Raras
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Coloboma da coroide e retina
ORPHA:98942CID-10 · Q14.8CID-11 · LA13.1DOENÇA RARA

O coloboma da coróide e da retina é um defeito genético raro do desenvolvimento durante a embriogênese, caracterizado pela ausência parcial do epitélio pigmentar da retina e da coróide, mais frequentemente localizado no quadrante inferonasal. Os pacientes geralmente apresentam visão reduzida e têm risco aumentado de descolamento de retina. Outras anomalias oculares (por exemplo, coloboma da íris, microcórnea, nistagmo, estrabismo, microftalmia) estão geralmente associadas, mas também podem ser isoladas.

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Introdução

O que você precisa saber de cara

📋

O coloboma da coróide e da retina é um defeito genético raro do desenvolvimento durante a embriogênese, caracterizado pela ausência parcial do epitélio pigmentar da retina e da coróide, mais frequentemente localizado no quadrante inferonasal. Os pacientes geralmente apresentam visão reduzida e têm risco aumentado de descolamento de retina. Outras anomalias oculares (por exemplo, coloboma da íris, microcórnea, nistagmo, estrabismo, microftalmia) estão geralmente associadas, mas também podem ser isoladas.

🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q14.8
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Sinais e sintomas

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

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Últimos 10 anos116publicações
Pico201817 papers
Linha do tempo
2026Hoje · 2026📈 2018Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

5 genes identificados com associação a esta condição.

Autosomal dominant
ACTG1Actin, cytoplasmic 2Disease-causing germline mutation(s) inModerado
FUNÇÃO

Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells. May play a role in the repair of noise-induced stereocilia gaps thereby maintains hearing sensitivity following loud noise damage (By similarity)

LOCALIZAÇÃO

Cytoplasm, cytoskeleton

VIAS BIOLÓGICAS (10)
Paradoxical activation of RAF signaling by kinase inactive BRAFSignaling by moderate kinase activity BRAF mutantsSignaling by high-kinase activity BRAF mutantsSignaling downstream of RAS mutantsMAP2K and MAPK activation
MECANISMO DE DOENÇA

Deafness, autosomal dominant, 20

A form of non-syndromic sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information.

OUTRAS DOENÇAS (6)
autosomal dominant nonsyndromic hearing loss 20Baraitser-winter syndrome 2Baraitser-Winter cerebrofrontofacial syndromecoloboma of iris
HGNC:144UniProt:P63261
FZD5Frizzled-5Disease-causing germline mutation(s) (gain of function) inAltamente restrito
FUNÇÃO

Receptor for Wnt proteins (PubMed:10097073, PubMed:20530549, PubMed:26908622, PubMed:9054360). Functions in the canonical Wnt/beta-catenin signaling pathway. In vitro activates WNT2, WNT10B, WNT5A, but not WNT2B or WNT4 signaling (By similarity). In neurons, activation by WNT7A promotes formation of synapses (PubMed:20530549). May be involved in transduction and intercellular transmission of polarity information during tissue morphogenesis and/or in differentiated tissues (Probable). Plays a rol

LOCALIZAÇÃO

Cell membraneGolgi apparatus membraneSynapsePerikaryonCell projection, dendriteCell projection, axon

VIAS BIOLÓGICAS (7)
Ca2+ pathwayAsymmetric localization of PCP proteinsWNT5A-dependent internalization of FZD2, FZD5 and ROR2Disassembly of the destruction complex and recruitment of AXIN to the membraneRegulation of FZD by ubiquitination
MECANISMO DE DOENÇA

Microphthalmia/Coloboma 11

A form of colobomatous microphthalmia, a disorder of eye formation, ranging from small size of a single eye to complete bilateral absence of ocular tissues. Ocular abnormalities like coloboma, opacities of the cornea and lens, scaring of the retina and choroid, and other abnormalities may also be present. Ocular colobomas are a set of malformations resulting from abnormal morphogenesis of the optic cup and stalk, and the fusion of the fetal fissure (optic fissure). MCOPCB11 is an autosomal dominant form with incomplete penetrance.

