Glioependymal/ependymal cyst
- Type
- Morphological anomaly
- Group
- Disorder
- Age of onset
- Infancy, Neonatal
- Status
- active
Glioependymal/ependymal cyst is a rare central nervous system malformation defined as a subarachnoid, supratentorial, interventricular or intraspinal, sometimes intracerebral or intramedullar cyst with an internal ependymal lining, possibly surrounded by glial tissue. It may be an incidental finding or may present at different ages with clinical features depending on its size and location. It may distort adjacent brain structures and cause macrocephaly, ventriculomegaly, hydrocephalus, focal neurological signs and other signs and symptoms. In some cases, it is associated with other cerebral malformations (e.g. corpus callosum agenesis, polymicrogyria, heterotopias).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Glioependymal/ependymal cyst | |
| fr | Kyste glioépendymaire/épendymaire | |
| de | Glioependymale/ependymale Zyste | |
| es | Quiste glioependimario/ependimario | |
| it | Cisti glioependimale/ependimale | |
| nl | Glio-ependymale/ependymale cyste | |
| pt | Quisto glioependimário/ependimário | |
| pl | Torbiel gleju wyściółkowego/torbiel wyściółkowa | |
| cs | Glioependymální/ependymální cysta | |
| uk | Гліоепендимальна/епендимальна кіста | |
| zh | 胶质室管膜/室管膜囊肿 | |
| tr | Glioependimal / ependimal kist |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G93.0 | NTBT | attributed | validated |
| ICD-11 | LA05.7 | NTBT | index | validated |
| MONDO | 0017105 | E | validated | |
| UMLS | C0338599 | 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:269194Central nervous system cystic malformation
rare developmental anomalies during embryogenesis; rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/269197
- This record as JSON: /data/d/269197.json