ABSTRACT
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.
Subject(s)
Retinal Telangiectasis , Male , Humans , Child , Adolescent , Retinal Telangiectasis/complications , Retinal Telangiectasis/diagnosis , Retrospective Studies , Fluorescein Angiography/methods , Prognosis , Fibrosis , Follow-Up StudiesABSTRACT
ABSTRACT We report two cases of stage 3A unilateral Coats' disease in pediatric patients. In both cases, disease control was achieved using a dexamethasone intravitreal implant in addition to other treatments. The treatment improved visual acuity in one patient and prevented the worsening of the decline in visual acuity in the other patient during follow-up periods of 7 and 3 years, respectively. One of the patients presented an increase in intraocular pressure, which was controlled with topical antiglaucoma medication, but developed a cataract that required surgery. In conclusion, dexamethasone intravitreal implant may be a useful adjuvant treatment to consider in some pediatric cases with Coats' disease.
RESUMO Relatamos dois casos de doença de Coats em estágio 3A unilateral em pacientes pediátricos. Em ambos os casos, o controle da doença foi obtido com implante intravítreo de dexametasona, além de outros tratamentos, com melhora da acuidade visual em um caso e sem piora da visão no outro, durante um período de acompanhamento de 7 e 3 anos. Um dos casos apresentou elevação da pressão intraocular controlada com medicação antiglaucoma tópica e desenvolveu catarata que exigiu cirurgia. Em conclusão, o implante intravítreo de dexametasona pode ser um tratamento adjuvante útil a ser considerado em alguns casos pediátricos com doença de Coats.
ABSTRACT
Objective:To investigate macular microvascular abnormalities in eyes with subfoveal fibrotic nodules secondary to Coats' disease.Methods:A cross-sectional study. From January 1, 2018 to July 30, 2021, 45 eyes of 45 patients diagnosed with Coats' disease with or without subfoveal fibrotic nodules in Eye and ENT Hospital, Shanghai Medical College of Fudan University were included in this study. There were 40 eyes in 40 males and 5 eyes in 5 females. All were under 21 years old. According to the presence or absence of subfoveal fiber nodules, the patients were divided into fibrotic group (26 cases, 26 eyes) and non-fibrotic group (19 cases, 19 eyes). Optical coherence tomography angiography was used to scan 3 mm×3 mm or 6 mm×6 mm macular area of both eyes. The software of the device automatically processed the images. The presence of FAZ edge anastomotic vascular arch ring breakage and abnormal microvascular branch (AMB) in the foveal avascular zone (FAZ) were observed.Results:In 26 eyes of fibrosis group, AMB originating from the parafoveal retinal capillary network was observed, which grew into and destroyed the integrity of the vascular arch ring at the edge of FAZ. AMB was crisscrossing and winding, and its curvature expands. B-scan images showed the blood flow signal in the subfoveal fiber nodule, and the blood flow signal traversed between the inner retina and the fiber nodule in 23 eyes (88.46%, 23/26). In the non-fibrosis group, all the vascular abnormalities were characterized by capillary dilation and defect, and no breakage of FAZ anastomotic vascular arch ring or AMB was observed.Conclusions:In Coats' disease with subfoveal fiber nodules, staggered and dilated AMBs emerge from the parafoveal vascular network, grow into and destroy the integrity of the vascular arch ring at the edge of FAZ, and grow down longitudinally into the fiber nodules.
