Ultra-Wide-Field Fluorescein Angiographic Features Influencing Visual Prognosis in Coats' Disease.

Purpose: To investigate the retinal vascular abnormalities in both affected and fellow eyes of presumed unilateral Coats' disease patients using ultra-wide-field fluorescein angiography (UWF-FA) and their association with visual prognosis. Methods: A retrospective review of 30 patients diagnosed with presumed unilateral Coats' disease, evaluated with UWF-FA from March 2003 to May 2024 in a tertiary referral hospital. Clinical features and multimodal imaging findings were evaluated and factors related to final visual outcomes were analyzed. Results: All 30 patients were diagnosed with presumed unilateral Coats' disease at presentation, comprising 36.7% childhood-onset and 63.3% adult-onset patients. Retinal vascular telangiectasia was observed in 51.7% of fellow eyes. The extent of telangiectasia and exudate in the affected eyes did not significantly correlate with extent of telangiectasia in the fellow eyes. In the more affected eyes, the childhood-onset group had a significantly greater extent of capillary drop-out compared to the adult-onset group (5.0 vs 2.8 clock hours, p = 0.023). In the fellow eyes, telangiectasia tended to be more frequent in the childhood-onset group (63.6% vs. 44.4%), without significance (p = 0.160). In the multivariable regression analysis, the final best-corrected visual acuity (BCVA) in the more affected eye was significantly associated with initial BCVA. The mean extent of telangiectasia in the temporal and nasal quadrants and the initial BCVA of the more affected eyes were identified as prognostic factors for final moderate to severe visual loss (Snellen BCVA < 20/66) (OR = 12.8, p = 0.043; OR = 11.8, p = 0.024, respectively). Conclusions: About half of presumed unilateral Coats' disease cases exhibited features of bilateral disease. Visual prognosis is associated with the peripheral retinal telangiectasia in the temporal and nasal quadrants as well as initial BCVA in the affected eyes while it is not associated with retinal vascular abnormalities in the fellow eyes.

Multimodal imaging diagnosis and analysis of prognostic factors in patients with adult-onset Coats disease.

AIM: To describe the multimodal imaging features, treatment, and outcomes of patients diagnosed with adult-onset Coats disease. METHODS: This retrospective study included patients first diagnosed with Coats disease at ≥18 years of age between September 2017 and September 2021. Some patients received anti-vascular endothelial growth factor (VEGF) therapy (conbercept, 0.5 mg) as the initial treatment, which was combined with laser photocoagulation as needed. All the patients underwent best corrected visual acuity (BCVA) and intraocular pressure examinations, fundus color photography, spontaneous fluorescence tests, fundus fluorescein angiography, optical coherence tomography (OCT), OCT angiography, and other examinations. BCVA alterations and multimodal image findings in the affected eyes following treatment were compared and the prognostic factors were analyzed. RESULTS: The study included 15 patients who were aged 24-72 (57.33±12.61)y at presentation. Systemic hypertension was the most common associated systemic condition, occurring in 13 (86.7%) patients. Baseline BCVA ranged from 2.0 to 5.0 (4.0±1.1), which showed improvement following treatment (4.2±1.0). Multimodal imaging revealed retinal telangiectasis in 13 patients (86.7%), patchy hemorrhage in 5 patients (33.3%), and stage 2B disease (Shield's staging criteria) in 11 patients (73.3%). OCT revealed that the baseline central macular thickness (CMT) ranged from 129 to 964 µm (473.0±230.1 µm), with 13 patients (86.7%) exhibiting a baseline CMT exceeding 250 µm. Furthermore, 8 patients (53.3%) presented with an epiretinal membrane at baseline or during follow-up. Hyper-reflective scars were observed on OCT in five patients (33.3%) with poor visual prognosis. Vision deteriorated in one patient who did not receive treatment. Final vision was stable in three patients who received laser treatment, whereas improvement was observed in one of two patients who received anti-VEGF therapy alone. In addition, 8 of 9 patients (88.9%) who received laser treatment and conbercept exhibited stable or improved BCVA. CONCLUSION: Multimodal imaging can help diagnose adult-onset Coats disease. Anti-VEGF treatment combined with laser therapy can be an option for improving or maintaining BCVA and resolving macular edema. The final visual outcome depends on macular involvement and the disease stage.

