exudative retinal detachment (ERD) may either complicate or herald the development of DS. In this article, we present a case of APL who developed ERD in advance of DS symptoms, its multimodal imaging characteristics, and review the published articles concerning this rare disease manifestation.

THE SIGNIFICANCE OF ARGON LASER IN PATIENTS WITH PERIPHERAL RETINAL BREAKS IN PREVENTING RETINAL DETACHMENT

&lt;p&gt;&lt;span&gt;Retinal detachment is a risk to one's vision that occurs about 1 in 10,000 times. Prior to the 1920s, this disease was completely blinding. Restoration of retinal detachments has been much simpler over the past 50 years thanks to procedures like scleral buckling, pneumatic retinopexy, and vitrectomy. Rhegmatogenous, tractional, and exudative retinal detachments are the three types. When subretinal fluid accumulates between the retinal pigment epithelium and the neurosensory retina, retinal detachment occurs. During this phase, three things might occur. Breaking the retina is one way to let vitreous directly enter the subretinal region. This retinal detachment is rhegmatogenous. Rhegmatogenous retinal detachments are frequently brought on by trauma- or posterior vitreous-separated-induced retinal tears. The growth of membranes on the vitreous or retinal surface is a second mechanism.These membranes have the capacity to physically divide the retinal pigment epithelium from the neurosensory retina. A tractional retinal detachment is what this is. Proliferative retinopathy caused by sickle cell anemia, diabetes, or other conditions that neovascularize the retina can cause tractional retinal detachment. Proliferative vitreoretinopathy following trauma or surgery may also contribute to tractional retinal detachments. Fluid exuding from a mass lesion or an accumulation of subretinal fluid caused by inflammatory mediators are the third causes of retinal detachment. Serous or exudative retinal detachment is the term used to describe this mechanism. Sarcoidosis and choroidal neoplasms are two examples of inflammatory or exudative retinal disease processes that can cause serous detachments. Patients with aggressively spreading cancers, such as testicular cancer, may also have serous retinal detachments. A case study of a retinal detachment without a profilactive argon laser and a known peripheral retinal break is presented. The patient arrived at our clinic with symptoms of retinal detachment that had been present for two to three weeks, as well as peripheral retinal breaks that had been photographed eight months prior.&lt;/span&gt;&lt;/p&gt;&lt;p&gt;&lt;span&gt;&lt;strong&gt;&lt;span&gt;Keywords: &lt;/span&gt;&lt;/strong&gt;&lt;span&gt;Retinal detachment; peripheral retinal breaks; rhegmatogenous; tractional; exudative; profilactive argon laser.&lt;/span&gt;&lt;/span&gt;&lt;/p&gt;

Vascular Tumors and Malformations of the Choroid

Choroidal vascular tumors; it may present as circumscribed and diffuse choroidal hemangiomas and rarely choroidal hemangiopericytoma and phacomatosis pigmentovascularis. Choroidal hemangiomas are benign vascular hamartomatous tumors that occur in two forms: circumscribed and diffuse. The circumscribed form is typically an isolated finding without systemic associations, while the diffuse form generally occurs in association with Sturge–Weber syndrome. Circumscribed choroidal hemangiomas are generally seen as well-circumscribed, round or oval, elevating dome-shaped orange or red masses located posterior to the equator in the fundus, whereas diffuse choroidal hemangiomas have a diffuse red-orange color appearance (tomatoketchup). Although the diagnosis of choroidal hemangioma is better investigated by means of multimodal imaging, indocyanine green angiography and ultrasonography are the most important tests in the differential diagnosis. For circumscribed choroidal hemangiomas with exudative retinal detachment or cystoid macular edema, a variety of treatment modalities are available. Symptomatic patients can be treated with oral propranolol, photodynamic therapy, transpupillary thermotherapy, plaque radiotherapy, external radiotherapy or intravitreal anti-vascular endothelial growth factors.In this review, the pathogenesis, clinical features and treatment methods in choroidal vascular tumors will be discussed.

Surgical and visual outcomes after vitreoretinal surgery for complex retinal capillary hemangioblastoma.