ABSTRACT
Objective:To investigate the efficacy and safety of traditional laser photocoagulation, laser combined with intravitreal injection of anti-vascular endothelial factor (anti-VEGF) drugs and intravitreal injection of anti-VEGF drugs alone in Coats disease.Methods:The patients diagnosed as Coats disease stage 2B-3A2 in Department of Ophthalmology, Eye and ENT Hospital of Shanghai Medical College of Fudan University from December 2016 to November 2019 were included in this study. Patients were divided into three groups, including laser group, combined group and drug group, according to the different treatment. In the laser group, the initial treatment was traditional laser photocoagulation alone. In the drug group, the anti-VEGF drug was injected into vitreous once a month for three months. The initial treatment of the eyes in the combined group was laser combined with intravitreal injection of anti-VEGF drugs, or laser treatment within 1 week after anti-VEGF drug treatment. The follow-up time was more than 6 months, and best-corrected visual acuity (BCVA), ultra-wide-angle fundus photography, and fluorescein fundus angiography were performed during follow-up. The treatment efficiency, subretinal fluid (SRF), macular edema, BCVA and complications were compared among the three groups.Results:Among 60 patients (60 eyes), there were 55 males (55 eyes) and 5 females (5 eyes), with the mean age of 17.1±2.0 years. Among 60 eyes, there were 26 eyes in 2B stage, 23 eyes in 3A1 stage, and 11 eyes in 3A2 stage. Twenty patients (20 eyes) was in the laser group, combined group and drug group, respectively. After the initial treatment of all eyes in the drug group, the abnormal blood vessels did not regress significantly; the absorption and increase of SRF were 4 (20.0%, 4/20) and 5 (25.0%, 5/20) eyes, respectively. Supplementary laser therapy was given to 16 eyes, and vitrectomy (PPV) was given to 4 eyes. Among the 16 eyes treated by laser, 10 eyes were effective (50.0%, 10/20); vitreous hemorrhage, fibrous membrane hyperplasia, and complicated cataract occurred in 1, 1, and 2 eyes during the treatment, respectively, and PPV was given again in all eyes. Recurrent and persistent macular edema occurred in 4 and 1 eyes, respectively. Among the eyes in the combined group, treatment were effective in 11 eyes (55.0%, 11/20); 5, 2, and 2 eyes had SRF, fibrous membrane hyperplasia, and complicated cataract during the treatment, and PPV was given again; the edema was repeated and persisted in 1 eye, respectively. Among the affected eyes in the laser group, 15 eyes (75.0%, 15/20) were treated effectively; 2, 2, and 1 eyes developed a large number of vitreous hemorrhage, fibrous membrane hyperplasia, and complicated cataract during the treatment, and PPV was given again.Conclusions:Anti-VEGF drugs alone are ineffective in the treatment of Coats disease, and ablation of other abnormal blood vessels is needed. In the treatment of Coats disease, anti-VEGF drugs can not only promote the absorption of SRF, but also may lead to its increase, and the application should be cautious.
ABSTRACT
Objective:To observe the characteristics of macular telangiectasia (MacTel) in multi-color and multi-mode fundus images.Methods:An abservational case series study was conducted.Sixteen eyes of 12 patients diagnosed with MacTel by fluorescein fundus angiography (FFA) from January to November 2019 in Shandong Eye Hospital were analyzed.There were 8 cases (8 eyes) with MacTel type Ⅰ, among which 4 cases were male and 4 cases were female, with an average age of (62.3±12.5) years.The other 4 cases (8 eyes) had MacTel type Ⅱ, all of which were female, with an average age of (58.7±10.5) years.Best corrected visual acuity, slit lamp microscopy, color fundus photography, multicolor scanning laser imaging, FFA, optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) were carried out in all the patients.This study adhered to the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of Shandong Eye Institute (No.2019S003).Results:In color fundus images of MacTel type Ⅰ eyes, annular macular exudation with macular edema occurred in 6 eyes, macular edema without hard exudates in 1 eye, and hard macular exudates without macular edema in 1 eye.However, the transparency of retina in temporal fovea in MacTel type Ⅱ eyes decreased, showing a gray color.In multi-color fundus images of MacTel type Ⅰ eyes, punctate granular yellow macular exudation and yellow-green macular edema were observed, which were clearer than those in color fundus images.Punctate exudation was seen in both the blue and green reflectance images, which was clearest in green reflectance image, followed by blue reflectance image and then the infrared reflectance image.In OCT images of MacTel type Ⅰ eyes, cystoid edema of inner retina or uneven reflection signal of outer plexiform retina were observed.Loss of inner and outer retinal structures and cavities were observable in MacTel type Ⅱ eyes, and outer retinal atrophy appeared in 2 eyes.In OCTA images, the destruction of superficial and deep capillary plexus in macular area were observed in both MacTel typeⅠand type Ⅱ eyes, and the destruction of deep capillary plexus was more obvious.In addition, more obviously increased vascular space, decreased vessel density, and increased foveal avascular zone were found in MacTel type Ⅱ eyes.In early stage of FFA, delayed capillary filling near fovea was seen in MacTel typeⅠeyes, and dilated temporal vessels in fovea, some of which showed tumor-like dilation, and the limited tumor-like dilation was enhanced in the later stage.Different degrees of dilated parafoveal blood vessels in the early stage, and the capillary in the temporal side of the macula showing diffuse strong fluorescence in the late stage of FFA was observed in MacTel type Ⅱ eyes.Conclusions:Multi-color scanning laser imaging can be used to observe the morphological characteristics of MacTel, and the imaging features of different types of MacTel are significantly different.