EXPRESSÃO TECIDUAL(Ubíquo)
Cólon transverso
27.6 TPM
Cólon sigmoide
18.0 TPM
Fígado
17.0 TPM
Tireoide
15.5 TPM
Estômago
13.4 TPM
OUTRAS DOENÇAS (7)
microphthalmia/coloboma 11coloboma of eyelidcoloboma of choroid and retinacoloboma of macula
HGNC:4043UniProt:Q13467
SALL2Sal-like protein 2Disease-causing germline mutation(s) (loss of function) inTolerante
FUNÇÃO

Probable transcription factor that plays a role in eye development before, during, and after optic fissure closure

LOCALIZAÇÃO

Nucleus

MECANISMO DE DOENÇA

Coloboma, ocular, autosomal recessive

An ocular anomaly resulting from abnormal morphogenesis of the optic cup and stalk, and incomplete fusion of the fetal intra-ocular fissure during gestation. The clinical presentation is variable. Some individuals may present with minimal defects in the anterior iris leaf without other ocular defects. More complex malformations create a combination of iris, uveoretinal and/or optic nerve defects without or with microphthalmia or even anophthalmia.

EXPRESSÃO TECIDUAL(Ubíquo)
Cerebelo
88.2 TPM
Cérebro - Hemisfério cerebelar
83.7 TPM
Pituitária
26.7 TPM
Hipotálamo
22.9 TPM
Brain Nucleus accumbens basal ganglia
22.0 TPM
INTERAÇÕES PROTEICAS (2)
OUTRAS DOENÇAS (8)
coloboma, ocular, autosomal recessivecoloboma of maculacoloboma of iriscoloboma of eye lens
HGNC:10526UniProt:Q9Y467
ABCB6ATP-binding cassette sub-family B member 6Disease-causing germline mutation(s) inTolerante
FUNÇÃO

ATP-dependent transporter that catalyzes the transport of a broad-spectrum of porphyrins from the cytoplasm to the extracellular space through the plasma membrane or into the vesicle lumen (PubMed:17661442, PubMed:23792964, PubMed:27507172, PubMed:33007128). May also function as an ATP-dependent importer of porphyrins from the cytoplasm into the mitochondria, in turn may participate in the de novo heme biosynthesis regulation and in the coordination of heme and iron homeostasis during phenylhydr

LOCALIZAÇÃO

Cell membraneMitochondrion outer membraneEndoplasmic reticulum membraneGolgi apparatus membraneEndosome membraneLysosome membraneLate endosome membraneEarly endosome membraneSecreted, extracellular exosomeMitochondrionEndosome, multivesicular body membraneMelanosome membrane

VIAS BIOLÓGICAS (1)
Mitochondrial ABC transporters
MECANISMO DE DOENÇA

Microphthalmia/Coloboma 7

A disorder of eye formation, ranging from small size of a single eye to complete bilateral absence of ocular tissues. Ocular abnormalities like opacities of the cornea and lens, scaring of the retina and choroid, and other abnormalities may also be present. Ocular colobomas are a set of malformations resulting from abnormal morphogenesis of the optic cup and stalk, and the fusion of the fetal fissure (optic fissure).

OUTRAS DOENÇAS (12)
microphthalmia, isolated, with coloboma 7familial pseudohyperkalemiadyschromatosis universalis hereditaria 3obsolete blood group, langereis system
HGNC:47UniProt:Q9NP58
PAX6Paired box protein Pax-6Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Transcription factor with important functions in the development of the eye, nose, central nervous system and pancreas. Required for the differentiation of pancreatic islet alpha cells (By similarity). Competes with PAX4 in binding to a common element in the glucagon, insulin and somatostatin promoters. Regulates specification of the ventral neuron subtypes by establishing the correct progenitor domains (By similarity). Acts as a transcriptional repressor of NFATC1-mediated gene expression (By s

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (5)
Synthesis, secretion, and inactivation of Glucagon-like Peptide-1 (GLP-1)Regulation of gene expression in beta cellsActivation of anterior HOX genes in hindbrain development during early embryogenesisFormation of the anterior neural plateSynthesis, secretion, and inactivation of Glucose-dependent Insulinotropic Polypeptide (GIP)
MECANISMO DE DOENÇA

Aniridia 1

A congenital, bilateral, panocular disorder characterized by complete absence of the iris or extreme iris hypoplasia. Aniridia is not just an isolated defect in iris development but it is associated with macular and optic nerve hypoplasia, cataract, corneal changes, nystagmus. Visual acuity is generally low but is unrelated to the degree of iris hypoplasia. Glaucoma is a secondary problem causing additional visual loss over time.

