Inferior vena cava interruption without azygos continuation
Synonyms: IVC interruption · Inferior caval vein interruption
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
A rare congenital anomaly of the inferior vena cava characterized by complete interruption of the vessel in which no direct continuity exists between the inferior vena cava and the azygos/hemiazygos system. Clinical manifestations depend on the variant drainage patterns or collaterals and include lower extremity deep vein thrombosis, thromboembolic attacks, leg swelling and pain, lower extremity varices, abdominal pain, intraabdominal varices, and hematochezia, among others. Additional venous abnormalities or cardiac malformations are frequently present.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Inferior vena cava interruption without azygos continuation | IVC interruption · Inferior caval vein interruption |
| fr | Interruption de la veine cave inférieure sans continuation azygos | |
| de | Unterbrechung der Vena cava inferior ohne Azygoskontinuität | IVC-Unterbrechung · Unterbrechung der unteren Hohlvene · Vena cava inferior-Unterbrechung |
| es | Interrupción de la vena cava inferior sin continuación por la vena ácigos | Interrupción de la VCI · Interrupción de la vena cava inferior |
| it | Interruzione della vena cava inferiore senza continuità con il sistema azygos | |
| nl | Interruptie van vena cava inferior zonder continuatie van vena azygos | Interruptie van vena cava inferior · Onderbreking van onderste holle ader · Onderbreking van onderste holle ader zonder azygoscontinuatie · VCI-onderbreking |
| pt | Interrupção da VCI | |
| pl | Przerwanie żyły głównej tylnej | Przerwanie IVC |
| cs | Přerušení dolní duté žíly | Interrupce dolní duté žíly · Přerušení VCI |
| uk | Перерив нижньої порожнистої вени без продовження в непарну вену (v.azygos) | Переривання нижньої порожнистої вени · Переривання НПВ |
| zh | 下腔静脉中断不伴奇静脉延续 | IVC中断 |
| tr | Azigos devamı olmayan inferior vena kava kesintisi | IVC kesintisi · Alt kaval ven kesintisi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q26.8 | NTBT | attributed | validated |
| ICD-11 | LA86.Y | NTBT | attributed | validated |
| MONDO | 0020452 | E | validated | |
| MONDO | 20452 | E | validated | |
| UMLS | C5680291 | 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/99123
- This record as JSON: /data/d/99123.json