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Familial cavitary optic disk anomaly(CODA)

MedGen UID:
370593
Concept ID:
C1969063
Congenital Abnormality
Synonyms: Cavitary optic disc anomalies; Cavitary optic disc anomaly; Familial cavitary optic disc anomaly
SNOMED CT: Familial CODA (cavitary optic disc anomaly) (1197365006); Familial cavitary optic disc anomaly (1197365006)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Gene (location): MMP19 (12q13.2)
 
Monarch Initiative: MONDO:0012687
OMIM®: 611543
Orphanet: ORPHA464760

Definition

A rare genetic eye disease with characteristics of congenital profound excavation of the optic nerve head with diminished visual field, in the absence of elevated intraocular pressure. Many patients lack a well-formed retinal artery and have multiple radial cilioretinal arteries instead. The condition is mostly bilateral, may worsen progressively, and is often complicated by serous macular detachment with profound visual loss. [from SNOMEDCT_US]

Clinical features

From HPO
Night blindness
MedGen UID:
10349
Concept ID:
C0028077
Disease or Syndrome
Inability to see well at night or in poor light.
Reduced visual acuity
MedGen UID:
65889
Concept ID:
C0234632
Finding
Diminished clarity of vision.
Peripapillary atrophy
MedGen UID:
473480
Concept ID:
C1719838
Pathologic Function
Thinning in the layers of the retina and retinal pigment epithelium around the optic nerve.
Visual field defect
MedGen UID:
854603
Concept ID:
C3887875
Finding
An absolute or relative reduction in the extent of the normal field of vision.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFamilial cavitary optic disk anomaly

Recent clinical studies

Diagnosis

Habib AM, Elkitkat RS, Saleh MI, Abd El-Salam MM
Retina 2016 Feb;36(2):408-14. doi: 10.1097/IAE.0000000000000713. PMID: 26225485
Honkanen RA, Jampol LM, Fingert JH, Moore MD, Taylor CM, Stone EM, Alward WL
Am J Ophthalmol 2007 May;143(5):788-794. Epub 2007 Mar 23 doi: 10.1016/j.ajo.2007.01.029. PMID: 17362864

Clinical prediction guides

Hwang ES, Morgan DJ, Pennington KL, Owen LA, Fingert JH, Bernstein PS, DeAngelis MM
BMC Med Genet 2019 Apr 27;20(1):63. doi: 10.1186/s12881-019-0800-4. PMID: 31029096Free PMC Article
Wang D, Pan X, Ji J, Gu S, Sun X, Jiang C, Xia W, Qiu Z, Kang X, Ding S, Liu Q, Chen X, Lu F, Zhao C
Sci Rep 2017 Aug 10;7(1):7799. doi: 10.1038/s41598-017-07730-7. PMID: 28798362Free PMC Article
Habib AM, Elkitkat RS, Saleh MI, Abd El-Salam MM
Retina 2016 Feb;36(2):408-14. doi: 10.1097/IAE.0000000000000713. PMID: 26225485
Fingert JH, Honkanen RA, Shankar SP, Affatigato LM, Ehlinger MA, Moore MD, Jampol LM, Sheffield VC, Stone EM, Alward WL
Am J Ophthalmol 2007 May;143(5):795-800. Epub 2007 Mar 19 doi: 10.1016/j.ajo.2007.01.042. PMID: 17368552Free PMC Article
Slusher MM, Weaver RG Jr, Greven CM, Mundorf TK, Cashwell LF
Ophthalmology 1989 Mar;96(3):342-7. doi: 10.1016/s0161-6420(89)32886-7. PMID: 2710526

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