Coats’ disease of adult‑onset in 48 eyes.

Background: Coats’ disease diagnosed in adulthood is an idiopathic, retinal exudative vascular disease without an inciting factor and has retinal features different from the childhood disease. Aim: To describe clinical features, treatment, and outcomes of eyes with Coats’ disease first diagnosed in patients 35 years or older. Materials and Methods: Retrospective chart review of patients first diagnosed with Coats’ disease at the age of 35 years or more at a tertiary eye care center between January 1995 and 2012. Eyes with retinal exudation or Coats’‑like response from secondary causes were excluded. Results: Forty‑five of 646 patients (7%) diagnosed with Coats’ disease had adult‑onset disease. Mean age at presentation was 47 years. Systemic hypertension was the most common (22%) systemic association and decreased vision the predominant presenting feature (83%). Localized (<6 clock h) presentation (74%) was unique to adults as against diffuse involvement (69%) in children (P < 0.001). Eyes were treated with laser photocoagulation 29 (60%), cryotherapy (4%), or both (2%) with surgical intervention in three (6%) eyes. Following treatment eight (35%) eyes improved, 11 (48%) eyes were stable while four (12%) eyes worsened due to complications. Conclusion: Adult‑onset Coats’ disease has less extensive involvement, more benign natural course, and a more favorable treatment outcome as against the childhood‑onset disease. The bilateral presentation emphasizes the need for regular follow‑up to detect possible future involvement of the fellow eye.

Enfermedad de Coats, presentación de un caso y revisión de la literatura / Coats Disease, case report and literatura review

La enfermedad de Coats, es una vasculopatía retiniana idiopática que sin tratamiento puede progresar a desprendimiento de retina exudativo y glaucoma neovascular. Se considera una patología de difícil diagnóstico, por su presentación clínica que simula otras vasculopatías retinianas ó retinoblastoma. Resulta indispensable contar con herramientas clínicas y paraclínicas para llegar a un diagnóstico acertado y poder realizar un manejo adecuado y oportuno. Presentamos el caso de un niño de 17 años, con enfermedad de Coats valorado y tratado en el Hospital Militar Central de Bogotá desde los 5 años de edad; Se realiza seguimiento clínico de 12 años de evolución y se discuten posibles diagnósticos diferenciales.

Coats disease is an idiopathic retinal vasculopathy that can progress to exudative retinal detachment and neovascular glaucoma if is not treated. It is considered a condition of difficult diagnosis due to clinical presentation, simulating retinoblastoma or other retinal vascular disease. It is essential to have clinical and paraclinical tools to have a correct diagnosis and to make adequate and timely handling. We report the case of a child of 17 years old with Coats disease assessed and treated at the Central Military Hospital in Bogota from 5 years of age during 12 years. Th e clinical evolution is described and a brief depiction of the disease is presented with its diff erential diagnoses.

Multimodality imaging in macular telangiectasia 2: A clue to its pathogenesis.

Macular telangiectasia type 2 also known as idiopathic perifoveal telangiectasia and juxtafoveolar retinal telangiectasis type 2A is an acquired bilateral neurodegenerative macular disease that manifests itself during the fifth or sixth decades of life. It is characterized by minimal dilatation of the parafoveal capillaries with graying of the retinal area involved, a lack of lipid exudation, right‑angled retinal venules, refractile deposits in the superficial retina, hyperplasia of the retinal pigment epithelium, foveal atrophy, and subretinal neovascularization (SRNV). Our understanding of the disease has paralleled advances in multimodality imaging of the fundus. Optical coherence tomography (OCT) images typically demonstrate the presence of intraretinal hyporeflective spaces that are usually not related to retinal thickening or fluorescein leakage. The typical fluorescein angiographic (FA) finding is a deep intraretinal hyperfluorescent staining in the temporal parafoveal area. With time, the staining may involve the whole parafoveal area but does not extend to the center of the fovea. Long‑term prognosis for central vision is poor, because of the development of SRNV or macular atrophy. Its pathogenesis remains unclear but multimodality imaging with FA, spectral domain OCT, adaptive optics, confocal blue reflectance and short wave fundus autofluorescence implicate Müller cells and macular pigment. Currently, there is no known treatment for this condition.

Demographic Features of Idiopathic Macular Telangiectasia in Korean Patients

PURPOSE: To investigate the clinical and demographic features of idiopathic macular telangiectasia (MacTel) in Korean patients since the introduction of spectral domain optical coherence tomography (SD-OCT). METHODS: We reviewed medical records of patients who were diagnosed with MacTel from 2009 to 2013. All patients underwent fluorescein angiography and SD-OCT and were classified as type 1 or type 2 according to the classification system proposed by Yannuzzi. RESULTS: Over a period of 5 years, 4 (18.2%) patients were diagnosed with type 1 MacTel and 18 (81.8%) patients were diagnosed with type 2 MacTel. All patients with type1 MacTel were male, and their mean age was 51 +/- 8.6 years. Among patients with type 2 MacTel, 3 (16.7%) were male, 15 (83.3%) were female, and the mean age was 60 +/- 13.6 years. Whereas all type 1 MacTel patients had either metamorphopsia or mild scotoma, of the 18 patients with type 2 MacTel, only 4 (22.2%) had those symptoms, 10 (55.6%) complained of only mild visual impairment, and the other 4 (22.2%) had no symptoms. Intraretinal cystoid spaces were observed in 26 (72.2%) of 36 eyes with type 2 MacTel by SD-OCT. These cystoid spaces had irregular boundaries and did not correspond to angiographic leakages. CONCLUSIONS: Type 2 MacTel was most common in the present study. The wider availability of SD-OCT may have contributed to the diagnosis of type 2 MacTel. Type 2 MacTel may be more prevalent than type 1 in Koreans, which corresponds to the results of Western countries.