Surgical Drainage of Large Macular Cystoid Spaces in Coats Disease.

Purpose: To describe an option of surgically draining large macular cystoid spaces in a patient with Coats disease. Methods: A case and its findings were analyzed. Results: A standard pars plana vitrectomy was performed to aspirate large macular cystoid spaces using a subretinal cannula with intraoperative optical coherence tomography guidance. Because of the viscous nature of the chronic fluid and lipid exudates, the contents of the large cystoid spaces were drained through a retinotomy using a soft-tipped aspiration cannula. Postoperative follow-up after surgical drainage showed immediate resolution of the macular cystoid spaces and gradual resolution of the dense subfoveal exudates over 1 year. Although surgical intervention led to the eventual resolution of the macular edema and exudates, visual recovery was limited by the chronicity of the condition. Conclusions: Surgical drainage of large macular cystoid spaces in Coats disease can be achieved in eyes that are refractory to medical management. Earlier surgical intervention in select cases may allow for visual rehabilitation.

Triple Trouble with Triple Solutions: A Unique Case Report of a Severe Exudative Retinal Detachment Accompanied by Retinal Traction and Two Retinal Holes in Coats Disease.

Introduction: Coats disease is a rare vasculature pathology that usually presents as retinal telangiectasia with possible progression to exudative retinal detachment (RD). Intravitreal anti-VEGF injections, cryotherapy, laser photocoagulation, and surgery are commonly used to control the disease and prevent its progression. Although iatrogenic tractional RDs secondary to anti-VEGF injections have been reported in patients with Coats disease, RDs in Coats disease are exudative, secondary to retinal exudation and vascular abnormalities. In this article, we present the first reported case of a severe exudative RD accompanied by retinal traction and two retinal holes in a patient with Coats disease. Case Presentation: A 32-year-old male initially presented with Coats disease stage 2A, which then progressed to 3A1 within a month of close follow-ups, finally leading to exudative RD. Following cryotherapy and bevacizumab injections, the exudative RD progressed, with the emergence of retinal traction and two retinal holes. This complex case was successfully treated with a scleral buckle vitrectomy accompanied by radial elements to support the RD, pars plana vitrectomy, silicon oil tamponade, and post-surgical bevacizumab injections. Six months following the surgical intervention, the patient's vision is restored at 20/30, and retinal imaging shows a totally flattened retina. Conclusion: We present the first reported case of a severe exudative RD accompanied by retinal traction and two retinal holes in a patient with Coats disease. The combination of surgical treatment and bevacizumab injections is thought to have collectively contributed to our patient's favorable outcome.

Long-term efficacy and complications of intravitreal anti-vascular endothelial growth factor agents combined with ablative therapies in juvenile Coats disease: a five year follow-up study.

