Raras
Buscar doenças, sintomas, genes...
Doença de Coats
ORPHA:190CID-10 · H35.0CID-11 · LD21.YOMIM 300216DOENÇA RARA

A doença de Coats (DC) é uma doença idiopática caracterizada por telangiectasia retiniana com deposição de exsudatos intrarretinianos ou sub-retinianos, podendo levar ao descolamento de retina e cegueira unilateral. A DC é classicamente uma condição isolada e unilateral que afeta crianças pequenas saudáveis.

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Introdução

O que você precisa saber de cara

📋

A doença de Coats (DC) é uma doença idiopática caracterizada por telangiectasia retiniana com deposição de exsudatos intrarretinianos ou sub-retinianos, podendo levar ao descolamento de retina e cegueira unilateral. A DC é classicamente uma condição isolada e unilateral que afeta crianças pequenas saudáveis.

Pesquisas ativas
1 ensaio
9 total registrados no ClinicalTrials.gov
Publicações científicas
783 artigos
Último publicado: 2026 Apr 8
Medicamentos
1 registrados
BEVACIZUMAB

Tem tratamento?

1 medicamento registrado
Ver detalhes, fases e interações →
BEVACIZUMAB

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: H35.0
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

👁️
Olhos
8 sintomas
❤️
Coração
1 sintomas

+ 3 sintomas em outras categorias

Características mais comuns

90%prev.
Morfologia vascular retiniana anormal
Muito frequente (99-80%)
90%prev.
Estrabismo
Muito frequente (99-80%)
55%prev.
Glaucoma
Frequente (79-30%)
55%prev.
Descolamento de retina
Frequente (79-30%)
55%prev.
Morfologia macular anormal
Frequente (79-30%)
17%prev.
Morfologia anormal da câmara anterior
Ocasional (29-5%)
12sintomas
Muito frequente (2)
Frequente (3)
Ocasional (3)
Sem dados (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 12 características clínicas mais associadas, ordenadas por frequência.

Morfologia vascular retiniana anormalAbnormal retinal vascular morphology
Muito frequente (99-80%)90%
EstrabismoStrabismus
Muito frequente (99-80%)90%
Glaucoma
Frequente (79-30%)55%
Descolamento de retinaRetinal detachment
Frequente (79-30%)55%
Morfologia macular anormalAbnormal macular morphology
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico783PubMed
Últimos 10 anos200publicações
Pico202543 papers
Linha do tempo
2026Hoje · 2026🧪 1985Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

1 gene identificado com associação a esta condição. Padrão de herança: Not applicable.

NDPNorrinCandidate gene tested inRestrito
FUNÇÃO

Activates the canonical Wnt signaling pathway through FZD4 and LRP5 coreceptor. Plays a central role in retinal vascularization by acting as a ligand for FZD4 that signals via stabilizing beta-catenin (CTNNB1) and activating LEF/TCF-mediated transcriptional programs. Acts in concert with TSPAN12 to activate FZD4 independently of the Wnt-dependent activation of FZD4, suggesting the existence of a Wnt-independent signaling that also promote accumulation the beta-catenin (CTNNB1). May be involved i

LOCALIZAÇÃO

Secreted

VIAS BIOLÓGICAS (1)
Interconversion of nucleotide di- and triphosphates
MECANISMO DE DOENÇA

Norrie disease

Recessive disorder characterized by very early childhood blindness due to degenerative and proliferative changes of the neuroretina. Approximately 50% of patients show some form of progressive mental disorder, often with psychotic features, and about one-third of patients develop sensorineural deafness in the second decade. In addition, some patients have more complex phenotypes, including growth failure and seizure.

EXPRESSÃO TECIDUAL(Tecido-específico)
Cérebro - Hemisfério cerebelar
40.0 TPM
Ovário
39.0 TPM
Cerebelo
34.9 TPM
Brain Caudate basal ganglia
30.1 TPM
Brain Frontal Cortex BA9
27.6 TPM
OUTRAS DOENÇAS (6)
Norrie diseaseexudative vitreoretinopathy 2, X-linkedpersistent hyperplastic primary vitreousCoats disease
HGNC:7678UniProt:Q00604

Medicamentos e terapias

BEVACIZUMABPhase 3

Mecanismo: Vascular endothelial growth factor A inhibitor

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

251 variantes patogênicas registradas no ClinVar.

🧬 NDP: NM_000266.4(NDP):c.370_372del (p.Leu124del) ()
🧬 NDP: NM_000266.4(NDP):c.47T>A (p.Leu16Gln) ()
🧬 NDP: NM_000266.4(NDP):c.47T>G (p.Leu16Arg) ()
🧬 NDP: NM_000266.4(NDP):c.181C>G (p.Leu61Val) ()
🧬 NDP: NM_000266.4(NDP):c.122G>A (p.Arg41Lys) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 7 variantes classificadas pelo ClinVar.