ABSTRACT
ABSTRACT We conducted retinal neovascularization under subfoveal fibrotic nodule for Coats disease by using optic coherence tomography-angiography before and after ranibizumab treatment. Our patient was an 8-year-old boy who was referred with suspicious left retinal mass. His visual acuity was 20/400 in the left eye and 20/20 in the right eye at the time of admission. Posterior segment evaluation of the left eye revealed telengiectatic vessels at the inferotemporal region of the peripheral retina with hard exudates around the optic disc and macula typical for Coats disease. His optic coherence tomography revealed a subfoveal fibrotic nodule after ranibizumab injections and laser photocoagulation treatment. The optic coherence tomography-angiography results revealed neovascularization under the subfoveal nodule at the superficial vascular complex layer. After 3 intravitreal ranibizumab injections, his neovascularization regressed on optic coherence tomography-angiography and his visual acuity improved. To the best of our knowledge, this is the first report demonstrating neovascularization under the subfoveal fibrotic nodule in Coats disease on the basis of comparative with the help of optic coherence tomography-angiography before and after the treatment.
RESUMO Demonstramos uma neovascularização da retina sob o nódulo fibrótico subfoveal na doença de Coats com a ajuda da Angiotomografia de Coerência Óptica (OCT-A) antes e após o tratamento com ranibizumabe. Paciente do sexo masculino de 8 anos foi encaminhado com suspeita de massa retiniana no olho esquerdo. A acuidade visual foi de 20/400 no olho esquerdo e de 20/20 no olho direito. A avaliação do segmento posterior do olho esquerdo revelou vasos telengiectáticos na região inferotemporal da retina periférica e exsudados duros em torno do disco óptico e mácula típica da doença de Coats. A angiotomografia de coerência óptica apresentou nódulo fibrótico subfoveal após injeções de ranibizumabe e tratamento com fotocoagulação a laser. A angiotomografia de coerência óptica mostrou neovascularização sob o nódulo subfoveal na camada superficial do complexo vascular. Após três injeções de ranibizumabe intravítreo, a neovascularização regrediu na angiografia por tomografia de coerência óptica e a acuidade visual melhorou. onde sabemos, este é o primeiro relato a mostrar neovascularização sob nódulo fibrótico subfoveal na Doença de Coats com a ajuda da angiografia por tomografia de coerência óptica antes e após o tratamento.