PURPOSE: To evaluate the long-term safety and efficacy of adjuvant intravitreal anti-VEGF therapy in juvenile Coats disease. METHODS: This retrospective, observational study included a total of 62 eyes in 62 pediatric patients with juvenile Coats disease treated with intravitreal anti-VEGF agents followed for a mean of 67.08 months (ranged from 60 to 93 months). All affected eyes were managed initially with one session of ablative treatment plus adjuvant intravitreal anti-VEGF agent (0.5 mg/0.05 ml ranibizumab or conbercept). Ablative treatment was repeated if telangiectatic retinal vessels were not completely regressed or recurred. Anti-VEGF therapy was repeated if subretinal fluid or macular edema still existed. Treatments above were repeated every 2 to 3 months. We reviewed clinical and photographic records of patients including the demographics, clinical characteristics and interventions. RESULTS: At final visit, all 62 affected eyes had partially or completely disease resolution; none progressed to advanced stage namely neovascular glaucoma or phthisis bulbi, respectively. No ocular or systemic side effects related to intravitreal injections were observed during follow-up. In terms of 42 affected eyes that could cooperate with visual examination, best corrected visual acuity improved in 14 (14/42, 33.3%) eyes, stabled in 25 (25/42, 59.5%) eyes, and worsened in 3 (3/42, 7.1%) eyes. In the field of complications, 22 (22/62, 35.5%) eyes developed cataracts; 33 (33/62, 53.2%) eyes developed vitreoretinal fibrosis, of whom 14 (14/33, 42.4%) eyes in the subgroup of stage 3B developed progressive TRD; 40 (40/62, 64.5%) eyes developed subretinal fibrosis. Multivariate regression analysis showed increased clinical stage may be associated with the development of vitreo- and subretinal fibrosis (adjusted odds ratio:16.77,17.59; 95% CI:4.50-62.53, 3.98-77.86, respectively, all P < 0.001). CONCLUSION: Adjuvant intravitreal ranibizumab or conbercept combined with ablative therapies may be a long-term safe and effective treatment for juvenile Coats disease.

Subfoveal Nodule Affecting Visual Prognosis in Coats Disease.

PURPOSE: To investigate the characteristics of subfoveal nodules in Korean patients with Coats disease and their association with visual outcomes. METHODS: A retrospective analysis was conducted within the medical records of patients with stage 2B or 3A1 Coats disease, including clinical features, imaging, presence of either a subfoveal nodule or macular fibrosis, and visual outcome. RESULTS: Twelve patients were present with stage 2B or 3A1 Coats disease, and nine patients (75%) presented with subfoveal nodule. Between the group without subfoveal nodule and the group with subfoveal nodule, there were no significant differences in age (mean, 14.0 ± 1.7 years vs. 27.7 ± 21.8 years; p = 0.482), sex (all men), stage of the disease (stage 2B: three patients vs. eight patients, p > 0.999; stage 3A1: none vs. one patient, p > 0.999), extension of retinal exudation (mean, 7.7 hours vs. 4.1 hours; p = 0.209) and peripheral telangiectasia (mean, 3.7 hours vs. 4.2 hours; p = 0.727), and follow-up duration (mean, 65.0 months vs. 46.1 months; p = 0.600). There were significantly more patients with severe visual loss (≤20 / 200) among the patients with subfoveal nodule (none vs. seven patients, p = 0.045), and the cause for severe visual loss was macular fibrosis in all cases. Macular fibrosis developed significantly more frequently in the patients with subfoveal nodule (none vs. seven = patients, p = 0.045). CONCLUSIONS: This study is the first study covering the analysis of subfoveal nodules in Korean patients with Coats disease. The existence of a subfoveal nodule at the initial diagnosis serves as an indicator predicting the development of macular fibrosis and a less favorable visual outcome in the patients with Coats disease. A multicenter study with a larger patient pool and further studies toward the therapeutic approach for the subfoveal nodule and macular fibrosis are needed.

Pediatric retinal vascular disorders: From translational sciences to clinical practice.

Pediatric retinal vascular diseases are a spectrum with overlapping phenotypes and related genes. Retinal vascular development is biphasic. Vasculogenesis is responsible for the formation of primordial vessels leading to the four major arcades in the posterior retina. Angiogenesis, which is vascular endothelial growth factor dependent, is responsible for the formation of new vessels through budding from existing vessels, forming the peripheral vessels, increasing the capillary density of the central retina, and forming the superficial and deep capillary plexus. This process is controlled by WNT signaling, which is important for cell proliferation, division, and migration. Disorders of WNT signaling, such as familial exudative vitreoretinopathy (FEVR), have overlapping clinical findings. Conversely, pathogenic variants in some of the FEVR-related genes are reported in conditions such as retinopathy of prematurity (ROP), persistent fetal vasculature, and Coats disease. The various overlapping features and underlying genetic basis in the pathogenesis of pediatric retinal vascular developmental diseases suggest that genetic variants may provide a framework or a background for these conditions, upon which further insults can affect the development at any phase (such as prematurity and oxygenation in ROP), influencing and determining the final phenotype.