EXPRESSÃO TECIDUAL(Tecido-específico)
Cérebro - Hemisfério cerebelar
40.8 TPM
Cerebelo
36.9 TPM
Córtex cerebral
3.5 TPM
Brain Caudate basal ganglia
3.4 TPM
Brain Anterior cingulate cortex BA24
3.3 TPM
OUTRAS DOENÇAS (17)
coloboma, ocular, autosomal dominantisolated optic nerve hypoplasiaautosomal dominant keratitisfoveal hypoplasia 1
HGNC:8620UniProt:P26367

Variantes genéticas (ClinVar)

662 variantes patogênicas registradas no ClinVar.

🧬 PAX6: NC_000011.10:g.(31780037_44652946)inv ()
🧬 PAX6: NM_001368894.2(PAX6):c.184G>A (p.Val62Met) ()
🧬 PAX6: NM_001368894.2(PAX6):c.530_531del (p.Tyr177fs) ()
🧬 PAX6: NM_001368894.2(PAX6):c.566-1G>C ()
🧬 PAX6: NM_001368894.2(PAX6):c.630_649dup (p.Arg217fs) ()
Ver todas no ClinVar

Vias biológicas (Reactome)

43 vias biológicas associadas aos genes desta condição.

Translocation of SLC2A4 (GLUT4) to the plasma membrane Gap junction degradation Formation of annular gap junctions Regulation of actin dynamics for phagocytic cup formation EPHB-mediated forward signaling EPH-ephrin mediated repulsion of cells Adherens junctions interactions Recycling pathway of L1 VEGFA-VEGFR2 Pathway Interaction between L1 and Ankyrins Cell-extracellular matrix interactions RHO GTPases activate IQGAPs RHO GTPases Activate WASPs and WAVEs RHO GTPases Activate Formins MAP2K and MAPK activation Signaling by moderate kinase activity BRAF mutants Signaling by high-kinase activity BRAF mutants Signaling by BRAF and RAF1 fusions Paradoxical activation of RAF signaling by kinase inactive BRAF Clathrin-mediated endocytosis RHOBTB2 GTPase cycle Signaling downstream of RAS mutants Signaling by RAF1 mutants Sensory processing of sound by inner hair cells of the cochlea Sensory processing of sound by outer hair cells of the cochlea FCGR3A-mediated phagocytosis Regulation of CDH1 Function Formation of the dystrophin-glycoprotein complex (DGC) GBP-mediated host defense Class B/2 (Secretin family receptors) Ca2+ pathway Asymmetric localization of PCP proteins Disassembly of the destruction complex and recruitment of AXIN to the membrane Regulation of FZD by ubiquitination WNT5A-dependent internalization of FZD2, FZD5 and ROR2 Signaling by RNF43 mutants Mitochondrial ABC transporters Defective ABCB6 causes MCOPCB7 Regulation of gene expression in beta cells Synthesis, secretion, and inactivation of Glucagon-like Peptide-1 (GLP-1) Synthesis, secretion, and inactivation of Glucose-dependent Insulinotropic Polypeptide (GIP) Activation of anterior HOX genes in hindbrain development during early embryogenesis Formation of the anterior neural plate

Diagnóstico

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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

Peripapillary Retinoschisis: The Expanded Spectrum and New Insights From Multimodal Imaging.

American journal of ophthalmology2026 Feb

To characterize and classify different forms of peripapillary retinoschisis (PPRS), and to clarify the nomenclature of this condition. A retrospective, multicenter, multinational case series of PPRS was performed from August 2021 to September 2024. Cases were included if they demonstrated retinoschisis contiguous with and thought to be originating from the optic disc. Demographic and clinical data collected included age, gender, visual acuity, intraocular pressure, axial length, refraction and referring symptoms. Mandatory investigations included optical coherence tomography of the optic disc and macula with radial scans, color fundus photography, and fundus autofluorescence. Select cases underwent fundus fluorescein and/or indocyanine green angiography. Retinoschisis was characterized by the meridian in relation to the optic disc, layer(s) of the retina affected and associated conditions. A literature review was performed to identify all causes of PPRS. A total of 47 eyes from 41 patients with PPRS were identified, comprising of 22 (54%) females and a mean age of 56 years (range 14-92 years). These were classified into 9 aetiologies: Congenital Disc Abnormalities (CDA, n = 16 eyes), peripapillary chorioretinal coloboma (n = 1), peripapillary atrophy (n = 3), glaucoma (n = 7), Peripapillary Pachychoroid Syndrome (PPS, n = 4), peripapillary choroidal neovascularization (PP-CNV, n = 3), high/ pathological myopia (n = 5), vitreopapillary traction (VPT, n = 4) and idiopathic (n = 4). The 9 aetiologies of peripapillary retinoschisis can be classified into 5 groups: non-glaucomatous optic disc abnormalities (CDA, PP-coloboma, PP-atrophy), glaucomatous optic disc abnormalities, peripapillary choroidal diseases (PPS and PP-CNV), vitreous optic disc interface abnormalities and idiopathic. A new entity, "Focal Optic Disc Dome" (FODD) was identified. Understanding the full spectrum of PPRS can assist in correct diagnosis and management.