6
1
Patogênica (85.7%)
VUS (14.3%)
VARIANTES MAIS SIGNIFICATIVAS
PCDH12: NM_016580.4(PCDH12):c.2008G>T (p.Glu670Ter) [Likely pathogenic]
RHO: NM_000539.3(RHO):c.891C>G (p.Ser297Arg) [Pathogenic]
PRSS23: NM_012193.4(FZD4):c.1282_1285del (p.Asp428fs) [Pathogenic/Likely pathogenic]
PRSS23: NM_012193.4(FZD4):c.313A>G (p.Met105Val) [Pathogenic]
RCBTB1: NM_018191.4(RCBTB1):c.707del (p.Asn236fs) [Pathogenic/Likely pathogenic]

Vias biológicas (Reactome)

1 via biológica associada aos genes desta condição.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
3Fase 32
2Fase 21
1Fase 11
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 1 medicamento · 6 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Doença de Coats

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

9 ensaios clínicos encontrados, 1 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
360 papers (10 anos)
#1

Understanding the Molecular Basis of Pathological Retinal Angiogenesis: Roles of Lipids, Non-Coding RNAs, and Cellular Crosstalk.

Investigative ophthalmology &amp; visual science2026 Mar 02

Pathological retinal neovascularization is a prevalent cause of blindness, impacting millions of individuals across all age groups, from infants to older adults. It is a key clinical feature of several retinal diseases, such as diabetic retinopathy (DR), retinopathy of prematurity (ROP), age-related macular degeneration (AMD), and Coats disease. Researchers have extensively investigated the involvement of vascular endothelial growth factor (VEGF) signaling, hypoxia inducible factor (HIF) signaling, and inflammation in the regulation of pathological retinal neovascularization. Recent breakthroughs have demonstrated the influence of several substances and cells in the retina on the regulation of pathological retinal neovascularization. This review emphasizes the rising significance of substances such as lipid mediators, non-coding ribonucleic acids, as well as the involvement of several retinal cell types, including endothelial cells (ECs), pericytes, glial cells, and the retinal pigment epithelium (RPE) in the regulation of pathological neovascularization. Here, we emphasize the interactions among these cell types in maintaining retinal homeostasis and their role in abnormal neovascularization in diseases like DR, AMD, ROP, etc. This review aims to highlight the importance of molecules and cells that go beyond the VEGF-centric therapeutic focus to treat pathological neovascularization.

#2

A Case of Coats Disease Diagnosed during Amblyopia Treatment.

Case reports in ophthalmology2026

Coats disease is a rare, nonhereditary retinal disorder, and early detection and treatment are crucial for preserving visual function. This case report details a pediatric case where Coats disease was diagnosed during follow-up for amblyopia. An 8-year-old boy with a history of bilateral hyperopic amblyopia and right congenital ptosis underwent eyeglass and occlusion therapy starting at age 2. By age 4, he had achieved good visual acuity. At a routine follow-up 4 years later, fundus examination revealed exudative lesions in the left macula. Optical coherence tomography (OCT) showed macular hyperreflective lesions and the thickened choroid in his left eye, leading to a diagnosis of Coats disease. The patient was treated with retinal photocoagulation. Ten months after treatment, his visual acuity remained stable, and a follow-up OCT showed a reduction in the hyperreflective lesions in the outer macular layers. Routine dilated fundus examinations and OCT-based retro-choroidal morphology for anisometropic amblyopia facilitated early detection and timely intervention for Coats disease, which lacks overt symptoms in its early stages. This case highlights the importance of retinal monitoring in amblyopic eyes to preserve visual function.

#3

International Consensuses and Guidelines on Diagnosing and Managing Coats Disease by the Academia Retina Internationalis (ARI), the Asia-Pacific Vitreo-retina Society (APVRS), and the Academy of the Asia-Pacific Professors of Ophthalmology (AAPPO).

American journal of ophthalmology2026 Feb

Coats disease is a rare, retinal vascular disorder characterized by telangiectasias, aneurysmal dilations, and progressive exudative retinal detachment. Limited understanding of the disease warranted the need to identify controversial issues through an extensive literature search and a debate and discussion among international panels of experts. Extensive literature search was done on multiple aspects of the disease-classification patterns, disease, Coats-plus, and Coats-like response. Other key factors included the etiology, possible genetic patterns, relationship with other vascular disorders, the role of inflammatory factors and vascular endothelial growth factor (VEGF) in the pathogenesis, diagnostic features, and complications. Considering the varying treatment patterns followed, imaging modalities, clinical findings, differential diagnosis, treatment options, prognostic factors, and emerging concepts in management were all covered in the search. Eighteen experts were included to opine on questions spanning classification, pathogenesis, diagnostic and treatment methods, prognostic controversies, and newer concepts in disease management. Of the 52 questions in 7 sections, the experts arrived at a consensus for 48 (92.3%) statements (with 75% voted as strong agreement or agreement). Most experts agreed on the suggested classification, diagnosis, and prognosis. The controversy, however, remained in questions regarding association with other vascular disorders, treatment of stages 3 and 4, possible benefits of newer anti-VEGF agents, and the role of artificial intelligence. These debates reflect the rarity of the condition, complex pathophysiology, and the challenges of treating a progressive blinding disease primarily affecting children. Hence, further studies are warranted, especially in areas that have not reached a consensus.

#4

Long-term outcome of Coats' disease: Implications for the classification of foveal vascular pathologies.

