Congenital stenosis of the inferior vena cava
Synonyms: Congenital stenosis of the IVC · Congenital stenosis of the inferior caval vein
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
A rare vascular anomaly characterized by congenital narrowing of the inferior vena cava mostly at the diaphragmatic level or hepatic segment, with or without web formation. Patients may present with deep vein thrombosis below the obstructed segment as well as swelling, pain, and varices of the lower extremities, abdominal pain/varices, or hematochezia. Presence of collateral veins between upper and lower segments of the stenosis, as well as venous aneurysms are typical associated findings.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital stenosis of the inferior vena cava | Congenital stenosis of the IVC · Congenital stenosis of the inferior caval vein |
| fr | Sténose congénitale de la veine cave inférieure | |
| de | Kongenitale Stenose der Vena cava inferior | Angeborene Stenose der unteren Hohlvene · IVC-Stenose, kongenitale |
| es | Estenosis congénita de la vena cava inferior | Estenosis congénita de la VCI |
| it | Stenosi congenita della vena cava inferiore | |
| nl | Congenitale stenose van vena cava inferior | Congenitale stenose van VCI · Congenitale stenose van onderste holle ader |
| pt | Estenose da VCI, congénita | |
| pl | Wrodzone zwężenie żyły głównej dolnej | Wrodzone zwężenie IVC |
| cs | Vrozená stenóza dolní duté žíly | Vrozená stenóza VCI |
| uk | Вроджений стеноз нижньої порожнистої вени | Вроджений стеноз нижньої порожнистої вени · Вроджений стеноз нижньої порожнистої вени |
| zh | 下腔静脉先天性狭窄 | 先天性IVC狭窄 |
| tr | İnferior vena kavanın Doğumsal stenozu | IVC'nin Doğumsal stenozu · Alt kaval venin Doğumsal stenozu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q26.0 | NTBT | inclusion | validated |
| ICD-11 | LA86.Y | NTBT | index | validated |
| MONDO | 0020451 | E | validated | |
| UMLS | C0265934 | 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:95499Congenital anomaly of the inferior vena cava
rare developmental anomalies during embryogenesis; rare cardiac malformations; rare circulatory system 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/99122
- This record as JSON: /data/d/99122.json