Congenital temporomandibular joint ankylosis
Synonyms: Congenital trismus
- Type
- Disease
- Group
- Disorder
- Status
- active
Congenital temporomandibular joint ankylosis is a rare maxillofacial disorder characterized by significant reduction in mouth opening (i.e. from a few millimeters to a few centimeters) in the absence of acquired factors (e.g. trauma, infection) contributing to the ankylosis. It is associated with variable degrees of facial dysmorphism (i.e. lateral deviation of the mandible and chin, lower facial asymmetry, retrognathia, micrognathia, dental malocclusion) and patients typically present with feeding and breathing difficulties. Developmental delay, hypotonia, seizures, and additional dysmorphic features (e.g. pectus excavatum, low-set ears, hypoplastic alae nasi) have also been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital temporomandibular joint ankylosis | Congenital trismus |
| fr | Ankylose congénitale temporo-mandibulaire | Trismus congénital |
| de | Kiefergelenkankylose, kongenitale | Trismus, kongenitaler |
| es | Anquilosis congénita de la articulación temporomandibular | Trismus congénito |
| it | Anchilosi congenita dell'articolazione temporomandibolare | Trisma congenito |
| nl | Congenitale ankylose van temporomandibulair gewricht | Congenitale ankylose van kaakgewricht · Congenitale trismus |
| pt | Anquilose congénita das articulações temporomandibulares | |
| pl | Wrodzona ankyloza stawu skroniowo-żuchwowego | Szczękościsk wrodzony |
| cs | Vrozená ankylóza temporomandibulárního kloubu | Vrozený trismus |
| uk | Вроджений анкілоз скронево-нижньощелепного суглоба | Вроджений тризм |
| zh | 先天性颞下颌关节强直 | 先天性牙关紧闭症 |
| tr | Doğumsal temporomandibular eklem ankilozu | Doğumsal trismus |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K07.6 | NTBT | attributed | validated |
| MONDO | 0016220 | E | validated | |
| UMLS | C4706319 | 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:210581Temporomandibular joint anomaly
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/210576
- This record as JSON: /data/d/210576.json