Anterior Uveitis and Coats Disease in a 16-Year-Old Girl with Noonan Syndrome-A Case Report.

Background: Noonan syndrome (NS) represents a fairly common genetic disorder with a highly variable phenotype. Its features include inherited heart defects, characteristic facial features, short stature, and mild retardation of motor skills. Case presentation: A 16-year-old Caucasian girl with NS reported visual deterioration, photophobia, and pain in the right eye (RE). The initial best-corrected visual acuity (BCVA) was 0.3 in the RE. An examination demonstrated conjunctival and ciliary body hyperemia, keratic precipitates, and flare in the anterior chamber. In addition, post-hemorrhagic floaters, tortuous vessels, and an epiretinal membrane in the RE were present. Diagnosis of unilateral anterior uveitis was made, and this resolved after the use of topical steroids and cycloplegic drops. Due to the presence of retinal telangiectasias and extraocular exudates (consistent with Coats' disease (CD) stage 2A) in the RE, laser therapy was performed. The patient remains under constant follow-up, and after one year, the BCVA in the RE was 0.7. Conclusions: Here, we report the clinical characteristics, genetic findings, and retinal imaging results of a patient with NS. To our knowledge, this is, to date, the first report of an association of NS with a PTPN11 mutation with anterior uveitis and CD.

Lifting the White Walker's curse-Management of Coats' disease.

Background: Coats' disease, described by George Coats in the early 1900s, is an idiopathic unilateral retinal vascular abnormality with exudation occurring in young males. It is characterized by retinal telangiectasia with intraretinal or subretinal exudation. Coats' disease is mostly diagnosed in the first to the second decade of life, with a common presentation of leukocoria. Younger patients have a more severe presentation and are associated with poor visual prognosis. Management of Coats' disease varies from observation, cryotherapy with anti-VEGFs (Vascular Endothelial Growth Factor), and surgery to enucleation. The mode of treatment depends on the age of presentation, the severity of the disease, and the stage of the disease. Through this video, we describe the clinical features, pathology, and surgical management of a 2-year-old child with grade 3B of Coats' disease. Purpose: To demonstrate successful surgical management of grade 3B of Coats' disease in a 2-year-old boy. Synopsis: Coats' disease mostly presents with a diagnostic dilemma due to its varied presentation. Early detection and treatment are the keys to salvaging the eye as well as the vision, hence, avoiding dreadful complications such as neovascular glaucoma or phthisis bulbi. We demonstrate successful surgical management of a child who presented with grade 3B of Coats' disease. Highlights: Through this video, we aim to describe the clinical features, pathology, and surgical management of a 2-year-old child with grade 3B of Coats' disease. Combination of external drainage with vitrectomy, challenges faced, and the importance of visual rehabilitation postoperatively. Video Link: https://youtu.be/0obpVTOkKKs.

Efficacy and Safety of Anti-Vascular Endothelial Growth Factor Drugs for Coats' Disease Treatment: A Systematic Review.

Purpose: The efficacy and safety of anti-vascular endothelial growth factor (anti-VEGF) treatment for Coats' disease remains controversial. This study was designed to evaluate the efficacy and safety of anti-VEGF treatment for Coats' disease. Methods: PubMed, Embase, The Cochrane Library, Clinical Trials, CNKI, and WanFang databases were systematically searched for clinical efficacy and safety studies on anti-VEGF treatment for Coats' disease through June 2021. Study selection, data extraction, and quality assessment were independently performed by 2 reviewers. Quality assessments were performed using the Joanna Briggs Institute Critical Appraisal tools and GRADE-CERQual. Results: A total of 1,501 articles were retrieved and reviewed, of which 24 case series involving 378 patients (range: 3-67 patients each with 3-71 eyes) were included in the analysis. No randomized controlled trials, case-controlled studies, or cohort studies were available for analysis. Most patients were male (60.0%-92.9%), aged 1.35-42.3 years, with a median follow-up time ranging from 3 to 63 months. Among the 24 case series, 22 reported changes in the visual acuity (VA) after anti-VEGF treatment and 21 reported safety outcomes. The results showed that VA improved in 73 patients (37.63%), was stable in 89 (45.87%), and worsening VA was observed in 12 cases (6.19%). The most common adverse event was fibrotic changes (n = 35). Systemic complications were not observed. Conclusions: The results of this study indicate that anti-VEGF drugs provide an effective and relatively safe treatment strategy for Coats' disease. However, conducting well-designed, prospective, randomized clinical trials are necessary to confirm our findings.

