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Journal ArticleDOI

Enfermedad de coats recidivante diagnosticada en sujeto adulto

01 Sep 2007-Vol. 82, Iss: 9, pp 555-558

TL;DR: The male diagnosed with Coats disease at the age of 30 years, had a relapse of this condition 14 years later and similar findings of unilateral retinal exudation and lipid deposition at boundary were seen.

AbstractespanolCaso clinico: Paciente varon que con 30 anos tuvo el primer episodio de enfermedad de Coats y recurrencia 14 anos mas tarde. Clinicamente ambos episodios tienen como sintoma de inicio vision borrosa y exploracion tipica de exudacion masiva retiniana con corona de exudados duros. La angiografia presenta hallazgos caracteristicos de telangiectasia retiniana con dilatacion anormal de vasos con hiperfluorescencia precoz y fuga tardia. Discusion: Coats es una entidad clinica poco frecuente y con grado de severidad variable, que hacen dificiles los estudios randomizados prospectivos. Pese a los hallazgos caracteristicos, hay que descartar otros diagnosticos tanto en el nino como en el adulto causantes de esa misma clinica. EnglishCase report: A male diagnosed with Coats disease at the age of 30 years, had a relapse of this condition 14 years later. His first symptom of blurred vision occurred in both episodes and similar findings of unilateral retinal exudation and lipid deposition at boundary were seen. Angiography showed the characteristic early hyperfluorescence of the telangiectasias and late leakage of dye. Discussion: Coats disease is relatively uncommon and, as the degree of involvement varies from case to case, a randomized, prospective study of treatment would probably not be feasible. Despite the characteristic features of this disease, the differential diagnoses must be considered in both children and adults

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Journal ArticleDOI
TL;DR: The bilateral presentation emphasizes the need for regular follow-up to detect possible future involvement of the fellow eye and has less extensive involvement, more benign natural course, and a more favorable treatment outcome as against the childhood-onset disease.
Abstract: 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.

20 citations


Journal ArticleDOI
TL;DR: An 18-year-old Caucasian male with a unilateral macular star and retinal vascular anomalies compatible with adult onset Coats’ disease is presented, which is usually diagnosed in childhood, but rare cases may occur in adults.
Abstract: Purpose: We present the case of an 18-year-old Caucasian male with a unilateral macular star and retinal vascular anomalies compatible with adult onset Coats’ disease Methods: Diagnosis was based on fundoscopic, fluorescein angiography and optical coherence tomography findings Results: The patient presented to our emergency department with complaints of low vision in his left eye (LE) detected 10 days before The best-corrected visual acuity in the LE was 20/50 Fundoscopy of the LE evidenced a complete macular star Optical coherence tomography showed increased retinal thickness, infiltration of the retinal wall, and detachment of the neuroepithelium Angiography revealed no appreciable diffusion in the macula Above the superior temporal (ST) arcade, anomalies in the retinal vasculature were found, with interruption of the peripheral vessels and vessels which were ‘sausage’-like After 1 month, the LE vision evolved to hand movements Laser photocoagulation was performed in the ST quadrant Intravitreal injection of bevacizumab 125 mg/005 ml and photodynamic therapy were performed without any significant changes, progression of ST serous detachment of the neuroepithelium, and finally progression to macular fibrosis Discussion: Coats’ disease is usually diagnosed in childhood, but rare cases may occur in adults Those cases usually have a more indolent course which was not observed in our patient When there is macular involvement, prognosis is more guarded, despite treatment