#2

Choroidal Cavitations within Optic Nerve Colobomas in CHARGE Syndrome.

Ophthalmology. Retina2026 Feb 12
#3

Hybrid cavitary disc anomaly - A case series and proposal of a novel classification system.

Indian journal of ophthalmology2025 Nov 01

Cavitary disc anomalies, including optic disc pit (ODP), optic nerve coloboma (ODC), and morning glory disc anomaly (MGDA), are congenital conditions with distinct embryological origins and systemic associations. Hybrid features combining these anomalies are rare, complicating diagnosis and management. To describe the clinical spectrum, multimodal imaging characteristics, and management strategies of hybrid cavitary disc anomalies, and to propose a novel classification system based on anatomical and physiological overlap. This retrospective case series included 22 eyes from 17 patients seen at a tertiary care center over 19 months (June 2023-December 2024). All underwent best-corrected visual acuity (BCVA) and fundus examination, multimodal imaging (color fundus photography and [optical coherence tomography] OCT), and neuroimaging when indicated. Findings were confirmed by a senior consultant with over 20 years of experience. Management tailored to visual prognosis and macular status. The median BCVA was Log MAR 0.30 ± 1.13. Various anomalies include ODP with ODC and retinal choroidal coloboma in 10 eyes (45.5%), ODP with MGDA in 3 eyes (13.6%), ODC with RCC and bridging, coloboma in 3 eyes (13.6%), and ODC with MGDA in 2 eyes (9.1%). ODP with ODC, dual ODP with ODC and RCC, and dual ODP with ODC or RCC in 1 eye were each present in 1 eye (4.5). One patient had bilateral hybrid anomalies with maculopathy. Surgical intervention was required in 4 eyes (18.2%). Hybrid cavitary disc anomalies, including MGDA and ODP, present unique diagnostic and management challenges. This study provides insights into these rare conditions, emphasizing the need for further monitoring and research.

#4

Retinal detachments associated with choroidal colobomas.

Taiwan journal of ophthalmology2025

To summarize the characteristics of the retinal detachments (RDs) that are associated with choroidal colobomas that occur in pediatric and adult patients. A choroidal coloboma is a rare disorder that results from an incomplete closure of the embryonic optic fissure, and their size can range from small colobomas with isolated chorioretinal involvement to large colobomas affecting the iris, choroid, retina, and optic nerve. A RD is occasionally associated with choroidal colobomas, and histological studies of the area of the choroidal coloboma show an absence of normal choroidal tissue, retinal pigment epithelium (RPE), and retina. Near the margin of the coloboma, the inner retinal layer has a central continuation of the marginal intercalary membrane (ICM) within the coloboma. The outer layer folds back, becomes disorganized, and fuses with the RPE. The inner retina gradually thins and merges with the marginal ICM with a high incidence of tears of the ICM developing along the edge of the coloboma or toward the center. Because of the high association of the causative retinal breaks being located within the colobomatous area, vitrectomy, endolaser photocoagulation around the margin of coloboma, and long-term tamponade with silicone oil or gas are recommended treatments. In addition, the presence of the macula within the area of the laser photocoagulation should be considered. However, the recurrence rate is high and multiple surgeries are required to reattach the detached retina.

#5

North Carolina Macular Dystrophy.

Advances in experimental medicine and biology2025

North Carolina Macular Dystrophy (NCMD) is an autosomal dominant, congenital, completely penetrant non-progressive macular malformation. The NCMD phenotype is highly variable even within the same family (Fig. 21.1). Grade 1 individuals have few drusen centrally while grade 2 can appear with confluent drusen to central vitelliform lesions. Grade 3 appears as dramatic choroidal excavations and coloboma like lesions (Small 1989; Small et al. 1991, 2022a). There can be some vision decline secondary to the development of choroidal neovascular membranes (CNVMs) (Bakall et al. 2018). Patients who develop CNVMs are the subjects who experience progressive moderate to severe vision impairment, typically confined to one eye. In grade 3 lesions, the CNVMs and resulting fibrosis typically occur along the temporal edge of the "coloboma" and do not affect the visual acuity. Peripheral drusen are variably reported.