Management of Coats-Like Disease in a Forty-Four-Year-Old Patient with FSHD Type I.

A forty-four-year-old female patient known for FSHD type I, with unremarkable past ocular history, complained of progressive visual acuity deterioration during a routine ophthalmological visit. Best-corrected visual acuity (BCVA) was 1.0 decimal Snellen equivalent bilaterally. Dilated fundus examination showed evidence of retinal Coats-like disease in the left eye, while the right eye showed significant retinal vascular tortuosity. Multimodal examinations (OCT scans and FA-fluorescein angiography) revealed large areas of retinal ischemia, thus confirming a retinal vascular disorder compatible with the diagnosis of Coats-like disease. Left eye laser photocoagulation of the ischemic areas was performed to avoid neovascular complications that had not been detected during follow-up visits (12 months), and BCVA in the left eye remained stable at 1.0 decimals Snellen equivalent. Coats-like disease in a patient affected by FSHD type I should always be screened even in the absence of any prior ocular diseases. Guidelines concerning the ophthalmological management of adults affected by FSHD are lacking. Based on this case, we recommend performing a yearly complete ophthalmological checkup with dilated fundus examination and retinal imaging. Patients should, furthermore, be encouraged to seek medical attention when noticing deterioration of visual acuity or other visual symptoms in order to avoid missing potential sight-threatening ocular complications.

Full-Thickness Macular Hole in Coats Disease Treated Using the Inverted Internal Limiting Flap Technique.

Purpose: To report vitrectomy with the inverted internal limiting membrane (ILM) flap technique in a patient with a full-thickness macular hole (FTMH) and Coats disease. Methods: A case and its long-term findings were analyzed. Results: A 27-year-old patient with Coats disease who was treated 5 years earlier with laser photocoagulation presented with an FTMH. Vitrectomy with the temporal inverted ILM flap technique was performed. The macular hole decreased in size on serial OCT scans but did completely close until 18 months postoperatively. The final visual acuity was 20/40 (0.3 logMAR). The patient's vision remained stable for the next 5 years. Conclusions: Although the healing process after vitrectomy with ILM peeling and the inverted flap technique in an FTMH coexisting with Coats disease is prolonged compared with an idiopathic FTMH, it is still possible to obtain satisfactory anatomic and functional results.

[Molecular genetics in diagnosis of Coats disease: combination of oligogenic variants associated with different forms of hereditary retinal dystrophy]. / Molekulyarno-geneticheskie nakhodki pri diagnostike bolezni Koatsa: sochetanie oligolokusnykh izmenenii, svyazannykh s raznymi nozologicheskimi formami nasledstvennoi retinopatii.

Coats disease (OMIM 300216) is a form of hereditary retinal dystrophy, which occurs due to congenital abnormality of retinal vessels and features unilateral exudative vitreoretinopathy. Coats disease mostly occurs sporadically; its genetic cause is still undetermined. Molecular genetic research including whole exome sequencing by the NGS method was used to define a genetic cause of the observed phenotype. Two heterozygous variants in different genomic loci associated with other forms of hereditary retinal dystrophy were detected, a rare variant in the HMCN1 gene c.9571C>T, p.(Arg3191Cys), and a known pathogenic variant in the NPHP4 gene c.2930C>T, p.(Thr977Met). The HMCN1 gene is responsible for dominant age-related macular degeneration (OMIM 603075), pathogenic variants in the NPHP4 gene cause recessive Senior-Løken syndrome 4 (OMIM 266900). These genes encode the proteins that are involved in the regulation of integrity of the blood-retinal barrier in the vascular endothelium (NPHP4) and retinal pigment epithelium (HMCN1). The identified mutation in the NPHP4 gene could lead to decreased function of the NPHP4 protein and contribute to the development of retinal degeneration, potentially of oligogenic nature.