9 citations


References
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Journal ArticleDOI
TL;DR: Carefully selected treatment can anatomically stabilize or improve the eye with Coats disease in 76% of eyes, however, poor visual outcome of 20/200 or worse commonly results, and patients with stages 4 and 5 have a poor visual prognosis.
Abstract: PURPOSE: To review the methods and results of management in a large series of patients with Coats disease, to determine risk factors for poor visual outcome and enucleation, and to propose a practical classification of Coats disease. METHODS: In a retrospective consecutive series in 150 patients, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with management, including observation, laser photocoagulation, cryotherapy, and various techniques of retinal detachment surgery and enucleation. The anatomic outcome, complications of treatment, visual results, and reasons for enucleation were tabulated. Factors predictive of poor visual outcome (20/200 or worse) and enucleation were determined using Cox proportional hazards regression models. Based on these observations, a staging classification of Coats disease, applicable to treatment selection and ocular prognosis, is proposed. RESULTS: In 117 patients (124 eyes) with a mean follow up of 55 months (range, 6 months to 25 years) primary management was observation in 22 eyes (18%), cryotherapy in 52 (42%), laser photocoagulation in 16 (13%), various methods of retinal detachment surgery in 20 (17%), and enucleation in 14 (11%). Anatomic improvement or stability was achieved in 76% of eyes, and final visual acuity was 20/50 or better in 17 eyes (14%), 20/60 to 20/100 in eight (6%), 20/200 to finger counting in 30 (24%), and hand motion to no light perception in 49 (40%) Enucleation was ultimately necessary in 20 eyes (16%). Risk factors predictive of poor visual outcome (20/200 or worse) included postequatorial ( P = .01), diffuse ( P = .01), or superior ( P = .04) location of the telangiectasias and exudation, failed resolution of subretinal fluid after treatment ( P = .02), and presence of retinal macrocysts ( P = .02). The main risk factors for enucleation were elevated intraocular pressure (greater than 22 mm Hg; P less than or equal to .001) and iris neovascularization ( P less than or equal to .001). Coats disease was classified into stage 1, telangiectasia only; stage 2, telangiectasia and exudation (2A, extrafoveal exudation; 2B, foveal exudation) stage 3, exudative retinal detachment (3A, subtotal; 3B, total); stage 4, total detachment and secondary glaucoma; and stage 5, advanced end-stage disease. Poor visual outcome (20/200 or worse) was found in 0% of eyes with stage 1, 53% with stage 2, 74% with stage 3, and 100% of stages 4 and 5 Coats disease. Enucleation was ultimately necessary in 0% of stages 1 and 2, 7% of stage 3, 78% of stage 4, and 0% of stage 5 disease. CONCLUSIONS: Carefully selected treatment can anatomically stabilize or improve the eye with Coats disease in 76% of eyes. However, poor visual outcome of 20/200 or worse commonly results. Patients who present with stages 1 to 3 Coats disease have the best visual prognosis, and patients with stages 4 and 5 have a poor visual prognosis.

284 citations


"Enfermedad de coats recidivante dia..." refers background in this paper

  • ...Shields clasificó la enfermedad atendiendo al tipo y localización de las lesiones (2)....

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  • ...Suele diagnosticarse en la primera década de la vida (1,2)....

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Journal ArticleDOI
TL;DR: Coats disease is a distinct clinical entity characterized by idiopathic retinal telangiectasia and retinal exudation that is usually unilateral, occurs mostly in young males, and can cause severe visual loss resulting from exudative retinal detachment.
Abstract: PURPOSE: The purpose of this report is to review the clinical variations and natural course of Coats disease, using strict diagnostic guidelines. METHODS: In a retrospective, consecutive series, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with the clinical features, complications, and diagnostic approaches to Coats disease. RESULTS: In 150 consecutive patients (158 eyes), Coats disease was diagnosed at a median age of 5 years (range, 1 month to 63 years), occurred in 114 males (76%), and was unilateral in 142 patients (95%). There was no predilection for race or laterality. The most common referral diagnoses were Coats disease in 64 cases (41%) and retinoblastoma in 43 (27%). The first symptom or sign was decreased visual acuity in 68 cases (34%), strabismus in 37 (23%), leukocoria in 31 (20%), and 13 patients (8%) were asymptomatic. Visual acuity at presentation was 20/200 to no light perception in 121 eyes (76%). The anterior segment was normal in 142 eyes (90%). The retinal telangiectasia involved the midperipheral or peripheral fundus in 156 of the 158 eyes (99%) and was restricted to the macular area in two eyes (1%); involved mainly the temporal fundus in 66 eyes (42%), inferior fundus in 41 eyes (26%), and more than one sector in 34 eyes (22%). Retinal exudation was present in all 12 clock hours in 86 eyes (55%) and six or more clock hours in 115 eyes (73%). There was a total retinal detachment in 74 eyes (47%) and neovascular glaucoma in 12 (8%). Retinal macrocysts were present in 18 eyes (11%), a vasoproliferative tumor in nine eyes (6%) and retinal neovascularization in four eyes (3%). Fluorescein angiography in 49 of the 158 eyes (37%) disclosed early hyperfluorescence of the telangiectasias and macular edema in 18 of eyes (37%). Ultrasonography typically showed a retinal detachment but no solid mass. CONCLUSIONS: Coats disease is a distinct clinical entity characterized by idiopathic retinal telangiectasia and retinal exudation. It is usually unilateral, occurs mostly in young males, and can cause severe visual loss resulting from exudative retinal detachment. The clinician should follow strict criteria in making the diagnosis, to avoid confusing Coats disease with other forms of exudative retinopathy.

