Epignathus
Synonyms: Oropharyngeal teratoma
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Antenatal, Neonatal
- Status
- active
Epignathus is a very rare and life threatening intraoral teratoma, usually arising from the maxilla, mandible, palate or base of skull and invading the cranium, nasopharynx or oral cavity. Epignathus is more commonly seen in females, and presents with various manifestations (depending on the tumor size) including obstructive polyhydramnios in the prenatal period and dyspnea, cyanosis, cough, difficulty in sucking and swallowing, and rarely vomiting (due to swallowing difficulties) postnatally. When large, they can lead to airway obstruction, asphyxia and death in the neonatal period.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Epignathus | Oropharyngeal teratoma |
| fr | Tératome épignathe | Tératome oropharyngé mature |
| de | Epignathus | |
| es | Epignato | Teratoma orofaríngeo |
| it | Epignato | |
| nl | Epignathus | Orofaryngeaal teratoom |
| pt | Epignato | |
| pl | Epignatus - guz z tkanek podniebienia | Potworniak ustno-gardłowy |
| cs | Epignathus | Orofaryngeální teratom |
| uk | Епігнатус | Тератома ротоглотки |
| zh | 上颌寄生胎畸胎 | 口咽畸胎瘤 |
| tr | Epignatus | Orofarengeal teratom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D37.0 | NTBT | attributed | validated |
| ICD-11 | LA70.Y | NTBT | index | validated |
| MONDO | 0015386 | E | validated | |
| UMLS | C0266725 | 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 (2):
- ORPHA:883Extragonadal teratoma
rare neoplastic diseases; rare transplant-related disorders - ORPHA:98061Rare otorhinolaryngologic tumor
rare otorhinolaryngological diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | <1 / 1 000 000 | 0.0017 / 100 000 | Worldwide | Value and class | Validated | 21701667[PMID]_ORPHANET |
| Prevalence at birth | 1-9 / 100 000 | 1.68 / 100 000 | Worldwide | Value and class | Validated | ORPHANET_21701667[PMID] |
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/141077
- This record as JSON: /data/d/141077.json