Supernumerary nostril
Synonyms: Accessory nostril
- Type
- Malformation syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Antenatal, Neonatal
- Status
- active
Supernumerary nostril is an extremely rare congenital malformation characterized by the presence of one or more accessory nostrils, with or without accessory cartilage, located medially, above, below or laterally to the other nostrils. Unlike in polyrhinia there is no duplication of the nasal septum/cavity. Supernumerary nostril is often associated with other congenital malformations usually of face.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Supernumerary nostril | Accessory nostril |
| fr | Narine surnuméraire | |
| de | Nasenlöcher, überzählige | |
| es | Fosa nasal supernumeraria | Fosas nasales accesorias |
| it | Narice soprannumeraria | Narici accessorie |
| nl | Surnumeraire neusvleugel | Bijkomende neusvleugel |
| pt | Narina supranumerária | |
| pl | Nozdrze nadliczbowe | Nozdrze dodatkowe |
| cs | Nadpočetná nostrila | Akcesorní nostrila |
| uk | Додаткова ніздря | Додаткова ніздря |
| zh | 先天性副鼻畸形 | 副鼻孔 |
| tr | Süpernümerik burun deliği | Aksesuar burun deliği |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q30.8 | NTBT | attributed | validated |
| ICD-11 | LA70.Y | NTBT | index | validated |
| MONDO | 0015389 | E | validated | |
| UMLS | C4021372 | E | validated | |
| GARD | 19946 | 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:156246Nose and cavum anomaly
rare developmental anomalies during embryogenesis; rare otorhinolaryngological 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 |
|---|---|---|---|---|---|---|
| Cases/families | 32 cases | Worldwide | Case(s) | Validated | 23348320[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 23348320[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
7 annotations · validated 2016-11-24| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0009934 | Supernumerary naris | |
| Frequent (79-30%) | ||
| HP:0000271 | Abnormality of the face | |
| Occasional (29-5%) | ||
| HP:0002006 | Facial cleft | |
| Very rare (<4-1%) | ||
| HP:3000040 | Abnormality of ethmoid sinus | |
| HP:0000453 | Choanal atresia | |
| HP:0000519 | Developmental cataract | |
| HP:0000482 | Microcornea | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 23483576[PMID]_25095909[PMID]_22687717[PMID]_16381135[PMID]_22185625[PMID]_15195526[PMID]_5074938[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/141096
- This record as JSON: /data/d/141096.json