263 citations


Journal ArticleDOI
TL;DR: Coats' disease can first be diagnosed in adulthood with retinal vascular abnormalities similar to those seen in younger patients, including limited area of involvement, slower apparent progression of disease, and hemorrhage near larger vascular dilatations.
Abstract: Purpose To investigate the manifestations and clinical course of Coats' disease diagnosed in adulthood and to describe the characteristics of patients who develop it. Design Retrospective observational case series. Participants Thirteen eyes of 13 patients with Coats' disease diagnosed after age 35 years. Methods All patients examined in the authors' referral practices for Coats' disease diagnosed after the age of 35 years were identified. Patients with a history of radiation exposure, intraocular inflammation, retinal vascular occlusion, or Coats' response were excluded. A review of their historical, clinical, and fluorescein angiographic features and demographics was performed. Results There were 13 patients with a mean age at diagnosis of 50 years (range, 36–79) and a mean follow-up of 5.8 years (range, 0–17). These patients manifested many findings typical of Coats' disease, including the unilateral nature of the disease (13/13 patients), male predominance (12/13), vascular telangiectasis, lipid exudation, macular edema, and areas of capillary nonperfusion with adjacent webs of filigreelike capillaries. Disease was limited to ≤6 clock hours in 10 of 13 patients (76.9%), did not generally involve the retinal vasculature anterior to the equator, and showed limited potential to expand during the follow-up period. A localized hemorrhage was noted in 10 of 13 patients (76.9%), almost always near larger aneurysmal dilatations. Limited exudative detachment of the retina was seen in 4 eyes, 3 of which responded to localized laser photocoagulation. One patient had subretinal fluid seen on ocular coherence tomography that did not require treatment. On average, patients lost 2.1 lines of visual acuity during the follow-up period; 6 patients had a decline in vision, 2 patients had improved vision, and 3 patients had stable vision. No patient had end-stage findings of Coats' disease, such as iris neovascularization or total exudative detachment. Conclusions Coats' disease can first be diagnosed in adulthood with retinal vascular abnormalities similar to those seen in younger patients. There are a number of important differences in disease manifestation in adults, including limited area of involvement, slower apparent progression of disease, and hemorrhage near larger vascular dilatations.

85 citations


"Enfermedad de coats recidivante dia..." refers background in this paper

  • ...En adultos, las alteraciones aparecen en la región ecuatorial y periférica, son más frecuentes las hemorragias asociadas a macroaneurismas, y la evolución es de progresión más lenta que en niños (3)....

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  • ...Todas estas enfermedades pueden tener una respuesta retiniana exudativa, similar al aspecto clínico de la enfermedad de Coats, conocido como Coats–like syndrome (3)....

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  • ...Generalmente, es más agresiva cuanto menor es la edad inicial de presentación (3)....

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  • ...A nivel de clínica no existe ni leucocoria ni estrabismo en el caso de adultos y suele ser bastante asintomático, y tampoco tiene complicaciones mayores como glaucoma neovascular (3)....

    [...]

  • ...Es probable que diferentes defectos den lugar a diferentes fenotipos de la enfermedad, con diferentes grados de severidad y edad de comienzo (3,4)....