Publicações recentes

Ver todas no PubMed

📚 EuropePMCmostrando 116

2026

Choroidal Cavitations within Optic Nerve Colobomas in CHARGE Syndrome.

Ophthalmology. Retina
2025

Hybrid cavitary disc anomaly - A case series and proposal of a novel classification system.

Indian journal of ophthalmology
2026

Peripapillary Retinoschisis: The Expanded Spectrum and New Insights From Multimodal Imaging.

American journal of ophthalmology
2025

Retinal detachments associated with choroidal colobomas.

Taiwan journal of ophthalmology
2025

North Carolina Macular Dystrophy.

Advances in experimental medicine and biology
2025

Bilateral Iris and Chorioretinal Colobomas in a Child With Suspected Lamb-Shaffer Syndrome.

Ophthalmic surgery, lasers & imaging retina
2024

The Evaluation of Ocular Posterior Segment Findings in 5527 Term Infants Using Smartphone-Based Fundus Imaging.

Journal of ophthalmology
2025

A case report of macular coloboma as an ocular clinical feature in Sturge Weber Syndrome.

International journal of surgery case reports
2025

Bilateral Colobomas Affecting the Iris, Optic Nerve, Choroid, and Retina.

Ophthalmology
2024

Bardet-Biedl syndrome with chorioretinal coloboma: a case series and review of literature.

Ophthalmic genetics
2024

RWC Update: Unstapling the Retina From a Firecracker Injury; Macula-On Retinal Detachment: When To Operate?; Choroidal Coloboma.

Ophthalmic surgery, lasers & imaging retina
2024

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

The EPMA journal
2024

Retinopathy With Variant of Unknown Significance and Atypical Chorioretinal Coloboma in the Setting of Prematurity.

Ophthalmic surgery, lasers & imaging retina
2023

Optic Disc Pit Associated With Choroidal Coloboma: Case Report And Short Review.

Journal of Ayub Medical College, Abbottabad : JAMC
2024

Choroid cavitation associated with macular coloboma. Multimodal study. Image en face.

Archivos de la Sociedad Espanola de Oftalmologia
2023

Outcomes of three-piece rigid scleral fixated intraocular lens implantation in subjects with deficient posterior capsule following complications in manual small incision cataract surgery.

Heliyon
2023

Combined choroidal vitiligo and retinochoroidal coloboma.

BMJ case reports
2023

Ocular blood vessel arrangement in choroidal coloboma.

Eye (London, England)
2024

Unilateral retinitis pigmentosa and macular coloboma with fellow eye pigmented paravenous chorioretinal atrophy.

Clinical & experimental optometry
2023

Retinal manifestations in autosomal recessive MPDZ maculopathy: report of two cases and literature review.

Ophthalmic genetics
2022

Zfp503/Nlz2 Is Required for RPE Differentiation and Optic Fissure Closure.

Investigative ophthalmology & visual science
2022

Fundus Changes in the Offspring of Mothers With Confirmed Zika Virus Infection During Pregnancy in French Guiana, Guadeloupe, and Martinique, French West Indies.

JAMA ophthalmology
2022

CONGENITAL POSTERIOR POLAR CHORIORETINAL HYPOPLASIA: Expansion of the Clinical Spectrum, Mutation, and Its Association With PRDM13.

Retina (Philadelphia, Pa.)
2022

Coloboma of the retina, choroid and iris co-existing with cardiac & Skeletal anomalies in a male Nigerian: A case of noonan syndrome.

Nigerian journal of clinical practice
2022

Findings of ocular examinations in healthy full-term newborns.

Arquivos brasileiros de oftalmologia
2022

Choroidal coloboma with internal limiting membrane folds: A rare association.

Indian journal of ophthalmology
2022

Coloboma associated retinal detachment: Anatomical and functional results in the era of microincision vitrectomy surgery with an evaluation of risk factors for a recurrence.

Indian journal of ophthalmology
2022

Clinical and Demographic Profile of Uveal Coloboma: A Hospital-Based Study of 14,371 Eyes of 9557 Indian Patients.

American journal of ophthalmology
2022

Atypical and extensive combined irido-retinochoroidal coloboma with microcornea.

BMJ case reports
2022

The Vascular Pattern In Vicinity Of Chorioretinal Coloboma: An Optical Coherence Tomography Angiography Study.