Acta ophthalmologica2026 Feb

To investigate the long-term outcome in patients with Coats' disease regarding initial stages. Within a retrospective, clinical, single centre study, patients with diagnosed Coats' disease treated from 1992 to 2019 (minimum follow-up of 12 months) were investigated. A total of, 75 out of 123 patients met the inclusion criteria. A total of, 77 eyes of 75 patients (80.5% male, 17.8 ± 18.6 years, median 10 years) were analysed. Mean follow-up time was 7.2 ± 5.5 years (range 1.1-21.1 years, median 6.3 years). Initial and final visual acuity was lower the higher the disease stage was. The need for surgical interventions was higher in more advanced stages. Notably, within stage 2B, significant differences in final visual acuity were observed depending on the presence of foveal exudation only (0.20 ± 0.29 logMAR) as compared to eyes with foveal vascular abnormalities (1.20 ± 0.55 logMAR, p < 0.001). Based on our analyses, it is suggested to amend the well-defined and established classification system according to Shields by sub-dividing stage 2B (telangiectasia and exudation with foveal exudation) into 2B1 (telangiectasia and exudation with foveal exudation) and 2B2 (telangiectasia and exudation with foveal exudation and foveal Coats' typical vascular abnormalities), since both subgroups significantly differ with respect to prognosis of visual acuity.

#5

NERVE FIBER LAYER SCHISIS IN COATS DISEASE.

Retinal cases &amp; brief reports2026 Mar 01

The aim of the study was to describe two cases of pediatric patients with Coats disease who developed nerve fiber layer schisis. This was an observational case series. Two male pediatric patients, ages 2 and 14 years, who were being treated for Coats disease were found to have nerve fiber layer schisis on optical coherence tomography. Both were treated with panretinal photocoagulation and indirect laser. The 14-year-old patient has had recurrent disease with a most recent visual acuity of 20/80 + 1 in the affected eye. The 2-year-old patient continues to be followed after receiving two rounds of panretinal photocoagulation, followed by two rounds of superotemporal laser. Nerve fiber layer schisis is a rare manifestation of Coats disease.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC531 artigos no totalmostrando 198

2026

Full-Thickness Macular Hole Associated with Adult-Onset Coats Disease : A Case Report.

Korean journal of ophthalmology : KJO
2026

Understanding the Molecular Basis of Pathological Retinal Angiogenesis: Roles of Lipids, Non-Coding RNAs, and Cellular Crosstalk.

Investigative ophthalmology &amp; visual science
2026

Misdiagnosis in Referrals for Advanced Retinopathy of Prematurity.

Ophthalmology. Retina
2026

A Case of Coats Disease Diagnosed during Amblyopia Treatment.

Case reports in ophthalmology
2025

Is occlusive retinal vascular disease linked to hereditary spherocytosis postsplenectomy? A case series.

Journal of medical case reports
2025

Prevalence and incidence of Coats disease in a large claims database.

PloS one
2025

Development of subfoveal nodule in Coats' disease: Prevalence, stages, prognosis, and treatment.

Acta ophthalmologica
2025

Progression of Coats Disease in Nigerian Female Treated with Repeat Intravitreal Bevacizumab: A Case Report.

Annals of African medicine
2025

Refractive Error Correction With Glasses in Congenital Ocular Fundus Anomalies: A Retrospective Series of 18 Children With Different Disease Entities Followed Up for More Than 10 Years.

Cureus
2025

Anti-vascular endothelial growth factor therapies in ophthalmology.

Medical hypothesis, discovery &amp; innovation ophthalmology journal
2026

International Consensuses and Guidelines on Diagnosing and Managing Coats Disease by the Academia Retina Internationalis (ARI), the Asia-Pacific Vitreo-retina Society (APVRS), and the Academy of the Asia-Pacific Professors of Ophthalmology (AAPPO).

American journal of ophthalmology
2025

Non-Neoplastic Disorders Mimicking Posterior Segment Inflammation.

Ocular immunology and inflammation
2025

Mapping Macrophage Diversity in Coats' Disease: A Lipid-Associated Subset in the Pathogenesis of Exudative Retinopathy.

Investigative ophthalmology &amp; visual science
2025

Retinal Capillary Haemangioblastoma: Clinical Spectrum, Imaging Insights, and Treatment Strategies.

Ocular oncology and pathology
2025

Coats Disease With Macular Nodule.

International ophthalmology clinics
2025

Coats-like Retinopathy Associated With 18p Deletion Syndrome.

Ophthalmic surgery, lasers &amp; imaging retina
2025

IN OUR HANDS, WE ADVISE TREATMENT FOR EARLY-STAGE COATS DISEASE.

Retina (Philadelphia, Pa.)
2025

Place of dexamethasone implant as an adjunctive treatment in Coats' disease: a retrospective single-center study.

BMC ophthalmology
2025

Comparison of Adult and Pediatric Eyes With Coats Disease Using Multimodal Imaging.

Journal of vitreoretinal diseases
2026

Long-term outcome of Coats' disease: Implications for the classification of foveal vascular pathologies.

Acta ophthalmologica
2025

SIMPLE OBSERVATION AS A MANAGEMENT OPTION FOR EARLY-STAGE COATS DISEASE.

Retina (Philadelphia, Pa.)
2025

Macular exudation associated with a racemose hemangioma, vasoproliferative tumor and Coats-like vasculopathy.