We evaluated the anatomical and functional outcomes after vitreoretinal surgery for complex retinal capillary hemangioblastoma (RCH). This was a retrospective case series of 15 patients (16 eyes) with tractional or combined retinal detachment (RD) managed with pars plana vitrectomy and tumor endoresection (ER) with/without feeder vessel ligation. The mean age at the time of surgery was 30 years (range, 14-46 years). The most common tumor locations were inferotemporal (six eyes) and temporal (six eyes) quadrants. Indications for surgery included exudative RD with fibrovascular proliferation (eight eyes), combined RD (five eyes), vitreous hemorrhage (four eyes), and rhegmatogenous RD (two eyes). Tumor destruction was performed with laser and/or cryotherapy in nine eyes (57%) and ER in seven eyes (43%). Feeder vessel was ligated and cauterized in 10 (63%) and six eyes (37%), respectively. Anatomical success after initial surgery was 50% (eight eyes), which improved to 88% (14 eyes) after they underwent a repeat procedure for recurrent RD (eight eyes). At the last visit, visual acuity improved in seven eyes (44%), was stable in four eyes (25%), and worsened in five eyes (31%) with a mean follow-up of 29 months (6-79 months). Comparison between the ER group and the laser/cryotherapy group revealed no significant difference in final retinal reattachment rate (89% vs. 86%, P > 0.05), with better visual outcomes in laser/cryotherapy group (57% vs. 78%, P < 0.05). Pars plana vitrectomy with/without tumor endoresection can be a safe and effective treatment option for complex RCHs, achieving good tumor control and anatomical success with limited functional success.

Topical brimonidine induced acute uveal effusion in a patient with nanophthalmos: a case report

BackgroundWe report a case of uveal effusion in a nanophthalmic eye after topical use of brimonidine.Case presentationA 42-year-old male patient with nanophthalmos experienced sudden blurred vision in the right eye after using topical brimonidine when picking up tennis balls repeatedly 6 weeks after bilateral YAG peripheral iridotomy. Ocular examination showed wide choroidal and exudative retinal detachment in the temporal and inferior region, involving the macula. Acute uveal effusion in the right, bilateral nanophthalmos was diagnosed. Oral and topical corticosteroids, combined with topical nonsteroids and atropine led to a complete resolution of the uveal effusion after one month.ConclusionThis case suggested a possible causal relationship between the topical use of brimonidine and acute uveal effusion in patients with nanophthalmos. Topical brimonidine should be used with caution in nanophthalmic eyes.

Surgical resection of a retinal pigment epithelium adenoma with exudative retinal detachment

Surgical resection of a retinal pigment epithelium adenoma with exudative retinal detachment

Diagnostic value of 3D Optical Coherence Tomography Multimode Images in the Diagnosis of Acute Central Serous Chorioretinopathy.

Spectral-Domain Optical Coherence Tomography (SD-OCT) provides non-invasive, high-speed, high-resolution, three-dimensional cross-section imaging of the macula. This study aimed to investigate the diagnostic value of the multimodal imaging technique of three-dimension (3D) optical coherence tomography (OCT) (3D-OCT) for the diagnosis and characterization of acute central serous chorioretinopathy (CSC). In this prospective clinical study 3D-OCT examinations of 82 cases with acute CSC were performed on the macular area, and the image characteristics were analyzed. Our study included a total of 87 eyes from 82 cases of CSC patients, 67 males and 15 females (mean age ± standard deviation (SD): 42.89 ±7.80 years old; age range: 27 to 56 years old. The 3D-OCT images were evaluated for the presence of subretinal fluid, subretinal space, fluctuation of the internal limiting membrane (ILM), folds of retinal pigment epithelial (RPE), retinal pigment epithelium detachment (PED), and flat irregular PED. The foveal thickness was measured using the manual caliper of OCT software. The OCT B-scan images showed 87 (100%) eyes had exudative retinal detachment (ERD), 38 (44%) had flat irregular PED, 36 (41%) had PED, 8 (9%) had subretinal turbidity structure, 2 (2%) had subretinal dot-like precipitates, 1 (1%) had focal choroidal excavation (FCE), and 1 (1%) eye had fluctuation of internal limiting membrane (FI). In the ILM-RPE thickness map, all eyes had a round or round like regular uniform domes. Fifty-seven (66%) domes were limited in the examination area and 30 (44%) domes were beyond the scope of this examination and only a partial section of the dome could be observed. In the en-face image, all eyes had a round or round-like black figure that corresponded with domes in the ILM-RPE thickness map. In RPE surface, 76 (87%) eyes had a shallow plate depression, 71(82%) had small focal uplift, and 1 (1%) eye had a focal concave feature. In the OCT ILM-RPE thickness, en-face image, and RPE surface maps, acute CSC exhibited specific imaging characteristics that can be helpful for reliable diagnosis and differential diagnosis of CSC.