ABSTRACT
Objective:To observe the effect of local retinal laser photocoagulation (local photocoagulation) on Coats disease.Methods:A retrospective clinical study. From January 1, 2006 to August 1, 2020, 48 patients (48 eyes) who were diagnosed as Coats disease and received focal photocoagulation at The Eye Hospital Affiliated to Wenzhou Medical University were included in the study. Among them, there were 40 males (40 eyes) and 8 females (8 eyes). The average age was 32.46±22.41 years old. Nine eyes were in stage 2A, and 39 eyes were in stage 2B. All affected eyes underwent best corrected visual acuity (BCVA), color fundus photography and fluorescein fundus angiography (FFA).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 during statistics. According to age, patients were divided into the adolescent group (age≤ 20 years old) and the adult group (age> 21 years old), with 18 eyes in 18 cases and 30 eyes in 30 cases, respectively. In the adolescent group, 18 eyes of 18 cases were all male; the average age was 11.17±3.31 years; the average logMAR BCVA was 0.83±0.60. Among the 30 patients in the adult group, 22 patients were male and 8 patients were female. the average age was 49.26±15.26 years old; the average logMAR BCVA was 0.82±0.59. All the affected eyes were treated with focal photocoagulation. Laser parameters were as followed: wavelength 577 nm, spot diameter 500 μm, exposure time 0.5 to 0.7 s, spot intensity level Ⅲ. FFA was FFA was performed 3 to 4 months after the first laser photocoagulation, and laser treatment was repeated as needed. The average follow-up after first treatment was 36.88±36.92 months. The changes in BCVA, abnormal blood vessels in the fundus, and hard exudation were observed.Results:Among 48 eyes, 36 eyes (75.00%, 36/48) received multiple local photocoagulation treatments. At the last follow-up, 36 eyes (75.00%, 36/48) had an improved or stable vision, and 17 eyes (35.42%, 17/48) had BCVA ≤ 0.32 logMAR units (≥ 0.5). The average logMAR BCVA of eyes in the adolescent group was 0.66±0.54, which was higher than the baseline, but the difference was not statistically significant ( Z=-1.126, P=0.260). The average logMAR BCVA of the eyes in the adult group was 0.96±0.79, which was lower than the baseline, but the difference was not statistically significant ( Z=-0.482, P=0.630). Among 48 eyes, abnormal blood vessels were completely or partially occluded in 42 eyes (87.50%, 42/48); of which, 29 eyes were completely occluded (60.42%, 29/48), and 13 eyes were partially occluded (27.08%, 13/48)). The hard exudation at macula or peripheral retina were completely absorbed or obviously absorbed in 40 eyes (83.33%, 40/48); among them, the complete and obvious absorption were 11 (22.92%, 11/48) and 29 (60.42%, 29/48) eyes. Conclusion:The treatment of focal photocoagulation with a larger spot, long exposure and weak level Ⅲ spot can effectively seal abnormal blood vessels in the eyes of Coats disease,reduce hard exudation and improve or stabilize vision.
ABSTRACT
Objective:To observe the imaging characteristics of optical coherence tomography angiography in macular telangiectasia type 2 (Mac-Tel 2).Methods:A retrospective case analysis. From October 2017 to June 2021, 11 patients (22 eyes) diagnosed as Mac-Tel type 2 by multi-modal imaging in Nanjing Medical University Eye Hospital were included in this study. There were 5 males (10 eyes) and 6 females (12 eyes). The age were 41.61±11.32 years old. All patients underwent the examinations of best corrected visual acuity, indirect ophthalmoscope, fundus color photography, fluorescein fundus angiography (FFA), optical coherence tomography (OCT), and OCT angiography (OCTA). The scope of 3 mm × 3 mm in macular area of eyes was scanned by OCTA. After automatic image processing, the system could provide the blood flow image of capillary layer, deep capillary layer, outer retina, choroidal capillary layer, and the B-scan image. The imaging characteristics were observed.Results:Among the 22 eyes, 14 eyes were in the early stage of the disease, and 8 eyes had secondary subretinal neovascularization (SRN) and/or choroidal neovascularization (CNV). FFA examination that in the early stage of the disease, the capillaries near the fovea were dilated, the blood vessels were stretched, and the late fluorescence was mainly stained; high-fluorescence leakage was seen when SRN and CNV were developed. OCTA examination showed that in the early stage of the disease, the temporal capillaries in the macular area were dilated and stretched, especially in the deep layer. The capillary space was enlarged, and the right-angled venules were seen to change and infiltrate into the deep layer; when the lesions invaded the outer retina, flower clusters-like SRN were seen; neovascularization was seen in the outer retina and choroidal capillary layer when CNV was developed. B-scan image showed that in the early stage of the disease, irregular weak reflex cavities and lamellar holes change between the neuroepithelial layers; secondary SRN and CNV showed strong little clumpy reflexes accompanied by abundant blood flow signals.Conclusion:The image characteristics of OCTA in the eyes of Mac-Tel 2 were dilated, stretched superficial and deep temporal capillaries in the macula area and right-angled changes in blood vessels.