Retinal cases &amp; brief reports
2025

Management of Vasoproliferative Tumor in a Patient With Coats' Disease: Multiple Therapeutic Approach in Current Era.

Ophthalmic surgery, lasers &amp; imaging retina
2025

Re: Tsai et al.: Clinical characteristics and treatment outcomes in unilateral Coats disease: a global collaborative study (Ophthalmol Retina. 2025;9:570-579).

Ophthalmology. Retina
2025

[National protocol for diagnosis and treatment of Coats disease: Summary for attending and primary care physicians].

Journal francais d'ophtalmologie
2025

Coats disease in infants, toddlers, and preschoolers: Clinical presentation and treatment outcomes.

Indian journal of ophthalmology
2025

Rare pediatric retinal diseases: A review.

Indian journal of ophthalmology
2026

[Intravitreal Faricimab Injections for Coats Disease: a Case Report].

Klinische Monatsblatter fur Augenheilkunde
2025

Pars Plana Vitrectomy with Foveal Cyst Aspiration as a Treatment Option for Macular Retinoschisis in a Coat-Like Retinitis Pigmentosa Case.

Retinal cases &amp; brief reports
2025

Exudative Retinal Detachment in Pediatric Coats Disease.

JAMA ophthalmology
2025

Coats' Disease: A Comprehensive Review of Its Pathophysiology, Diagnosis, and Advances in Treatment.

Seminars in ophthalmology
2025

Oral fluorescein angiography in pediatric ophthalmology.

Survey of ophthalmology
2025

[Analysis of 5-year efficacy of adjuvant external drainage of subretinal fluid therapy in Coats disease with severe exudative retinal detachment].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2025

Active External Drainage of Subretinal Fluid without Vitrectomy to Treat Advanced Coats Disease with Exudative Retinal Detachment - A Case Report and Literature Review.

Retinal cases &amp; brief reports
2025

Pediatric Eye Screening: Current Standards and Gaps in Care.

Ophthalmic surgery, lasers &amp; imaging retina
2025

Ultra-Widefield OCT Images of Proliferative Vitreoretinopathy under Silicone Oil.

Case reports in ophthalmology
2025

INTRAOPERATIVE 3-DIMENSIONAL FLUORESCEIN ANGIOGRAPHY-GUIDED PARS PLANA VITRECTOMY FOR THE TREATMENT OF MULTIPLE RETINAL DISEASES.

Retina (Philadelphia, Pa.)
2025

Clinical characteristics and management outcomes of pediatric patients with Coats disease complicated with retinoschisis (retinal cyst).

Frontiers in medicine
2025

[Retinal vasoproliferative tumor (retinal reactive astrocytic tumor)].

Journal francais d'ophtalmologie
2024

Late presentation of Coats disease in a 12-year-old boy: a case report.

The Pan African medical journal
2025

Machine learning demonstrates clinical utility in distinguishing retinoblastoma from pseudo retinoblastoma with RetCam images.

Ophthalmic genetics
2025

Perifoveal vascular anomalous complex and telangiectatic capillaries: An overview of two entities potentially sharing a common pathophysiology.

Survey of ophthalmology
2025

Coats disease presenting with vitreous hemorrhage and neovascular glaucoma.

American journal of ophthalmology case reports
2025

Coats' disease in adulthood with preserved vision after intravitreal aflibercept injection combined with laser photocoagulation : a case report.

BMC ophthalmology
2025

12-year cumulative incidence rate of rare retinal diseases: a nationwide study in Korea.

Eye (London, England)
2026

NERVE FIBER LAYER SCHISIS IN COATS DISEASE.

Retinal cases &amp; brief reports
2025

Retinoblastoma and beyond: pediatric orbital mass lesions.

Neuroradiology
2025

Comparison of retinal vascular abnormalities in the fellow eyes of children with Coats' disease and normal subjects using ultra-widefield fundus fluorescein angiography.

Photodiagnosis and photodynamic therapy
2024

Bilateral Coats' disease in a 5-year-old boy.

Asia-Pacific journal of ophthalmology (Philadelphia, Pa.)
2025

Targeted Multimodality Treatment to Eliminate the Need for Enucleation in Advanced Coats Disease.

Journal of vitreoretinal diseases
2025

Clinical Characteristics and Treatment Outcomes in Unilateral Coats Disease: A Global Collaborative Study.

Ophthalmology. Retina
2025

Clinical Profiles of Retinal Vasoproliferative Tumors.

Journal of vitreoretinal diseases
2024

Diagnostic Dilemma: Ocular Features and Treatment Outcome of a Case of Coats-Like Retinitis Pigmentosa.

Cureus
2025

Clinical Presentation and Treatment Outcomes of Adult-Onset Coats Disease.

Journal of vitreoretinal diseases
2024

Management of persistent exudative retinal detachment: exploring etiology and surgical outcomes.

BMC ophthalmology
2024

Intraretinal hyperreflective line: potential biomarker in various retinal disorders.

Medical hypothesis, discovery &amp; innovation ophthalmology journal
2024

A Brief Review on Adult-Onset Coats' Disease.

Romanian journal of ophthalmology
2024

Eales' disease with secondary coats'-like reaction: a case report.

BMC ophthalmology
2024

Retinal detachment in Coats' disease.