Avascular Peripheral Retina in Infants.

Avascular peripheral retina in an infant is a common characteristic of numerous pediatric retinal vascular disorders and often presents a diagnostic challenge to the clinician. In this review, key features of each disease in the differential diagnosis, from retinopathy of prematurity, familial exudative vitreoretinopathy, Coats disease, incontinentia pigmenti, Norrie disease, and persistent fetal vasculature, to other rare hematologic conditions and telomere disorders, will be discussed by expert ophthalmologists in the field.

Pseudoretinoblastoma: Distribution based on gender, age, and laterality.

PURPOSE: To investigate the distribution of pseudoretinoblastoma (PSRB) cases based on gender, age, and laterality. MATERIALS AND METHODS: The clinical records of 607 patients (851 eyes) who were referred for diagnosis of retinoblastoma or simulating conditions between October 1998 and May 2021 were retrospectively evaluated. Patients were stratified by age as follows: ≤1 year, >1-3 years, >3-5 years, and >5 years. RESULTS: Of 190/607 PSRB patients, 129 (67.9%) were males and 61 (32.1%) females (p = 0.001). The 3 most common diagnoses in males were Coats disease (20.2%), persistent fetal vasculature (PFV, 14.0%), and chorioretinal coloboma (6.2%). In females, the 3 most common diagnoses included PFV (21.3%), retinal dysplasia, congenital glaucoma, and combined hamartoma (each 6.6%). PFV was the most common diagnosis in ≤1 year old patient group (26.6%). Coats disease and PFV were the most common diagnoses in >1-3 years old patient group (each 16.7%). Coats disease was the most common diagnosis in >3-5 years old (30.8%) and >5 years old patient groups (13.1%). PSRBs were unilateral in 121/190 (63.7%) patients. Coats disease usually presented unilaterally (p < 0.001) while PFV, optic nerve head drusen, and retinopathy of prematurity as bilateral diseases (p = 0.019, p = 0.001, and p = 0.001 respectively). DISCUSSION: PSRB diagnoses show differences depending on gender, age, and laterality. In our study, the most common PSRB lesions were Coats disease in males and PFV in females. PFV was the most frequent diagnosis in ≤3 years and Coats disease in >3 years of age groups. Coats disease and PFV were the most common unilateral and bilateral PSRB diagnoses respectively.

Genetic evaluation in phenotypically discordant monozygotic twins with Coats Disease.

PURPOSE: To report the unique case of a pair of phenotypically discordant monozygotic twins, with one of them affected by unilateral Coats disease. CASE REPORT: Both patients underwent a complete ophthalmologic evaluation and were genetically tested with whole-exome sequencing (WES). Any known or unknown potential genetic determinant of Coats disease wasn't found. CONCLUSION: It may suggest a non-genetic etiology for this disorder. This represents, to the best of our knowledge, the first case of genetic analysis of monozygotic twins, one of whom is affected by Coats disease. Further studies are warranted, including performing genetic analysis directly on retinal biopsy tissue.

Changing trends in pseudoretinoblastoma diagnoses: A 10 year review from the United Kingdom.

