Limited dorsal myeloschisis
Synonyms: LDM
- Type
- Clinical group
- Group
- Group of disorders
- Status
- active
A rare dysraphic abnormality characterized by a persistent connection between the neural tissue and overlying skin. The stalk-like connection consists of a fibroneural tract (mainly composed of fibrous attenuated mesenchymal tissue and neural elements without an epithelial lining) connecting the skin lesion to the underlying dorsal surface of the spinal cord. Fibroneural stalk varies in thickness and complexity and they pass through the deep fascia, a bifid lamina/ the interspinous ligament, and the dura. It can be associated with filum anomaly. Chiari II malformation is not present.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Limited dorsal myeloschisis | LDM |
| fr | Myéloschisis dorsal limité | = en |
| de | Begrenzte dorsale Myeloschisis | = en |
| es | Mielosquisis dorsal limitada | MDL |
| it | Mieloschisi dorsale limitata | = en |
| nl | Beperkte dorsale myeloschisis | = en |
| pt | Mielosquisis dorsal limitada | = en |
| pl | Ograniczone grzbietowe rozszczepienie rdzenia | Ograniczony grzbietowy rozszczep rdzenia |
| cs | Omezená dorzální myeloschíza | = en |
| uk | Обмежений дорсальний мієлошизис | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| UMLS | C5781237 | 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
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/645196
- This record as JSON: /data/d/645196.json