Arquivos brasileiros de oftalmologia
2024

Preventable vision loss in children with Coats disease.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2024

Ultra-Widefield Fluorescein Angiographic Features Influencing Visual Prognosis in Coats Disease.

Korean journal of ophthalmology : KJO
2024

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

International journal of ophthalmology
2024

Lens-Sparing Perfluoro-Octane-Assisted External Drainage of Total Exudative Retinal Detachment in Coats' Disease.

Ophthalmic surgery, lasers &amp; imaging retina
2024

Injectable Fluocinolone Implant for the Management of Cystoid Macular Edema in Coats' Disease.

Ophthalmic surgery, lasers &amp; imaging retina
2024

The Red Reflex Test and Leukocoria in Childhood.

Acta medica portuguesa
2024

Global estimates on the number of people blind or visually impaired by Uncorrected Refractive Error: a meta-analysis from 2000 to 2020.

Eye (London, England)
2024

Global estimates on the number of people blind or visually impaired by age-related macular degeneration: a meta-analysis from 2000 to 2020.

Eye (London, England)
2024

Global estimates on the number of people blind or visually impaired by diabetic retinopathy: a meta-analysis from 2000 to 2020.

Eye (London, England)
2024

Navigated Focal Laser of Macular Telangiectasia Type I (Adult-Onset Coats' Disease).

Ophthalmic surgery, lasers &amp; imaging retina
2024

Apparent Diffusion Coefficient (ADC) Differentiates Retinoblastoma from Coats Disease on MRI.

American journal of ophthalmology
2024

Surgical Drainage of Large Macular Cystoid Spaces in Coats Disease.

Journal of vitreoretinal diseases
2024

Coats-Like Presentation of Familial Exudative Vitreoretinopathy Associated With a Novel LRP5 Variant.

Ophthalmic surgery, lasers &amp; imaging retina
2024

Global estimates on the number of people blind or visually impaired by glaucoma: A meta-analysis from 2000 to 2020.

Eye (London, England)
2024

Automated detection of nine infantile fundus diseases and conditions in retinal images using a deep learning system.

The EPMA journal
2024

Global estimates on the number of people blind or visually impaired by cataract: a meta-analysis from 2000 to 2020.

Eye (London, England)
2023

Exudative Retinal Detachment in Nigerians: Demographics and Causes.

Journal of the West African College of Surgeons
2024

Advantages of the Utilization of Wide-Field OCT and Wide-Field OCT Angiography in Clinical Practice.

Diagnostics (Basel, Switzerland)
2024

Pediatric orbital lesions: ocular pathologies.

Pediatric radiology
2024

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.

Case reports in ophthalmology
2024

Comparison of quantification of intraretinal hard exudates between optical coherence tomography en face image versus fundus photography.

Indian journal of ophthalmology
2023

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

Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological Society
2024

Extending Peripheral Retinal Vascularization in Retinopathy of Prematurity Through Regulation of VEGF Signaling.

American journal of ophthalmology
2024

Scleral incision and open fluid surgery combined with vitreous cavity injection (conbercept) for the treatment of Coats disease: A case report.

Asian journal of surgery
2024

Subfoveal Nodule Affecting Visual Prognosis in Coats Disease.

Korean journal of ophthalmology : KJO
2024

A Rare Association: Neovascular Glaucoma Accompanying Anterior Chamber Synchysis Scintillans.

Turkish journal of ophthalmology
2023

Vascular abnormalities in fellow eyes of patients with unilateral coats' disease.

Scientific reports
2023

Severe Corneal Melt Post Trans-Scleral Cyclodiode in a Case of Neovascular Glaucoma Secondary to Coats Disease: A Case Report.

Case reports in ophthalmology
2023

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

Children (Basel, Switzerland)
2024

Coats' syndrome: A rare cause of infant leukocoria to keep in mind.

Radiology case reports
2023

AN EIGHT-YEAR RETROSPECTIVE STUDY OF THE ETIOLOGIES, CLINICAL CHARACTERISTICS, AND VISUAL OUTCOMES OF PEDIATRIC LAMELLAR MACULAR HOLE.

Retina (Philadelphia, Pa.)
2023

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

Indian journal of ophthalmology
2023

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

Journal of ocular pharmacology and therapeutics : the official journal of the Association for Ocular Pharmacology and Therapeutics
2023

Retracted: Clinical Study of Intravitreal Injection of Anti-VEGF Drugs Combined with Triamcinolone Acetonide in the Treatment of Coats Disease.

Computational and mathematical methods in medicine
2023

Coats' Disease in a Patient With Cornelia de Lange Syndrome: Management With Laser and Bevacizumab.

Journal of pediatric ophthalmology and strabismus
2024

Unusual anterior and posterior segment features of coats disease.

European journal of ophthalmology
2024

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.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
2023

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

Case reports in ophthalmology
2023

Laser versus Anti-VEGF: A Paradigm Shift for Treatment-Warranted Retinopathy of Prematurity.

Ophthalmology and therapy
2023

ABNORMALITIES IN THE RETINAL CAPILLARY PLEXUSES IN COATS DISEASE IN ADULTHOOD ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

Retina (Philadelphia, Pa.)
2023

Optical Coherence Tomography and Optical Coherence Tomography Angiography in Pediatric Retinal Diseases.

