Macrocystic lymphatic malformation
Synonyms: Cavernous lymphangioma · Cavernous lymphatic malformation · Macrocystic lymphangioma
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Infancy, Neonatal
- Status
- active
A rare common cystic lymphatic malformation characterized by a benign cystic lesion composed of dilated lymphatic channels. Macrocystic lesions consist of cysts larger than 1 cm in diameter. They usually present at birth or during the first years of life and most often occur in the head and neck region but may affect any site. Symptoms depend on the location and extent of the lesion. Infection, trauma, or intracystic hemorrhage can lead to lesional expansion. Malignant transformation does not occur.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Macrocystic lymphatic malformation | Cavernous lymphangioma · Cavernous lymphatic malformation · Macrocystic lymphangioma |
| fr | Malformation lymphatique macrokystique | Lymphangiome caverneux · Lymphangiome macrokystique · Lymphangiome profond · Malformation lymphatique caverneuse · Malformation lymphatique profonde |
| de | Lymphatische Malformation, makrozystische | Kavernöse lymphatische Fehlbildung · Lymphangiom, kavernöses · Lymphangiom, makrozystisches |
| es | Malformación linfática macroquística | Linfangioma cavernoso · Linfangioma macroquístico · Malformación linfática cavernosa |
| it | Malformazione linfatica macrocistica | Linfangioma cavernoso · Linfangioma macrocistico · Malformazione linfatica cavernosa |
| nl | Macrocystische lymfatische malformatie | Macrocysteuze lymfatische malformatie · Macrocysteus lymfangioom · Caverneus lymfangioom · Caverneuze lymfatische malformatie · Macrocystisch lymfangioom |
| pt | Malformação linfática macroquística | |
| pl | Malformacja limfatyczna makrotorbielowata | Jamista malformacja limfatyczna · Macrocystic lymphangioma · Naczyniak limfatyczny jamisty |
| cs | Makrocystická lymfatická malformace | Kavernózní lymfangiom · Kavernózní lymfatická malformace · Makrocystický lymfangiom |
| uk | Макрокістозна лімфатична мальформація | Кавернозна лімфангіома · Кавернозна лімфатична мальформація · Макрокістозна лімфангіома |
| zh | 海绵状淋巴管畸形 | 海绵状淋巴管畸形 |
| tr | Makrokistik lenfatik malformasyon | Kavernöz lenfanjiyom · Makrokistik lenfanjiyom · Kavernöz lenfatik malformasyon |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D18.1 | NTBT | attributed | validated |
| ICD-11 | LA90.10 | E | specific | validated |
| MONDO | 0019328 | E | validated | |
| UMLS | C0205828 | E | validated | |
| GARD | 6010 | 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:458833Isolated lymphatic malformation
rare developmental anomalies during embryogenesis; rare genetic diseases; rare circulatory system diseases; rare otorhinolaryngological diseases; rare surgical maxillo-facial diseases
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:79486Cystic hygroma moved to · inactive
- ORPHA:217410OBSOLETE: Circumscribed lymphatic malformation referred to · inactive
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/79489
- This record as JSON: /data/d/79489.json