Demographic Features of Idiopathic Macular Telangiectasia in Korean Patients

PURPOSE: To investigate the clinical and demographic features of idiopathic macular telangiectasia (MacTel) in Korean patients since the introduction of spectral domain optical coherence tomography (SD-OCT). METHODS: We reviewed medical records of patients who were diagnosed with MacTel from 2009 to 2013. All patients underwent fluorescein angiography and SD-OCT and were classified as type 1 or type 2 according to the classification system proposed by Yannuzzi. RESULTS: Over a period of 5 years, 4 (18.2%) patients were diagnosed with type 1 MacTel and 18 (81.8%) patients were diagnosed with type 2 MacTel. All patients with type1 MacTel were male, and their mean age was 51 +/- 8.6 years. Among patients with type 2 MacTel, 3 (16.7%) were male, 15 (83.3%) were female, and the mean age was 60 +/- 13.6 years. Whereas all type 1 MacTel patients had either metamorphopsia or mild scotoma, of the 18 patients with type 2 MacTel, only 4 (22.2%) had those symptoms, 10 (55.6%) complained of only mild visual impairment, and the other 4 (22.2%) had no symptoms. Intraretinal cystoid spaces were observed in 26 (72.2%) of 36 eyes with type 2 MacTel by SD-OCT. These cystoid spaces had irregular boundaries and did not correspond to angiographic leakages. CONCLUSIONS: Type 2 MacTel was most common in the present study. The wider availability of SD-OCT may have contributed to the diagnosis of type 2 MacTel. Type 2 MacTel may be more prevalent than type 1 in Koreans, which corresponds to the results of Western countries.

Clinical Features of Idiopathic Juxtafoveal Telangiectasis in Koreans

PURPOSE: To describe the clinical characteristics of idiopathic juxtafoveal telangiectasis (IJT) in Koreans. METHODS: Medical records of 16 patients with IJT were analyzed during the period from 1997 to 2009. Diagnosis was based on biomicrosopic and fluorescein angiographic findings and the group was determined according to the Gass and Blodi classification. RESULTS: We analyzed eight patients in group 1A (50%), two in group 1B (12.5%), and six in group 2A (37.5%). Diverse treatment modalities, such as macular laser photocoagulation, photodynamic therapy, intravitreal antiangiogenic agent, and steroid injection, were applied for macular edema in nine eyes; however, only two eyes showed visual improvement. CONCLUSIONS: In this case series, group 1A was the most common. For macular edema related to IJT, current treatment strategies had no consistent effect.

Bilateral Coats' Disease: A Case Report

PURPOSE: To report a case of bilateral Coats' disease. CASE SUMMARY: A 19-month-old boy presented with esodeviation of his eyes, which started 5 months prior. A fundus exam showed total bullous exudative retinal detachment with retinal vascular telangiectasia in the right eye and localized exudative retinal detachment with vascular telangiectasia at the inferior periphery in the right eye. Fluorescein angiogram of the left eye showed retinal telangiectatic vessels, avascular area and fluorescein leakeage from telangiectatic vessels. The patient received external drainage of subretinal fluid and intravitreal air injection of the right eye and Argon LASER photocoagulation and cryotheraphy of the left eye. A cytologic exam of the subretinal fluid drained from the right eye showed no malignant cells. Forty-four months after the operation, his best corrected visual acuity was no light perception in the right eye and 0.4 in the left eye. Both fundi were flat and stable. No complications, such as glaucoma, recurred retinal detachment, or pain, occurred. CONCLUSIONS: Coats' disease rarely occurs bilaterally and can be involved asymmetrically. The disease presents more severely when bilateral and can progress after long-term observation. Proper treatment and long-term follow-up of both eyes are necessary to prevent visual loss and preserve eyes.

Coats' disease: An Indian perspective.

Aim: To describe the clinical features, treatment and outcome patterns in 307 eyes with Coats' disease. Materials and Methods: Retrospective chart review of patients diagnosed with Coats' disease between January 1996 and January 2006 from a single referral center in southern India. Results: Two hundred and eighty patients (307 eyes) with mean age of 15.67 years (range: Four months-80 years) were included. Decreased vision (77%), unilateral affection (90%) and male preponderance (83.4%) were chief presenting features. Anterior segment involvement was seen in 67 (21.8%) eyes. Retinal telangiectasia were seen in 302 (99%) eyes, exudation in 274 (89%) eyes and retinal detachment in 158 (51.5%) eyes. Four-quadrant disease was seen in 207 (67.2 %) eyes. Visual acuity was < 20/200 in 249 (80.9%) eyes. One hundred and nine of 176 treated eyes (61.93%) had favorable anatomical outcome; 207 of 280 eyes (74%) had an optimal structural outcome. Seventeen (5.3%) eyes were enucleated. Complications following treatment included phthisis bulbi (7%), neovascular glaucoma (5%), epiretinal membrane (4.4%) and rubeosis iridis (4.4%). Conclusion: Indian patients with Coats' disease have a high male predominance, the majority of whom present with severe visual impairment and extensive four-quadrant exudation. Unusual presentations such as pain, vitreous hemorrhage and a high incidence of anterior segment involvement are distinctive to Indian eyes.