Spinal dermal sinus
Synonyms: Dermal sinus tract
- Type
- Morphological anomaly
- Group
- Disorder
- Inheritance
- Unknown
- Status
- active
A rare closed dysraphism with stalk characterized by a dorsal midline dermal sinus tract lined by keratinizing stratified squamous epithelium extending to the the intrathecal space. Other components such as hair follicles and shafts, mesenchymal derivatives (blood vessels and fibrous tissue) and occasionally nerve fibers can be observed. Inflamed granulation tissue containing mixed neutrophils, plasma cells, lymphocytes, and histiocytes is consistently found in the tract. It can also be associated with an intradural dermoid cyst. This malformation is at risk to cause intrathecal infections (meningitis, empyema) that justify a prophylactic surgery.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Spinal dermal sinus | Dermal sinus tract |
| fr | Sinus dermique spinal | Sinus dermique à tractus |
| de | Spinaler Dermalsinus | |
| es | Seno dérmico espinal | Tracto del seno dérmico |
| it | Seno dermico spinale | Tratto sinusale del derma |
| nl | Spinale dermale sinus | Dermale fistel |
| pt | Sinus dérmico espinhal | Tracto de sinus dérmico |
| pl | Zatoka skórna kręgosłupa | = en |
| cs | Spinální dermální sinus | = en |
| uk | Спинномозковий шкірний синус | Шкірно-синусний тракт |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q76.0 | NTBT | attributed | validated |
| MONDO | 0958344 | E | validated | |
| MONDO | 958344 | E | validated | |
| UMLS | C5706141 | 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:645193Dysraphism with stalk
rare developmental anomalies during embryogenesis; rare genetic diseases; rare neurological 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/645188
- This record as JSON: /data/d/645188.json