ABSTRACT
PURPOSE: To report a rare case of bilateral occlusive juxtafoveolar retinal telangiectasias associated with central nervous system lesions and renal impairment. OBSERVATIONS: A 47-year-old woman presented to clinic with subjective vision loss in the right eye with best-corrected visual acuity (BCVA) 20/80. Fundoscopic examination, fluorescein angiography (FA), and optical coherence tomography with adjunct angiography (OCT/OCT-A) revealed macular microhemorrhages, enlarged foveal avascular zones (FAZ), and occlusive juxtafoveal telangiectasis with pruning of the macular capillaries in both eyes. Patient subsequently developed memory loss, dizziness, nystagmus, and diplopia secondary to intermittent exotropia. She was found to have a two-millimeter aneurysm of the proximal posterior cerebellar artery along with several scattered white matter changes on brain magnetic resonance imaging (MRI). Genetic workup revealed no mutations in the TREX1 gene. With continued surveillance over 18 months, the patient's BCVA deteriorated to 20/200 OU and she developed mild renal impairment, without further CNS complications. CONCLUSION AND IMPORTANCE: Patients who present with vision loss secondary to occlusive juxtafoveolar telangiectasias should undergo imaging of the central nervous system (CNS) for architectural abnormalities in cerebral vasculature and white matter. Further investigation of patients with the Gass-Blodi type 3 macular telangiectasia - cerebroretinal vasculopathy phenotype is required to optimize management protocols for both retinal and CNS lesions. At this time, no interventions have demonstrated clear benefit in vision preservation or recovery.
ABSTRACT
BACKGROUND: Coats disease is an idiopathic exudative outer retinopathy caused by abnormal retinal vascular development. AIM: To evaluate the long-term outcomes of intravitreal conbercept injection with laser photocoagulation as a treatment for Coats disease in adults. METHODS: This retrospective case series study included patients diagnosed with Coats disease and treated with intravitreal conbercept injection and 532-nm laser photocoagulation at the Ophthalmology Department of Shenzhen People's Hospital between January 2016 and January 2017. Best-corrected visual acuity (BCVA) measurements, noncontact tonometry, ophthalmoscopy, fundus photography, fundus fluorescein angiography and optical coherence tomography were performed before treatment and at 1 wk, 1 mo, 3 mo, 6 mo, 9 mo, 12 mo, 24 mo and 36 mo after therapy. Best-corrected visual acuity was measured using the early treatment of diabetic retinopathy study chart. RESULTS: The study included eight eyes of 8 patients (7 men) aged 36.10 ± 6.65 years. The average BCVA of the affected eye before treatment was 51.17 ± 15.15 letters (range, 28-70 letters), and the average central macular thickness was 303.30 ± 107.87 µm (range, 221-673 µm). Four eyes were injected once, three were injected twice, and one was injected three times. Average follow-up duration was 37.33 ± 2.26 mo. Average BCVA of the affected eye was 51.17 ± 15.15 letters before treatment and was increased by 13.50 ± 3.20, 16.25 ± 7.73, 18.25 ± 8.96, 18.03 ± 5.27, 18.63 ± 3.35, 19.75 ± 6.96, 18.05 ± 5.36 and 17.88 ± 3.45 letters at 1 wk, 1 mo, 3 mo, 6 mo, 9 mo, 12 mo, 24 mo and 36 mo after treatment, respectively (P < 0.01). The patients showed varying degrees of subretinal fluid resorption after treatment. None of the patients had serious complications such as increased intraocular pressure, development/progression of cataracts, endophthalmitis or retinal detachment. CONCLUSION: Intravitreal injection of conbercept combined with 532-nm laser photocoagulation may be a feasible treatment for Coats disease in adult patients.