AIM: To study the different types and frequency of pseudoretinoblastoma (pseudoRB) lesions who present to a retinoblastoma centre due to concern that the condition may be retinoblastoma. METHODS: A retrospective chart review of 341 patients presenting sporadically to the Royal London Hospital from January 2009 to December 2018. RESULTS: 220 patients (65%) were confirmed to have retinoblastoma, while 121 (35%) had pseudoRB. There were 23 differential diagnoses in total. The top 3 differential diagnoses were Coats' disease (34%), Persistent Foetal Vasculature (PFV) (17%) and Combined Hamartoma of Retina and Retinal Pigment Epithelium (CHR-RPE) (13%). PseudoRBs differed with age at presentation. Under the age of 1 (n = 42), the most likely pseudoRB conditions were PFV (36%), Coats' disease (17%) and CHR-RPE (12%). These conditions were also the most common simulating conditions between the ages of 1 and 2 (n = 21), but Coats' disease was the most common in this age group (52%), followed by CHR-RPE (19%) and PFV (14%). Between the ages of 2 and 5 (n = 32), Coats' disease remained the most common (44%) pseudoRB lesion followed by CHR-RPE (13%), or PFV, Retinal Astrocytic Hamartoma (RAH), familial exudative vitreoretinopathy (FEVR) (all 6.3%). Over the age of 5 (n = 26), pseudoRBs were most likely to be Coats' disease (35%), RAH (12%), Uveitis, CHR-RPE, FEVR (all 7.7%). CONCLUSION: 35% of suspected retinoblastoma cases are pseudoRB conditions. Overall, Coats' disease is the most common pseudoRB condition, followed by PFV. Hamartomas (CHR-RPE & RAH) are more prevalent in this cohort, reflecting improvements in diagnostic accuracy from referring ophthalmologists.

Pediatric Coats disease of stage 2 and greater managed with 532 nm wavelength laser using indirect ophthalmoscope combined with ranibizumab / 中华眼底病杂志

Objective:To investigate the efficacy of 532 nm wavelength laser using indirect ophthalmoscope combined with ranibizumab (IVR) in treating stage 2 and greater pediatric Coats disease.Methods:A retrospective, non-controlled clinical study. From February 2018 to August 2020, 21 eyes of 21 patients with Coats disease stage 2 and greater diagnosed by examination in the Eye Center of Beijing Tongren Hospital were included in the study. Among them, 20 patients were males; 1 patient was female. Mean age was 5.00±1.92 years old. Stage 2A, 2B, 3A, 3B, and 4 were 2, 8, 7, 2, and 2 eyes, respectively. All eyes underwent wide-field fundus color photography and fluorescein fundus angiography (FFA). Best corrected visual acuity (BCVA) was performed in 17 eyes. Abnormal dilated retinal blood vessels, interretinal and subretinal exudates were found in all eyes. Abnormally dilated capillaries and aneurysms in the retina was shown in FFA examination. All eyes underwent 532 nm laser photocoagulation using indirect ophthalmoscope combined with IVR. Patients with severe retinal detachment of stage 3B or greater were treated by external drainage of subretinal fluid (SRF). The subsequent treatment was the same as before. The follow-up time was 35.67±6.13 months. Relevant examinations were performed using the same equipment and methods before. The frequency of treatment, visual acuity changes, anatomic prognosis, and complications were observed.Results:The frequency of eye photocoagulation was 2.43±0.98. The number of IVR treatments was 2.00±0.89. Three eyes were treated with SRF drainage in the first time. At the last follow-up, visual acuity improved, no change, and decreased in 5, 11, and 1 eyes after BCVA examination, respectively. In 21 eyes, the retina was in situ in 17 eyes; 5 eyes with retinal cysts. During the follow-up, cataract and vitreous hyperplasia occurred in 1 eye, which was treated by vitrectomy, and mild vitreous hyperplasia occurred in 1 eye.Conclusion:Indirect ophthalmoscope 532 nm wavelength laser combined with IVR is an effective treatment for pediatric Coats disease.