Ophthalmic genetics
2022

Novel insights into chorioretinal and juxtapapillary colobomas by optical coherence tomography.

Veterinary ophthalmology
2022

Review of evidence for environmental causes of uveal coloboma.

Survey of ophthalmology
2021

Ocular Coloboma With Choroidal Neovascular Membrane: A Case Report.

Cureus
2022

Risk of Retinal Detachment in Children with Ocular Coloboma.

Ophthalmology
2021

Optic disc pit with multiquadrant peripapillary retinoschisis and choroidal coloboma.

BMJ case reports
2022

Atypical superonasal iris, lens and retino-choroidal coloboma.

Clinical & experimental optometry
2021

North Carolina Macular Dystrophy: Phenotypic Variability and Computational Analysis of Disease-Associated Noncoding Variants.

Investigative ophthalmology & visual science
2021

Ocular coloboma-a comprehensive review for the clinician.

Eye (London, England)
2021

Confirming and expanding the phenotypes of FZD5 variants: Coloboma, inferior chorioretinal hypoplasia, and high myopia.

Molecular vision
2021

Intravitreal ranibizumab for the management of serous maculopathy secondary to optic disc coloboma-associated choroidal neovascularisation.

BMJ case reports
2021

[Clinical phenotype and analysis of CHD7 gene variants in three children patients with CHARGE syndrome].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2021

Structure of the Retinal Margin and Presumed Mechanism of Retinal Detachment in Choroidal Coloboma.

Ophthalmology. Retina
2020

Ocular coloboma: Genetic variants reveal a dynamic model of eye development.

American journal of medical genetics. Part C, Seminars in medical genetics
2021

Clinical spectrum of non-syndromic microphthalmos, anophthalmos and coloboma in the paediatric population: a multicentric study from North India.

The British journal of ophthalmology
2020

Compound heterozygous splicing CDON variants result in isolated ocular coloboma.

Clinical genetics
2020

Secondary intrachoroidal cavitation in a case of iridofundal coloboma.

Medical hypotheses
2020

Build Your Own Eye: A Method for Teaching Ocular Anatomy and Pathophysiology.

Journal of education & teaching in emergency medicine
2020

A rare case of temporal atypical retinochoroidal coloboma associated with posterior embryotoxon.

Indian journal of ophthalmology
2020

Mitf-family transcription factor function is required within cranial neural crest cells to promote choroid fissure closure.

Development (Cambridge, England)
2020

[Unilateral chorio retinal coloboma].

Journal francais d'ophtalmologie
2021

Retinal astigmatism induced by a chorioretinal coloboma?

Clinical & experimental optometry
2021

Chorioretinal colobomas in neonatal intensive care using ocular coherence tomography.

Clinical & experimental optometry
2020

A Syrian patient with Steel syndrome due to compound heterozygous COL27A1 mutations with colobomata of the eye.

American journal of medical genetics. Part A
2020

Pars-plana vitrectomy with phacofragmentation for hyperdense cataracts in eyes with severe microcornea and chorio-retinal coloboma: A novel approach.

Indian journal of ophthalmology
2020

Clinical Spectrum and Long-Term Anatomical and Functional Outcomes of Pars Plana Vitrectomy in Retinal Detachments Associated With Choroidal Coloboma.

Journal of vitreoretinal diseases
2020

Multicolour imaging in retinochoroidal coloboma.

Clinical & experimental optometry
2020

Swept-Source OCT Analysis of the Margin of Choroidal Coloboma: New Insights.

Ophthalmology. Retina
2019

Goltz syndrome: Primary diagnosis by an ophthalmologist.

Indian journal of ophthalmology
2019

Rare association of juvenile retinoschisis with retinochoroidal coloboma.

BMJ case reports
2019

Hyperacute spontaneous closure of a traumatic macular hole in a colobomatous eye.

American journal of ophthalmology case reports
2019

A Study on Pattern of Retinal Detachment in Patients with Choroidal Coloboma and Its Outcome after Surgery at a Tertiary Eye Hospital in Nepal.

Journal of ophthalmology
2018

Atypical Choroidal Coloboma.

Ophthalmology. Retina
2019

Zebrafish mab21l2 mutants possess severe defects in optic cup morphogenesis, lens and cornea development.

Developmental dynamics : an official publication of the American Association of Anatomists
2019

A rare case of bilateral choroidal coloboma within deep posterior staphyloma associated with macular hole retinal detachment.