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Journal ArticleDOI
Abstract: . Purpose: An increasing variety of eponymous terms have been used to describe atypical cases of Coats’ disease. A group of typical cases of Coats’ disease and other cases of differing severity were classified as one of four subtypes of idiopathic retinal telangiectasis with exudation and compared with regard to clinical outcome. Methods: In a retrospective clinical review patients with typical and atypical Coats’ disease were classified as severe, focal, juxtafoveal or associated (with another disease) forms of idiopathic retinal telangiectasis with exudation. Results: 53 eyes in 50 patients were examined of which 62% (n=31) were male. 12 eyes were classified as severe (group 1), 22 focal (group 2), 12 juxtafoveal (group 3) and 7 associated (group 4). The mean age at diagnosis was lowest in group 1 eyes (6.8 years). The best visual acuity at presentation was 6/60 in group 1 whereas high proportions of eyes in the other groups had initial visual acuities of 6/24 or better. In group 1 only one eye was treated, the majority of eyes were blind or had been enucleated whereas 34 (79%) of eyes in the other groups were suitable for treatment and 29 eyes (67%) retained pre-treatment visual acuity or better at last follow-up. Conclusions: Idiopathic retinal telangiectasis with exudation is a spectrum of disease, which is synonymous with Coats’ disease. In this retrospective study eyes with severe idiopathic retinal telangiectasis with exudation corresponding to typical Coats’ disease, have poorer vision at presentation, are less suitable for treatment and have worse outcomes than eyes with other subtypes. The spectrum of disease severity seen in idiopathic retinal telangiectasis with exudation may be due to second somatic mutations in genes with an existing germline mutation (the two hit theory) and a mosaic phenotype.

42 citations


"Enfermedad de coats recidivante dia..." refers background in this paper

  • ...Gass, clasifica en un grupo diferente a los sujetos con hipertensión arterial, policitemia vera, coronariopatía y diabetes, aunque considere idiopáticas las telangectasias (4)....

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  • ...Es probable que diferentes defectos den lugar a diferentes fenotipos de la enfermedad, con diferentes grados de severidad y edad de comienzo (3,4)....

    [...]


Journal ArticleDOI
TL;DR: Coats Disease can recur so it is important that parents educate any affected children regarding the necessity of follow-up, including throughout their adult years, as well as documenting the recurrent nature of Coats Disease.
Abstract: Purpose To document the recurrent nature of Coats Disease. Methods This study is a retrospective chart review approved by our Institutional Review Board. Thirteen patients (13 eyes) were diagnosed with Coats Disease starting in 1966. Age, visual acuity, time of onset, method of treatment, and the number and intervals of recurrences were documented. Recurrences were recorded once initial treatment proved successful and most exudates had absorbed. The length of follow-up for each patient and the most recent visual acuity was recorded. Results The average follow-up period for the 13 patients was 12.4 years with a range from 4.0 to 37.5 years. Eleven patients (85%) were male, two (15%) were female and all had unilateral involvement. The average age at diagnosis was 7.0 years with a range from 9 months to 27 years. Four out of the twelve treated patients (33%) had recurrences, and three of the four had multiple recurrences. The average elapsed time from successful treatment to the first recurrence was 4.3 years with a range from 3.3 to 5.4 years. The average number of recurrences was 3.3. Conclusion Coats Disease can recur so it is important that parents educate any affected children regarding the necessity of follow-up, including throughout their adult years.

31 citations


"Enfermedad de coats recidivante dia..." refers background in this paper

  • ...(en niños cada 6 meses aproximadamente (5), por la falta de queja y posibilidad de desarrollar ambliopía)....

    [...]

  • ...Se han descrito recidivas de las telangectasias y exudación en el 7% de los casos tras 10 años después de un tratamiento satisfactorio (1,5), que pueden ser múltiples, en intervalos variables de tiempo, con lo que el seguimiento debe ser de por vida....

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  • ...Sin tratamiento la progresión natural de la enfermedad puede conducir a complicaciones como DR total bulloso, glaucoma neovascular y ptisis bulbi (5)....

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