Long-term outcomes of adjuvant intravitreal injection of conbercept therapy in juvenile Coats disease / 中华眼底病杂志

Objective:To study the long-term effects and outcomes of adjuvant intravitreal injection of conbercept (IVC) therapy in juvenile Coats disease.Methods:A retrospective case series study. From January 1, 2015 to December 31, 2018, 40 patients (40 eyes) who were diagnosed as juvenile Coats disease at Beijing Tongren Hospital Affiliated to Capital Medical University were included in the study. Among them, there were 37 males (37 eyes) and 3 females (3 eyes). All patients had unilateral Coats disease. The average age was 55.00 (44.75, 81.25) months. Five eyes were in stage 2B, 15 eyes were in stage 3A, 19 eyes were in stage 3B and 1 eye was in stage 4. Idiopathic retinal vascular telangiectasia associated with extensive subretinal fluid (SRF) (stage 3 or above) or massive foveal exudation and edema (stage 2B) were found in fundus examination. All affected eyes underwent wide-field color fundus images and fluorescein fundus angiography. Thirty-one eyes underwent best corrected visual acuity (BCVA) examination. The BCVA was carried out using a standard logarithmic visual acuity chart, which was converted into the logarithmic minimum angle of resolution (logMAR) visual acuity. All cases received adjuvant IVC combined with treatments such as retinal photocoagulation. The average number of injections was 4 (1, 5). The average follow-up after initial treatment was 59.00 (52.50, 63.00) months. The changes in BCVA, occlusion of abnormal blood vessels in fundus, absorption of SRF and ocular and systemic complications were observed.Results:At last follow-up, among 31 affected eyes with the examination of BCVA, 13 (32.5%, 13/40) eyes had an improved vision, 12 eyes(30.0%, 12/40) had a stable vision and 6 eyes (15.0%, 6/40) had a decreased vision. The difference between average logMAR BCVA of the affected eyes in each stage after treatment and that before treatment was not statistically significant ( Z=-0.56, -1.80, -0.84; P>0.05). Abnormal blood vessels in fundus were all partially or completely occluded, and SRF was obviously or completely absorbed in all cases; of which, 28 eyes (70.0%, 28/40) were completely occluded, and 12 eyes (30.0%, 12/40) were partially occluded. No patient underwent eye enucleation. Nineteen eyes (47.5%, 19/40) developed vitreoretinal fibrosis; 8 eyes (20.0%, 8/40) developed tractional retinal detachment; 15 eyes (37.5%, 15/40) developed complicated cataract. None had ocular or systemic complications related to IVC therapy during follow-up. Conclusions:IVC combined with classic treatments such as photocoagulation in juvenile Coats disease can keep or improve the visual acuity in most juvenile patients by reducing SRF. IVC is a long-term safe and effective adjuvant therapy in juvenile Coats disease.

Coats disease in India: clinical presentation and outcome in 675 patients (690 Eyes).

PURPOSE: To study the clinical profile, treatment, and visual outcome of patients with Coats disease in India. METHODS: This was a cross-sectional, observational hospital-based study of patients diagnosed with Coats disease during a 10-year period using an electronic medical record system. RESULTS: We identified 675 patients with Coats disease with a prevalence rate of 0.025%. The mean age of the patients was 16.8 years (median, 12 years). Majority were males (75%) with unilateral presentation (98%) in first decade of life (n = 309, 46%). The most common presentation was foveal exudation (stage 2B, n = 161, 23.3%), followed by exudative retinal detachment-extrafoveal (stage 3A1, n = 143, 20.7%), and extrafoveal exudation (stage 2A, n = 136, 19.7%). Treatment modalities included observation (48 eyes, 17%), laser photocoagulation ± intravitreal bevacizumab/triamcinolone acetonide (n = 82, 29%), cryotherapy ± intravitreal bevacizumab/triamcinolone acetonide (n = 64, 23%), and surgical intervention (n = 86, 31%). Despite appropriate treatment, at mean follow-up of 16 months, there was no significant difference between presenting and final visual acuity (48% vs. 48%, p > 0.05). Using multivariate regression analysis, factors associated with poor visual outcome were younger age (< 0.001; - 0.02 to - 0.1), unilateral disease (0.04; - 0.68 to - 0.01), cataract (0.004; 0.13 to 0.69), retinal detachment (< 0.001; 0.49 to 0.82), and glaucoma (< 0.001; 0.34 to 0.94). CONCLUSION: The most common clinical presentation of Coats disease in India is foveal exudation in first and second decade. At initial presentation, about half of the affected eyes had blindness and one-third underwent surgical intervention.