Indian journal of ophthalmology
2019

Retinal capillary hemangioblastoma associated with retinochoroidal coloboma in Von Hippel-Lindau disease.

Indian journal of ophthalmology
2019

Visualization of the Superior Ocular Sulcus during Danio rerio Embryogenesis.

Journal of visualized experiments : JoVE
2019

[Congenital abnormalities of the optic disc].

Journal francais d'ophtalmologie
2019

Familial exudative vitreoretinopathy in a patient with choroidal coloboma.

BMJ case reports
2018

[Bilateral chorioretinian colobome: about a case].

The Pan African medical journal
2019

Macular Colobomata: Comparison of Clinical and Optical Coherence Tomography Features With Serologic Results.

American journal of ophthalmology
2018

Bilateral congenital macular coloboma: Swept-source optical coherence tomography findings.

La Tunisie medicale
2018

Atypical superior iris and retinochoroidal coloboma.

Indian journal of ophthalmology
2018

Association of giant retinal tear with iridofundal coloboma in a case of paediatric retinal detachment.

BMJ case reports
2018

[Bilateral iris coloboma].

The Pan African medical journal
2018

Fundus examination of 199 851 newborns by digital imaging in China: a multicentre cross-sectional study.

The British journal of ophthalmology
2021

SUCCESSFUL SURGICAL OUTCOME OF FULL-THICKNESS MACULAR HOLE WITH CHOROIDAL COLOBOMA.

Retinal cases & brief reports
2018

Intercalary membrane as the inner wall overlying optic and chorio-retinal colobomas. Deep penetration Swept Source-OCT study.

Indian journal of ophthalmology
2018

The cellular bases of choroid fissure formation and closure.

Developmental biology
2018

Optical Coherence Tomography Angiography of Pigmented Paravenous Retinochoroidal Atrophy.

Ophthalmic surgery, lasers & imaging retina
2018

Low-vision aids improve the visual performance of children with bilateral chorioretinal coloboma.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2018

A case of exudative vitreoretinopathy and chorioretinal coloboma associated with microcephaly in a female with contiguous Xp11.3-11.4 deletion.

Ophthalmic genetics
2018

Cell Behaviors during Closure of the Choroid Fissure in the Developing Eye.

Frontiers in cellular neuroscience
2018

Atypical Retinal Lamination Pattern Associated with Bilateral Chorioretinal Coloboma.

Ophthalmology. Retina
2018

Retino-choroidal coloboma: Study through retinography, fluorescence angiography, ecography and optical coherence tomography.

Archivos de la Sociedad Espanola de Oftalmologia
2018

Multi-modality imaging findings of huge intrachoroidal cavitation and myopic peripapillary sinkhole.

BMC ophthalmology
2018

Ocular and uteroplacental pathology in a macaque pregnancy with congenital Zika virus infection.

PloS one
2019

OUTCOMES OF VITRECTOMY WITH SILICONE OIL TAMPONADE FOR MANAGEMENT OF RETINAL DETACHMENT IN EYES WITH CHORIORETINAL COLOBOMA.

Retina (Philadelphia, Pa.)
2017

Endoscopic Vitrectomy for Microcornea, Posterior Megalolenticonus, Persistent Fetal Vasculature, Coloboma Syndrome.

Ophthalmology
2017

Ophthalmic findings in Frank-ter Haar syndrome: report of a sibling pair.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2017

Congenital anomalies of the optic disc: insights from optical coherence tomography imaging.

Current opinion in ophthalmology
2017

Aborted choroidal coloboma: fundus imaging and optical coherence tomography.

BMJ case reports
2017

Zebrafish zic2 controls formation of periocular neural crest and choroid fissure morphogenesis.

Developmental biology
2017

Pediatric Choroidal Coloboma with Macular Hole at the Edge of the Coloboma.

Ophthalmology
2019

SOLITARY RETINAL CAPILLARY HEMANGIOMA IN A PATIENT WITH BILATERAL CHORIORETINAL COLOBOMA.

Retinal cases & brief reports
2017

Chorioretinal Coloboma Complications: Retinal Detachment and Choroidal Neovascular Membrane.

Journal of ophthalmic & vision research
2017

SURGICAL OUTCOMES AND COMPLICATIONS OF RHEGMATOGENOUS RETINAL DETACHMENT IN EYES WITH CHORIORETINAL COLOBOMA: The Results of the KKESH International Collaborative Retina Study Group.

Retina (Philadelphia, Pa.)
2016

Coexistence of optic pit and coloboma of iris, lens, and choroid: a case report.