ABSTRACT
Macular telangiectasia type 2 (MacTel 2) is an idiopathic entity which is characterized by dilated capillary network near, or around, the fovea of the macula. The spectrums secondary to macular retinal vascular inflammation or obstruction are excluded. It is a binocular disease in general. MacTel 2 is now widely recognized as a macular neurodegenerative disease rather than a simple vascular disease. This article reviews the epidemiological features, clinical features, staging, special examinations, pathogenesis and treatment of MacTel 2. (Chin J Ophthalmol, 2019, 55:68-73).
Subject(s)
Diabetic Retinopathy , Retinal Telangiectasis , Fluorescein Angiography , Humans , Retinal Telangiectasis/diagnosis , Retinal Telangiectasis/therapy , Tomography, Optical CoherenceABSTRACT
Ophthalmological abnormalities in facioscapulohumeral dystrophy may lead to treatable vision loss, facilitate diagnostics, could help unravelling the pathophysiology and serve as biomarkers. In this study, we provide a detailed description of the ophthalmological findings in a well-defined cohort of patients with facioscapulohumeral dystrophy using state of the art retina imaging techniques. Thirty-three genetically confirmed patients (aged 7-80 years) and 24 unrelated healthy controls (aged 6-68 years) underwent clinical ophthalmological examination, fundus photography, optical coherence tomography/angiography, genotyping and neurological examination. All patients had normal corrected visual acuity and normal intraocular pressure. In 27 of the 33 patients, weakness of the orbicularis oculi was observed. Central retinal pathology, only seen in patients and not in healthy controls, included twisting (tortuosity) of the retinal arteries in 25 of the 33 patients and retinal pigment epithelium defects in 4 of the 33 patients. Asymmetrical foveal hypoplasia was present in three patients, and exudative abnormalities were observed in one patient. There was a correlation between the severity of retinal tortuosity and the D4Z4 repeat array size (R 2 = 0.44, P < 0.005). Follow-up examination in a subgroup of six patients did not show any changes after 2 years. To conclude, retinal abnormalities were frequent but almost always subclinical in patients with facioscapulohumeral dystrophy and consisted primarily of arterial tortuosity and foveal abnormalities. Retinal tortuosity was seen in the retinal arterioles and correlated with the D4Z4 repeat array size, thereby providing clinical evidence for an underlying genetic linkage between the retina and facioscapulohumeral dystrophy.
ABSTRACT
PURPOSE: We report the detection of peripheral retinal vascular abnormalities in the fellow eye with normal fundus in children with unilateral Coats' disease. METHODS: The clinical records of patients diagnosed with Coats' disease were retrospectively reviewed. We recorded the subjects' characteristics and obtained fundus photography and fluorescein angiography (FA) images. The main outcome measure was peripheral vascular abnormalities in the contralateral eye with normal fundus in children with unilateral Coats' disease, observed with FA. RESULTS: Out of 47 patients with Coats' disease, two (4.3%) were diagnosed with clinically bilateral Coats' disease. Of the 45 patients with presumed unilateral Coats' disease, four (8.9%) had bilateral abnormal peripheral vasculature in FA. The mean age of these four patients was 6.4 ± 5.4 years (range, 1 to 14 years), and three patients were male (75%). All four had peripheral retinal nonperfusion, and two (50%) received laser photocoagulation due to peripheral leakage with telangiectatic vessels. CONCLUSIONS: Coats' disease may more often be a bilateral disease with asymmetry than previously thought. Patients with Coats' disease should undergo careful examination of the fellow eye with FA in order to detect and treat vascular abnormalities that are not visible clinically.
