Terrien marginal degeneration
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare disorder of the anterior segment of the eye characterized by slowly progressive, bilateral, asymmetric, usually non-inflammatory degeneration of the peripheral cornea, resulting in stromal thinning, vascularization, lipid deposition, and against-the-rule astigmatism with decreased visual acuity. Degeneration typically involves the superior aspect of the cornea first and extends circumferentially, leading to circumferential ectasia of the peripheral cornea. Opacification of the central cornea may occur at a very advanced stage. In rare cases, the condition is complicated by perforation.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Terrien marginal degeneration | |
| fr | Dégénérescence marginale de Terrien | |
| de | Marginale Degeneration Terrien | Terrien's marginale Hornhautdegeneration |
| es | Degeneración marginal de Terrien | |
| it | Degenerazione marginale di Terrien | |
| nl | Marginale degeneratie van Terrien | |
| pt | Degeneração marginal Terrien | |
| pl | Zwyrodnienie brzeżneTerriena | |
| cs | Terrienova marginální degenerace | |
| uk | Террієнова маргінальна дегенерація | |
| zh | 特里昂边缘变性 | |
| tr | Terrien marjinal dejenerasyonu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H18.4 | NTBT | attributed | validated |
| ICD-11 | 9A78.4 | NTBT | attributed | validated |
| MONDO | 0033818 | E | validated | |
| UMLS | C0271283 | E | validated | |
| MedDRA | 10043286 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:519288Rare disorder with corneal involvement as a major feature
rare ophthalmic disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/519410
- This record as JSON: /data/d/519410.json