Diagnostics (Basel, Switzerland)
2023

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

Journal of vitreoretinal diseases
2023

FURTHER EVIDENCE AGAINST BILATERAL MANIFESTATION OF COATS DISEASE IN OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY OF THE MACULA.

Retina (Philadelphia, Pa.)
2022

Paradoxical Exudative Retinopathy and Macular Star Formation After Treatment Initiation in Coats Disease.

Journal of vitreoretinal diseases
2022

Optical Coherence Tomography Angiography in Pediatric Retinal Disorders.

Journal of vitreoretinal diseases
2023

An Unusual Optical Coherence Tomography Appearance in Coats Disease.

Retina (Philadelphia, Pa.)
2023

Adult-onset Coats disease.

Survey of ophthalmology
2023

[Molecular genetics in diagnosis of Coats disease: combination of oligogenic variants associated with different forms of hereditary retinal dystrophy].

Vestnik oftalmologii
2023

Avascular Peripheral Retina in Infants.

Turkish journal of ophthalmology
2023

Initial Experience With Biosimilar Bevacizumab-bvzr For Intravitreal Use in Children: A Case Series and Literature Review.

Ophthalmic surgery, lasers &amp; imaging retina
2022

Advanced Coats' disease: Treating the effect before the cause.

Oman journal of ophthalmology
2022

A patient with X-linked retinoschisis and exudative retinal detachment associated with a pathogenic hemizygous variant c.304c>T in RS1.

Ophthalmic genetics
2023

Vasoproliferative retinal tumors: Clinical presentation and treatment outcome.

European journal of ophthalmology
2023

Familial exudative vitreoretinopathy (FEVR) in a child with novel microarray-defined deletion of 11q14 previously diagnosed as retinopathy of prematurity (ROP).

Ophthalmic genetics
2022

The Male to Female Ratio in Treatment-Warranted Retinopathy of Prematurity: A Systematic Review and Meta-analysis.

JAMA ophthalmology
2022

Clinicopathological Correlations in Enucleated Globes of Late-Stage Coats Disease with a Review of the Literature.

Journal of epidemiology and global health
2022

Coats disease in female population: A comparison of clinical presentation and outcomes.

Frontiers in medicine
2023

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

International ophthalmology
2022

Longitudinal characterization and treatment response of retinal arterial macroaneurysms in adult-onset coats disease.

American journal of ophthalmology case reports
2022

A Novel Approach to Subretinal Fluid Drainage in Coats'-Related Exudative Retinal Detachment.

Ophthalmic surgery, lasers &amp; imaging retina
2022

Coats disease in adolescence and adulthood with preserved vision after laser photocoagulation monotherapy: two case reports.

Journal of medical case reports
2022

Clinical Study of Intravitreal Injection of Anti-VEGF Drugs Combined with Triamcinolone Acetonide in the Treatment of Coats Disease.

Computational and mathematical methods in medicine
2022

The Masquerading Retinopathy of Revesz Syndrome.

Ophthalmic surgery, lasers &amp; imaging retina
2022

Fovea-threatening and fovea-involving peripheral Coats disease: effects of posture and intervention.

International journal of retina and vitreous
2023

Genetic evaluation in phenotypically discordant monozygotic twins with Coats Disease.

European journal of ophthalmology
2022

Spectrum of Mutations in NDP Resulting in Ocular Disease; a Systematic Review.

Frontiers in genetics
2022

Unilateral Xanthocoria and Retinal Vascular Anomalies in a 3-Year-Old Boy: Retinoblastoma or Coats Disease?

Journal of pediatric ophthalmology and strabismus
2023

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

European journal of ophthalmology
2022

Etiology of white pupillary reflex in pediatric age group.

Romanian journal of ophthalmology
2022

Commentary: Advanced Coats' disease - Is it worth chasing the goal of globe salvage?

Indian journal of ophthalmology
2022

Updating Understanding of Macular Microvascular Abnormalities and Their Correlations With the Characteristics and Progression of Macular Edema or Exudation in Coats' Disease.

Frontiers in medicine
2023

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

European journal of ophthalmology
2022

Long-term follow-up of a case of Coats disease in a 10-year-old boy with spontaneous peeling of preretinal macular fibrosis: a case report.

BMC ophthalmology
2023

Wide-field Fundus Imaging and Fluorescein Angiography Findings in Various Pseudoretinoblastoma Conditions.

Journal of pediatric ophthalmology and strabismus
2023

Dexamethasone intravitreal implant as an adjuvant treatment for pediatric patients with Coats' disease.

Arquivos brasileiros de oftalmologia
2022

Coats plus in prematurity.

Ophthalmic genetics
2022

Multimodal imaging of unusual macular macroaneurysm rupture after navigated retinal laser in a patient with adult onset Coats disease.

American journal of ophthalmology case reports
2022

Imaging Findings of Pediatric Orbital Masses and Tumor Mimics.

Radiographics : a review publication of the Radiological Society of North America, Inc
2022

Multimodal treatment of Coats-like exudative vitreoretinopathy in Goldmann-Favre syndrome.