Subject(s)
Fluorescein Angiography , Retinal Telangiectasis/diagnosis , Retinal Vessels/pathology , Adolescent , Child , Child, Preschool , Female , Fundus Oculi , Humans , Infant , Laser Coagulation , Male , Retinal Telangiectasis/surgery , Retinal Vessels/surgery , Retrospective Studies , Visual Acuity/physiologyABSTRACT
Retinopathy ofprematurity,familial exudative vitreoretinopathy and Coats disease are the most common neonates and infants retinal vascular diseases,which may lead to severe visual damage because of either tractional retinal detachment caused by the proliferation of pathogenic neovascularization,or exudative retinal detachment due to the extremely leakage from abnormal retinal vessels.Classic treatment is retinal laser photocoagulation which could destroy these abnormal vessels or reduce non vascular areas to diminish the growth of new vessels,however the side effects induced by laser it self such as visual field damage,hemorrhage,retinal tear,fail to control the progression of the disease make the laser treatment hard to improve the vision of these young patients.Anti-vascular endothelial growth factor (VEGF) agents have been widely applied in various adult retinal and choroidal vascular diseases,they are even possible to replace the pan retinal photocoagulation in proliferative diabetic retinopathy,while there are still many unsolved problems in the applying in neonates and infants retinal vascular diseases,like dosage,timing,retreatment and systemic side effects.We should realize the importance of selecting the laser photocoagulation and anti-VEGF for neonates and infants retinal vascular diseases.
ABSTRACT
Adult Coats disease is characterized by abnormal expansion of retinal capillaries,often accompanied by massive lipid exudation and exudative retinal detachment.Unlike Coats disease in young children,adult Coats disease is mostly limited to peripheral retina,with slow progress and better prognosis.Adult Coats disease should be identified with Coats-like diseases such as exudative age-related macular degeneration,diabetic retinopathy,obsolete retinal vein occlusion,idiopathic macular telangiectasia 1,obsolete posterior uveitis,retinal vasculitis,or acute retinal necrosis.Because the pathogenesis of Coats disease is not clear,it lacks specific treatment measures for the cause of disease.The purpose of simple or combined laser photocoagulation,freezing,vitreous intravitreal injection against vascular endothelial growth factor drugs or triamcinolone and surgery is to eliminate abnormal blood vessels and exudation,maintain visual function,which can also improve retinal detachment and prevent neovascular glaucoma and other complications.To explore the similarities and differences of adult Coats disease with Coats disease in young children,to further promote the study of the pathogenesis of adult Coats disease and to provide new targets for its treatment are the direction of future research.
ABSTRACT
PURPOSE: We report the detection of peripheral retinal vascular abnormalities in the fellow eye with normal fundus in children with unilateral Coats' disease. METHODS: The clinical records of patients diagnosed with Coats' disease were retrospectively reviewed. We recorded the subjects' characteristics and obtained fundus photography and fluorescein angiography (FA) images. The main outcome measure was peripheral vascular abnormalities in the contralateral eye with normal fundus in children with unilateral Coats' disease, observed with FA. RESULTS: Out of 47 patients with Coats' disease, two (4.3%) were diagnosed with clinically bilateral Coats' disease. Of the 45 patients with presumed unilateral Coats' disease, four (8.9%) had bilateral abnormal peripheral vasculature in FA. The mean age of these four patients was 6.4 ± 5.4 years (range, 1 to 14 years), and three patients were male (75%). All four had peripheral retinal nonperfusion, and two (50%) received laser photocoagulation due to peripheral leakage with telangiectatic vessels. CONCLUSIONS: Coats' disease may more often be a bilateral disease with asymmetry than previously thought. Patients with Coats' disease should undergo careful examination of the fellow eye with FA in order to detect and treat vascular abnormalities that are not visible clinically.
