ORPHA:71276
Silent sinus syndrome
Synonyms: CMA grade 3 · Chronic maxillary atelectasis grade III · Imploding antrum syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare otorhinolaryngologic disease characterized by adult-onset progressive enophthalmos due to collapse of some or all of the maxillary sinus walls and remodeling of the orbital floor.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Silent sinus syndrome | CMA grade 3 · Chronic maxillary atelectasis grade III · Imploding antrum syndrome |
| fr | Syndrome du sinus silencieux | Atélectasie maxillaire chronique de grade III · CMA grade 3 · Collapsus progressif du sinus maxillaire · Syndrome "silent sinus" |
| de | Silent-sinus-Syndrom | Chronische maxilläre Atelektase Grad III · Enophthalmus und Hypotropie durch Kieferhöhlenatelektase · Syndrom des implodierenden Antrums |
| es | Síndrome del seno silente | AMC grado III · Atelectasia maxilar crónica grado III · Síndrome de implosión maxilar |
| it | Sindrome del seno silente | CMA grado 3 · Sindrome da collasso dell'antro · Atelettasia mascellare cronica di grado III |
| nl | Silent sinus-syndroom | CMA graad 3 · Chronische maxillaire atelectase graad III · Imploding antrum-syndroom |
| pt | Síndrome seio silencioso | Síndrome de implosão |
| pl | Zespół cichej zatoki | = en |
| cs | Silent sinus syndrom | Syndrom zhroucení předsíně |
| uk | Синдром мовчазного синуса | Синдром вибухового антруму |
| zh | 隐匿性鼻窦综合征 | 隐匿性鼻窦综合征 |
| tr | Sessiz sinüs sendromu | İç içe geçmiş antrum sendromu |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J34.8 | NTBT | attributed | validated |
| ICD-11 | CA0B | E | specific | validated |
| MONDO | 0019108 | E | validated | |
| UMLS | C3698095 | E | validated | |
| MedDRA | 10075540 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:98036Rare otorhinolaryngologic disease
rare otorhinolaryngological diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 558 cases | Worldwide | Case(s) | Validated | 37219052[PMID] | |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | 37219052[PMID]_ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
3 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000490 | Deeply set eye | |
| Occasional (29-5%) | ||
| HP:0000478 | Abnormality of the eye | |
| HP:0000504 | Abnormality of vision | |
Run the differential on the 1 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/71276
- This record as JSON: /data/d/71276.json