American journal of ophthalmology case reports
2022

EVIDENCE AGAINST A CONTRALATERAL COATS PHENOTYPE BY OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

Retina (Philadelphia, Pa.)
2022

Unilateral peripheral vascular retinal disease associated with a developmental arcade vessel anomaly in adult-onset Coats' disease.

American journal of ophthalmology case reports
2022

Coats'-like Response Associated with Linear Scleroderma.

Journal of ophthalmic &amp; vision research
2022

Plentiful melanin pigment containing histiocyte-like cells in Coats disease: Awareness avoids diagnostic pitfall.

Cytopathology : official journal of the British Society for Clinical Cytology
2022

Phenotype of Coats disease in females.

BMJ open ophthalmology
2022

Coats' disease - Prognostic factors for globe and vision salvage in children, a long-term experience.

Indian journal of ophthalmology
2021

Rhegmatogenous retinal detachment in Coats' disease: a case report.

Journal of medical case reports
2022

Treatment of Advanced Coats' Disease With Combination Therapy of Laser Photocoagulation, Intravitreal Ranibizumab, and Sub-Tenon Methylprednisolone Acetate.

Journal of pediatric ophthalmology and strabismus
2022

Coats Disease and Premature Telomere Shortening.

Journal of pediatric ophthalmology and strabismus
2022

Coats Disease in 9 Patients: A Hispanic Case Series.

Klinische Monatsblatter fur Augenheilkunde
2021

Diagnosis and Management of Coats' Disease.

Ophthalmic surgery, lasers &amp; imaging retina
2021

Coats' disease: trends and long-term treatment outcomes in a tertiary referral centre.

Therapeutic advances in ophthalmology
2021

[Primary Diseases of 393 Cases of Pediatric Retinal Detachment and the Distribution of Clinical Characteristics].

Sichuan da xue xue bao. Yi xue ban = Journal of Sichuan University. Medical science edition
2021

Clinical Characteristics of Pediatric Coats' Disease With Retinal Cyst Using Wide-Angle Fluorescein Angiography.

Frontiers in medicine
2021

Intraocular osseous metaplasia presenting as a solid mass in chronic retinal detachment: a case report.

International journal of retina and vitreous
2021

Vitrectomy and external drainage of subretinal fluid containing high concentration of vascular endothelial growth factor for advanced coats disease.

Scientific reports
2021

Advanced Coats' disease: Cytopathology to the rescue!

Diagnostic cytopathology
2022

Coats disease masquerading as acute posterior uveitis in a young adult.

The Kaohsiung journal of medical sciences
2022

Retinal arteriolar macroaneurysms with supravalvular pulmonic stenosis in the United Arab Emirates.

Ophthalmic genetics
2021

Choroidal detachment secondary to intravitreal dexamethasone implant in a patient with Coats' disease.

Journal francais d'ophtalmologie
2021

Optical coherence tomography angiography and multimodal imaging in the management of coats' disease.

American journal of ophthalmology case reports
2021

Coats' Disease Diagnosed During Adulthood.

Cureus
2021

Pseudo-enophthalmos and Coats' disease: Is there a relationship?

Journal francais d'ophtalmologie
2021

Resolution of Foveal Lipid Deposition in Adult-Onset Coats Disease With Combined Focal Laser Photocoagulation and Anti-VEGF Therapy.

Ophthalmic surgery, lasers &amp; imaging retina
2022

Adjunctive Intravitreal Triamcinolone Acetonide for Exudative Retinal Detachment in Coats Disease.

Journal of vitreoretinal diseases
2021

Novel compound heterozygous STN1 variants are associated with Coats Plus syndrome.

Molecular genetics &amp; genomic medicine
2021

Bilateral Retinal Vein Occlusion-Simulated Coats' Disease.

Diagnostics (Basel, Switzerland)
2021

The Final Diagnosis: Retinoblastoma or Pseudoretinoblastoma.

Journal of pediatric ophthalmology and strabismus
2021

Coats disease with retinal neovascularization under subfoveal nodule: optical coherence tomography-angiography findings before and after ranibizumab treatment.

Arquivos brasileiros de oftalmologia
2021

Multimodal Imaging Findings and Treatment with Dexamethasone Implant in Three Cases of Idiopathic Macular Telangiectasia Type 1.

Case reports in ophthalmology
2021

Idiopathic Peripheral Retinal Telangiectasia in Adults: A Case Series and Literature Review.

Journal of clinical medicine
2022

Secondary vasoproliferative tumor in adult-onset Coats disease.

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie
2022

Subthreshold micropulse laser for adult onset Coats' associated exudative maculopathy.

European journal of ophthalmology
2021

Feasibility and Clinical Utility of Ultra-Widefield-Navigated Swept-Source Optical Coherence Tomography Imaging.

Journal of vitreoretinal diseases
2021

Clinical features and prognostic factors in 71 eyes over 20 years from patients with Coats' disease in Korea.

Scientific reports
2021

Retrospective Evaluation of Somatic Alterations in Cell-Free DNA from Blood in Retinoblastoma.

Ophthalmology science
2020

Optical Coherence Tomography Angiography Findings After Intravitreal Ranibizumab in Patients With Coats Disease.

Frontiers in medicine
2021

An Evaluation of the Microvasculature of Macular Nodules in Coats Disease Using Optical Coherence Tomography Angiography: A Report of 3 Cases.

