Primary condylar hyperplasia
Synonyms: Type 1 condylar hyperplasia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare temporomandibular joint anomaly characterized by progressive, asymmetrical, non-neoplastic overgrowth of a mandibular condyle. It is unilateral in most cases and leads to progressive facial asymmetry, mandibular deviation, articular dysfunction, and dental malocclusion.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary condylar hyperplasia | Type 1 condylar hyperplasia |
| fr | Hyperplasie condylienne primitive | Hyperplasie condylienne type 1 |
| de | Kondyläre Hyperplasie, primäre | Hyperplasie, kondyläre, Typ 1 · Kondylushyperplasie, primäre |
| es | Hiperplasia condilar primaria | Hiperplasia condilar tipo 1 |
| it | Iperplasia condilare primitiva | |
| nl | Primaire condylaire hyperplasie | Condylaire hyperplasie type 1 |
| pt | Hiperplasia condilar primária | |
| pl | Pierwotna hiperplazja kłykcia żuchwy | Hiperplazja kłykcia żuchwy typu 1 |
| cs | Primární kondylární hyperplázie | Type 1 condylar hyperplasia · Hyperplázie kondylů typu 1 |
| uk | Первинна гіперплазія гострих кондилом | Гіперплазія кондилом 1-го типу |
| zh | 原发性踝增生 | 1型踝增生 |
| tr | Primer kondiler hiperplazi | Kondiler hiperplazi Tip 1 |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K07.6 | NTBT | attributed | validated |
| MONDO | 0018793 | E | validated | |
| UMLS | C5568570 | 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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_26629479[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
6 annotations · validated 2021-05-28| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:3000077 | Abnormal mandible condylar process morphology | |
| Frequent (79-30%) | ||
| HP:0000306 | Abnormality of the chin | |
| HP:0010754 | Abnormality of the temporomandibular joint | |
| HP:0009102 | Anterior open-bite malocclusion | |
| HP:0000324 | Facial asymmetry | |
| Occasional (29-5%) | ||
| HP:0001572 | Macrodontia | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 18778456[PMID]_19164410[PMID]_26147813[PMID]_30737324[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/477781
- This record as JSON: /data/d/477781.json