Paramedian nasal cleft
Synonyms: Alar cleft · Alar rim cleft · Cleft nose · Isolated cleft of the ala nasi · Isolated coloboma of the nose · Tessier number 1 cleft
- Type
- Morphological anomaly
- Group
- Disorder
- Age of onset
- Antenatal, Neonatal
- Status
- active
Paramedian nasal cleft is a rare developmental defect during embryogenesis characterized by a unilateral or bilateral coloboma of the nose, ranging in severity from a small notch, resulting in minor deviation of the nasal septum, to variable-sized clefts of the nasal ala which may be associated with small cysts or sinuses in the nasal midline. Defect may be isolated or may occur in association with cleft lip and/or other craniofacial anomalies (e.g. hypertelorism, broadening of nasal root, midline cleft). Dorsum and apex of nose are usually well preserved.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Paramedian nasal cleft | Alar cleft · Alar rim cleft · Cleft nose · Isolated cleft of the ala nasi · Isolated coloboma of the nose · Tessier number 1 cleft |
| fr | Fente nasale paramédiane | Fente du nez · Fente faciale 1 de Tessier à l'étage nasal |
| de | Nasenspalte, paramediane | Nasenkolobom, isoliertes · Nasenspalte · Gesichtsspalte, Tessier 1 · Nasenflügelrand-Spalte · Nasenflügelspalte · Nasenflügelspalte, isolierte |
| es | Hendidura nasal paramediana | Coloboma aislado de la nariz · Fisura número 1 de Tessier · Hendidura aislada del ala nasal · Hendidura alar · Hendidura de la rima alar · Hendidura del borde alar · Hendidura número 1 de Tessier · Nariz hendida |
| it | Schisi nasale paramediana | |
| nl | Paramediane nasale spleet | Aangezichtsspleet Tessier nummer 1 · Gespleten neus · Geïsoleerd coloboom van de neus · Geïsoleerde spleet van de neusvleugel · Neusvleugelspleet · Spleet van de neusvleugelrand |
| pt | Fenda nasal | |
| pl | Przyśrodkowy rozszczep nosa | Rozszczep nosa · Rozszczep twarzy numer 1 wg Tessiera |
| cs | Paramediální rozštěp nosu | Rozštěp číslo 1 dle Tessiera |
| uk | Парамедіанна назальна розщілина | Розщілина Алар · Розщілина обода Алара · Розщілина носа · Ізольована розщілина ала-насі · Ізольована колобома носа · Розщілина Тессьє № 1 |
| zh | 旁正中鼻裂 | 孤立性鼻翼裂 |
| tr | Paramedyan nazal yarık | Alar yarık · Yarık burun · Alar ağız yarığı · Tessier 1 numaralı yarık · Burun izole kolobomu · Ala nasi'nin izole yarığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 614687 | E | validated | |
| ICD-10 | Q18.8 | NTBT | attributed | validated |
| ICD-11 | LA51 | NTBT | index | validated |
| MONDO | 0015414 | E | validated | |
| UMLS | C4759655 | 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:155867Paramedian facial cleft
rare developmental anomalies during embryogenesis; rare surgical maxillo-facial diseases
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/141242
- This record as JSON: /data/d/141242.json