The fellow eye of retinal detachment patients: Vision and clinical presentation.

The fellow eye of a retinal detachment is at risk of developing a retinal detachment and other visually debilitating disease. To investigate the rate of bilaterality of retinal detachment (RD), the presenting visual acuity (VA), and the presence of ocular morbidity in the fellow eye of patients with RD. A multicenter, prospective, cross-sectional study examining the fellow eye of consecutive patients who were diagnosed with different types of RD. The patients were seen within one year and examined in four Nigerian eye hospitals and clinics. Demographics, VA, and clinical findings at the presentation were reported on examination of the fellow eyes. Twenty-seven (11.4%) out of 237 patients (264 eyes) had an RD in the fellow eye. The mean age of all study patients was 46.2 ± 16.8 years, M/F: 161 (67.9%)/76 (32.1%). The rates of bilaterality for rhegmatogenous, exudative, and tractional RDs were 4.2%, 11.1%, and 31.1%, respectively. Diagnosis of RD in an eye was associated with a risk of developing fellow eye rhegmatogenous retinal detachment (RRD) (P < 0.001) and tractional RD (P < 0.001), respectively. RRD in an eye was associated with a 17% risk of developing RD in the fellow eye (β = -1.6, OR = 0.202, P < 0.001). The BCVA in the fellow eye of the three types of RD varied significantly (P < 0.001). The fellow eye was blind in 25.2% of RRD, 54.1% of tractional retinal detachment (TRD), and 11.1% of exudative retinal detachment (ERD). Bilateral RD eyes were blind in RRD (85.7%), TRD (71.1%), and ERD (50%). One hundred and seven eyes (40.5%) of the total 264 RD eyes studied had other fellow eye events at the presentation. A patient with an RD in one eye is at significant risk of developing a blinding RD in the fellow eye. This risk varies with the type of RD and is highest with TRD. However, RRD, the commonest type of RD, can benefit from prophylactic treatment to the fellow eye RD predisposing lesions.

Vitreoretinopathy-Associated Pediatric Retinal Detachment Treatment Outcomes: IRIS® Registry (Intelligent Research in Sight) Analysis.

To determine the treatment patterns and outcomes of pediatric retinal detachments (RDs) associated with hereditary vitreoretinopathies. Retrospective cohort analysis using IRIS® Registry (Intelligent Research in Sight) database. Patients < 18 years old with a rhegmatogenous RD and a systemic disorder associated with vitreoretinal degeneration (e.g., Stickler syndrome) or other malformation of the vitreous from 2013-2019. Cases were identified using International Classification of Diseases, Ninth and Tenth Revisions (ICD-9, ICD-10) diagnostic codes from the IRIS® Registry cohort. Other hereditary vitreoretinopathies that are not encoded by specific ICD code(s) were captured by text search. Nonspecific vitreous abnormality ICD codes were also included. Exclusion criteria included traumatic retinal detachments using ICD codes for ocular trauma and serous or exudative retinal detachment. Surgical procedures were identified using Current Procedural Terminology (CPT) codes for repair of retinal detachment. Baseline demographic information collected included age, gender, race/ethnicity, geographic region of the provider location, and health insurance status. Main outcomes measured in this study were average time to first surgery, number of eyes presenting with bilateral detachments, and choice of initial surgical procedure. A total of 2115 eyes of 1722 patients were identified (mean age, 10.4 years; 58% male). The median time to first surgery was 7 days (interquartile range, 40 days). One thousand four hundred seven eyes of 1134 patients had ≥ 1 year of follow-up, with 506 eyes (36%) developing a fellow eye RD. Thirty-three percent of patients presenting with bilateral detachments, and 349 eyes had initial RD surgery within 1 year of the index date documented by CPT code. Fellow eye detachment occurred a mean of 32 days after initial presentation. The mean number of surgeries per eye within 1 year was 1.68. Best-corrected visual acuity did not improve from a baseline 20/54 to 20/62. The initial procedure was most commonly complex RD repair (n = 176), followed by scleral buckle (n = 102), pars plana vitrectomy (n = 89), laser (n = 59), cryotherapy (n = 5), and pneumatic retinopexy (n = 5). There were 51 new diagnoses of glaucoma and 37 new diagnoses of aphakia within 1 year after the surgical procedure. IRIS Registry data provide insight into rare pediatric vitreoretinopathy-associated RDs, which have a high rate of reoperation and fellow eye involvement. Proprietary or commercial disclosure may be found after the references.