Subject(s)
Child , Humans , Male , Fluorescein Angiography , Fluorescein , Light Coagulation , Outcome Assessment, Health Care , Photography , Retinal Telangiectasis , Retinaldehyde , Retrospective StudiesABSTRACT
BACKGROUND: To study the interaction between idiopathic macular telangiectasis type 2 (MacTel2) and coexistent diabetic retinopathy (DR) during long term follow up. METHODS: A retrospective chart review was done for all eyes with MacTel2 and DR with a minimum 2 years follow up. Eyes with other retinal disorders and poor quality imaging were excluded. Data collected included demographics, presenting visual acuity, systemic evaluation, treatments done, duration of follow up, and final visual outcomes. RESULTS: Out of 951 patients with MacTel2, 277 patients had diabetes. Out of 277 patients, 44 eyes of 22 patients had MacTel2 coexisting with DR. Twenty-eight eyes of 14 patients were included in this study. All cases of MacTel2 were bilateral with a preponderance of women (71.42%). Mean follow up was 93.07 ± 84.03 months with a mean random blood sugar level of 135.41 ± 45.47 mg% at presentation. Twenty-five (89.28%) eyes presented with mild non-proliferative diabetic retinopathy. Two (7.14%) eyes progressed in their DR staging from baseline. Stage III MacTel2 were noted in 11 (39.28)% eyes at baseline. None of these eyes progressed to stage V during follow up. Mean presenting logMAR BCVA was 0.214 ± 0.227 which dropped to 0.399 ± 0.301 at last visit (p = 0.0005). Diabetic macula edema (DME) was not noted in any eye till last follow up. CONCLUSIONS: 12.5% of eyes with MacTel2 in diabetic patients had coexistent DR. MacTel2 led to slowly progressive visual loss irrespective of the presence of DR.
ABSTRACT
Coats disease is a relatively rare and idiopathic disorder characterized by retinal telangiectasia and massive intra-retinal and (or) sub-retinal lipid accumulation, resulting in complications including retinal detachment and neovascular glaucoma. Previous reports have revealed that Coats disease can be associated with other disorders, especially some inherited diseases, such as retinitis pigmentosa (RP) and facioscapulohumeral muscular dystrophy (FSHD). Coats disease associated with other inherited disorders is generally called Coats-like retinopathy, which has some unique features that differs from the classic Coats disease, for example there is no sex and age preference, more bilateral cases, more severe cases and more genetic factors involved. Patients of Coats-like retinopathy with RP and FSHD may have mutations in Crumbs homologue gene 1 and D4Z4 genes.
ABSTRACT
A 1-year-old male Indian rhesus macaque presented with a bilateral blindness. Ocular examination, gross and histopathological evaluation, and immunohistochemistry were performed. The major findings were retinal telangiectasia, accumulation of exudate in the intraretinal and subretinal space, and retinal detachment. Coat-like retinopathy was diagnosed, and it has not been previously reported in veterinary medicine.
Subject(s)
Macaca mulatta , Monkey Diseases/diagnosis , Retinal Detachment/veterinary , Retinal Telangiectasis/veterinary , Animals , Exudates and Transudates/metabolism , Male , Retinal Detachment/diagnosis , Retinal Telangiectasis/diagnosisABSTRACT
Objective To observe the efficacy of adjuvant intravitreal injection of anti-vascular endothelial growth factor (VEGF) therapy for advanced Coats disease.Methods This study is a retrospective case series study.Fourteen patients (14 eyes),presenting Coats Stages 3B and 4 (8 and 6 eyes,respectively) were enrolled.All the patients were treated with adjuvant intravitreal anti-VEGF therapy.The intravitreal anti-VEGF injections varied from 1 to 7,with a median injections of 2.14.In 14 eyes,combined therapy was subretinal fluid drainage in 4 eyes,photocoagulation in 2 eyes,vitrectomy in 8 eyes.The follow-up period was ranged from 4 to 36 months,with a median follow-up of 18.8 months.Visual acuity and retinal reattachment were observed in follow up.Results At last follow up,global suvival was 100.0％ with no enucleation performed in any patient because of disease progression.Except for 2 children who were unable to cope with the visual acuity test,visual acuity was improved in 2 patients,stable in 8 patients,and decreased in 2 patients.5 patients (35.7％) achieved in complete retinal reattachment,3 patients (21.4％) were succeed in partial retinal reattachment,and the remain 6 patients(42.8％) failed in retinal reattachment.Two patients developed cataract after vitrectomy,and no other adverse reaction was observed during follow-up.Conclusion Anti-VEGF therapy combined with classic treatments in advanced Coats disease can keep or impove the visual acuity in most patients by reducing of subretinal exudation.