Arquivos brasileiros de oftalmologia
2017

Cataract, strabismus and chorioretinal coloboma in paediatric HIV infection.

Journal of optometry
2017

Spontaneous rupture of chorioretinal coloboma in an 8-year-old child is treated by temporal fascia graft.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2016

Complex limbal choristoma in linear nevus sebaceous syndrome managed with scleral grafting.

Indian journal of ophthalmology
2016

Utility of optical coherence tomography in a case of bilateral congenital macular coloboma.

Indian journal of ophthalmology
2016

Atypical chorioretinal coloboma in a Golden Retriever: a retinographic, fluoroangiographic, and optical coherence tomography study.

Veterinary ophthalmology
2017

Association of Pediatric Choroidal Neovascular Membranes at the Temporal Edge of Optic Nerve and Retinochoroidal Coloboma.

American journal of ophthalmology
2016

Hypotonic maculopathy secondary to scleral defect in atypical retinochoroidal coloboma.

European journal of ophthalmology
2016

Chorioretinal coloboma in a patient with pancreas divisum: clinical and imaging features.

European journal of ophthalmology
2017

[Optic disc pit-associated maculopathy and iris-retinochoroidal-coloboma - a rare combination].

Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
2016

A case of iridofundal coloboma with persistent fetal vasculature and lens subluxation.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2017

A NEW TECHNIQUE WITH AUTOLOGOUS FIBRIN FOR THE TREATMENT OF PERSISTENT OPTIC PIT MACULOPATHY.

Retinal cases & brief reports
2016

Antivascular Endothelial Growth Factor Monotherapy for Choroidal Neovascularization Associated With Retinochoroidal Coloboma: Case Series.

Asia-Pacific journal of ophthalmology (Philadelphia, Pa.)
2016

Ocular Findings in Infants With Microcephaly Associated With Presumed Zika Virus Congenital Infection in Salvador, Brazil.

JAMA ophthalmology
2015

Yap and Taz regulate retinal pigment epithelial cell fate.

Development (Cambridge, England)
2015

[Bilateral chorio-retinal coloboma].

Journal francais d'ophtalmologie
2015

Reticular Pseudodrusen in Sorsby Fundus Dystrophy.

Ophthalmology
2015

Clinical and Echographic Features of Retinochoroidal and Optic Nerve Colobomas.

Investigative ophthalmology & visual science
2015

Spontaneous resealing of perforated scleral ectasia associated with atypical retinochoroidal coloboma.

Clinical & experimental ophthalmology
2015

Sox4 regulates choroid fissure closure by limiting Hedgehog signaling during ocular morphogenesis.

Developmental biology

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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. Peripapillary Retinoschisis: The Expanded Spectrum and New Insights From Multimodal Imaging.
    American journal of ophthalmology· 2026· PMID 41110675mais citado
  2. Choroidal Cavitations within Optic Nerve Colobomas in CHARGE Syndrome.
    Ophthalmology. Retina· 2026· PMID 41677486mais citado
  3. Hybrid cavitary disc anomaly - A case series and proposal of a novel classification system.
    Indian journal of ophthalmology· 2025· PMID 41148017mais citado
  4. Retinal detachments associated with choroidal colobomas.
    Taiwan journal of ophthalmology· 2025· PMID 40995329mais citado
  5. North Carolina Macular Dystrophy.
    Advances in experimental medicine and biology· 2025· PMID 40736823mais citado
  6. Bilateral Iris and Chorioretinal Colobomas in a Child With Suspected Lamb-Shaffer Syndrome.
    Ophthalmic Surg Lasers Imaging Retina· 2025· PMID 40163633recente
  7. Bardet-Biedl syndrome with chorioretinal coloboma: a case series and review of literature.
    Ophthalmic Genet· 2024· PMID 39402987recente
  8. RWC Update: Unstapling the Retina From a Firecracker Injury; Macula-On Retinal Detachment: When To Operate?; Choroidal Coloboma.
    Ophthalmic Surg Lasers Imaging Retina· 2024· PMID 38466966recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:98942(Orphanet)
  2. MONDO:0020354(MONDO)
  3. GARD:16875(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q56014418(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

Coloboma da coroide e retina
Compêndio · Raras BR

Coloboma da coroide e retina

ORPHA:98942 · MONDO:0020354
CID-10
Q14.8 · Outras malformações congênitas da câmara posterior do olho
CID-11
MedGen
UMLS
C0240896
Wikidata
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