Fourth branchial cleft anomaly
Synonyms: Fourth branchial cleft cyst · Fourth branchial cleft fistula
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
A rare otorhinolaryngeal malformation characterized by a soft, fluctuant mass, abscess or draining tract along the anterior border of the lower half of sternocleidomastoid muscle, occasionally leading to development of retropharyngeal abscess, acute suppurative thyroiditis, stridor, respiratory distress, odynophagia, and dysphagia. Anomaly occurs as a tract from the piriform sinus to the thyroid gland. A fourth branchial cleft fistula passes deep to the superior laryngeal nerve but superficial to the recurrent laryngeal nerve, which is the main difference in comparison to the third branchial cleft fistula.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Fourth branchial cleft anomaly | Fourth branchial cleft cyst · Fourth branchial cleft fistula |
| fr | Anomalie de la quatrième fente branchiale | Fistule de la quatrième fente branchial · Kyste de la quatrième fente branchiale |
| de | Kiemenbogen, 4., Anomalie des | |
| es | Anomalía de la cuarta hendidura branquial | Fístula de la cuarta hendidura branquial · Quiste de la cuarta hendidura branquial |
| it | Schisi del quarto arco branchiale | |
| nl | Anomalie van vierde kieuwspleet | Cyste van vierde kieuwspleet · Fistel van vierde kieuwspleet |
| pt | Anomalia da quarta fenda branquial | |
| pl | Anomalia rozszczepowa czwartego łuku skrzelowego | Przetoka z rozszczepem czwartego łuku skrzelowego · Torbiel z rozszczepem czwartego łuku skrzelowego |
| cs | Anomálie čtvrté branchiální štěrbiny | Cysta čtvrté branchiální štěrbiny · Píštěl čtvrté branchiální štěrbiny |
| uk | Аномалія четвертої зябрової щілини | Четверта гілкова щілинна кіста · Четверта гілкова нориця |
| zh | 第四鳃裂畸形 | 第四鳃裂瘘管 |
| tr | Dördüncü brankial yarık anomalisi | Dördüncü branşiyal yarık kisti · Dördüncü branşiyal yarık fistülü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q18.0 | NTBT | attributed | validated |
| MONDO | 0015378 | E | validated | |
| UMLS | C3873490 | E | validated | |
| GARD | 19936 | 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:155835Cysts and fistulae of the face and oral cavity
rare developmental anomalies during embryogenesis; rare otorhinolaryngological 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/141037
- This record as JSON: /data/d/141037.json