Lipomatous non-saccular limited dorsal myeloschisis
Synonyms: Lipomatous flat LDM · Lipomatous flat limited dorsal myeloschisis · Lipomatous non-saccular LDM
- Type
- Histopathological subtype
- Group
- Subtype of disorder
- Inheritance
- Unknown
- Status
- active
A rare non-saccular limited dorsal myeloschisis histologically characterized by skin tissue with a jagged depression of squamous epithelium continued to subcutaneous mature adipose tissue with loose connective tissue. GFAP-immunopositive glial cells are embedded in the subcutaneous lipoma. The extradural and intradural stalk have cord-like fibrocollagenous tissue containing adipose tissue, peripheral nerve fibers, clusters of melanocytes, and skeletal muscle fibers.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Lipomatous non-saccular limited dorsal myeloschisis | Lipomatous flat LDM · Lipomatous flat limited dorsal myeloschisis · Lipomatous non-saccular LDM |
| fr | Myéloschisis dorsal limité non-sacculaire de type lipomateux | LDM non-sacculaire lipomateux · LDM plat lipomateux · Myéloschisis dorsal limité plat de type lipomateux |
| de | Lipomatöse nicht-sakkuläre begrenzte dorsale Myeloschisis | Lipomatöse flache begrenzte dorsale Myeloschisis · Lipomatöse nicht-sakkuläre LDM · Lipomatöses flache LDM |
| es | Mielosquisis dorsal limitada no sacular lipomatosa | MDL no sacular lipomatosa · MDL plana lipomatosa · Mielosquisis dorsal limitada plana lipomatosa |
| it | Mieloschisi lipomatosa non sacculare limitata dorsale | LDM lipomatoso non sacculare · LDM lipomatoso piatto · Mieloschisi lipomatosa piatta limitata al dorso |
| nl | Lipomateuze niet-sacculaire beperkte dorsale myeloschisis | Lipomateuze niet-sacculaire LDM · Lipomateuze platte LDM · Lipomateuze platte beperkte dorsale myeloschisis |
| pt | Mielosquisis lipomatosa não-sacular dorsal limitada | LDM lipomatosa não-sacular · LDM lipomatosa plana · Mielosquisis lipomatosa plana dorsal limitada |
| pl | Tłuszczakowaty nieworkowaty ograniczony rozszczep grzbietowej części rdzenia | = en |
| cs | Lipomatózní ohraničená dorzální myeloschíza bez vaku | = en |
| uk | Ліпоматозний несаккулярний обмежений дорсальний мієлошизис | Ліпоматозний плоский LDM · Ліпоматозний плоский обмежений дорсальний мієлошизис · Ліпоматозна несакральна LDM |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0970960 | E | validated | |
| UMLS | C5816723 | 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:645343Non-saccular limited dorsal myeloschisis
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/645300
- This record as JSON: /data/d/645300.json