Coats-like Vasculopathy in Inherited Retinal Disease: Prevalence, Characteristics, Genetics, and Management

To describe the largest, most phenotypically and genetically diverse cohort of patients with inherited retinal disease (IRD)-related Coats-like vasculopathy (CLV). Multicenter retrospective cohort study. A total of 67 patients with IRD-related CLV. Review of clinical notes, ophthalmic imaging, and molecular diagnosis from 2 international centers. Visual function, retinal imaging, management, and response to treatment were evaluated and correlated. The prevalence of IRD-related CLV was 0.5%; 54% of patients had isolated retinitis pigmentosa (RP), 21% had early-onset severe retinal dystrophy, and less frequent presentations were syndromic RP, sector RP, cone-rod dystrophy, achromatopsia, PAX6-related dystrophy, and X-linked retinoschisis. The overall age of patients at CLV diagnosis was 30.7 ± 16.9 years (1-83). Twenty-one patients (31%) had unilateral CLV, and the most common retinal features were telangiectasia, exudates, and exudative retinal detachment (ERD) affecting the inferior and temporal retina. Macular edema/schisis was observed in 26% of the eyes, and ERD was observed in 63% of the eyes. Fifty-four patients (81%) had genetic testing, 40 of whom were molecularly solved. Sixty-six eyes (58%) were observed, 17 eyes (15%) were treated with a single modality, and 30 eyes (27%) had a combined approach. Thirty-five eyes (31%) were "good responders," 42 eyes (37%) were "poor responders," 22 eyes (19%) had low vision at baseline and were only observed, and 12 eyes (11%) did not have longitudinal assessment. Twenty-one observed eyes (62%) responded well versus 14 (33%) treated eyes. Final best-corrected visual acuity was significantly worse than baseline (P = 0.002); 40 patients (60%) lost 15 ETDRS letters or more over follow-up in 1 or both eyes, and 21 patients (31%) progressed to more advanced stages of visual impairment. Inherited retinal disease-related CLV is rare, sporadic, and mostly bilateral; there is no gender predominance, and it can occur in diverse types of IRD at any point of the disease, with a mean onset in the fourth decade of life. Patients with IRD-related CLV who have decreased initial visual acuity, ERD, CLV changes affecting 2 or more retinal quadrants, and CRB1-retinopathy may be at higher risk of a poor prognosis. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Tectonic Keratoplasty Using Acellular Preserved Human Cornea for the Inciting Eye in Sympathetic Ophthalmia

Purpose: We report a case of sympathetic ophthalmia in which the inciting eye was treated with a tectonic keratoplasty using acellular preserved human cornea.Case summary: A 68-year-old man whose left eye was injured by a nail visited our institute complaining of a recent decrease in bilateral vision. The best corrected visual acuity was 0.32 for the right eye and 0.16 for the left. On slit-lamp examination, a superior peripheral corneal perforation with no wound leakage due to iris plugging was seen in the left eye (i.e., the inciting eye). The right eye (i.e., the sympathizing eye) had mutton-fat keratic precipitates and copious fibrinoid strands in the anterior chamber. Bilateral exudative retinal detachment with choroidal folds was prominent in both eyes. The next day, the necrotic iris lump was removed from the inciting eye and adhesiolysis of the iris and tectonic keratoplasty using acellular preserved human cornea were performed. Systemic steroid was started immediately postoperatively. The exudative retinal detachment improved in both eyes and cataract surgery was undertaken for the inciting eye 4 months later. Twenty-one months after the initial visit, the uncorrected visual acuity was 1.0 in the inciting eye, and both eyes had a sunset glow fundus appearance, without optic atrophy or recurrent chorioretinitis.Conclusions: Tectonic keratoplasty using acellular preserved human cornea with anti-inflammatory treatment may be a favorable therapeutic option for the inciting eye with peripheral corneal perforation in sympathetic ophthalmia.