Journal of vitreoretinal diseases
2021

Experience with Intravitreal Ranibizumab as an Adjunct to Ablation Therapy in Eyes with Exudative Coats' Disease.

Clinical ophthalmology (Auckland, N.Z.)
2021

Macular fibrosis in Coats' disease demonstrated by Optical Coherence Tomography Angiography - new data toward pathophysiology.

European journal of ophthalmology
2020

Three-year follow-up of Coats disease treated with conbercept and 532-nm laser photocoagulation.

World journal of clinical cases
2022

As the worm turns: An infant with exotropia.

Survey of ophthalmology
2020

MR Imaging Features to Differentiate Retinoblastoma from Coats' Disease and Persistent Fetal Vasculature.

Cancers
2021

Fovea-sparing coats disease: A rare clinical entity.

European journal of ophthalmology
2020

Multiple Therapy Approach for Stage 3 Coats Disease: Long-Term Follow-Up.

Journal of ophthalmology
2020

Three-Year Results of Management of Adult-Onset Coats' Disease by Possibly Targeting Placental Growth Factor.

Cureus
2021

Vascular findings in primarily affected and fellow eyes of middle-aged patients with Coats' disease using multimodal imaging.

The British journal of ophthalmology
2020

Efficacy and Safety Outcomes of Intravitreal Dexamethasone Implant Therapy for the Treatment of Adult Coats' Disease.

Journal of ophthalmology
2021

Retinoschisis in Coats Disease: Clinical Picture, Therapeutic Considerations, and Management Outcomes.

Journal of vitreoretinal diseases
2020

Long-Term Clinical Results and Management following Vitrectomy in Undetected Retinoblastoma Eyes.

Ocular oncology and pathology
2021

Case of Coats disease associated with neurofibromatosis type 1.

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie
2021

Subretinal Mononuclear Cells in Coats' Disease Studied with RPE65 and CD163: Evidence for Histiocytoid Pigment Epithelial Cells.

American journal of ophthalmology
2021

External Drainage Alone Versus External Drainage With Vitrectomy in Advanced Coats Disease.

American journal of ophthalmology
2020

Full-Thickness Macular Hole with Coats Disease: A Case Report.

Case reports in ophthalmology
2020

Coats retinopathy with pachychoroid and central serous chorioretinopathy in the fellow eye.

BMJ case reports
2020

Advanced Coats' disease treated with intravitreal brolucizumab combined with laser photocoagulation.

American journal of ophthalmology case reports
2020

Ocular Manifestations of Chronic Granulomatous Disease: First Report of Coats' Disease and Literature Review.

Journal of clinical immunology
2020

[Typical presentation of Coats' disease].

Journal francais d'ophtalmologie
2020

Surgical Management of Advanced Coats Disease in 32 Eyes: A 20-Year Study.

Journal of vitreoretinal diseases
2020

Levels of cytokines in the aqueous humor guided treatment of refractory macular edema in adult-onset coats' disease.

BMC ophthalmology
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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Understanding the Molecular Basis of Pathological Retinal Angiogenesis: Roles of Lipids, Non-Coding RNAs, and Cellular Crosstalk.
    Investigative ophthalmology &amp; visual science· 2026· PMID 41837574mais citado
  2. A Case of Coats Disease Diagnosed during Amblyopia Treatment.
    Case reports in ophthalmology· 2026· PMID 41551199mais citado
  3. International Consensuses and Guidelines on Diagnosing and Managing Coats Disease by the Academia Retina Internationalis (ARI), the Asia-Pacific Vitreo-retina Society (APVRS), and the Academy of the Asia-Pacific Professors of Ophthalmology (AAPPO).
    American journal of ophthalmology· 2026· PMID 41110679mais citado
  4. Long-term outcome of Coats' disease: Implications for the classification of foveal vascular pathologies.
    Acta ophthalmologica· 2026· PMID 40616365mais citado
  5. NERVE FIBER LAYER SCHISIS IN COATS DISEASE.
    Retinal cases &amp; brief reports· 2026· PMID 39752583mais citado
  6. Therapeutic effect of faricimab in macular telangiectasia type 1 and Coats' disease: case series.
    BMC Ophthalmol· 2026· PMID 41952137recente
  7. Full-Thickness Macular Hole Associated with Adult-Onset Coats Disease : A Case Report.
    Korean J Ophthalmol· 2026· PMID 41840934recente
  8. Misdiagnosis in Referrals for Advanced Retinopathy of Prematurity.
    Ophthalmol Retina· 2026· PMID 41724239recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:190(Orphanet)
  2. OMIM OMIM:300216(OMIM)
  3. MONDO:0010269(MONDO)
  4. GARD:6121(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Artigo Wikipedia(Wikipedia)
  8. Q915322(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Doença de Coats
Compêndio · Raras BR

Doença de Coats

ORPHA:190 · MONDO:0010269
Prevalência
Unknown
Herança
Not applicable
CID-10
H35.0 · Retinopatias de fundo e alterações vasculares da retina
CID-11
Ensaios
1 ativos
Medicamentos
1 registrados
Início
Childhood
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0154832
EuropePMC
Wikidata
Wikipedia
Papers 10a
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