Azygos continuation of the inferior vena cava
Synonyms: Azygos continuation of the IVC · Azygos continuation of the inferior caval vein · Inferior vena cava interruption with azygos continuation
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
A rare vascular anomaly characterized by absence of the hepatic segment of the inferior vena cava and presence of an enlarged azygos vein (or in rare cases hemiazygos vein, if there is a left-sided inferior vena cava) draining the venous blood from the caudal segments. The post-hepatic segment of the inferior vena cava is present, draining only the hepatic veins into the right atrium. Most patients remain asymptomatic, if the anomaly is isolated. Association with congenital heart disease and asplenia or polysplenia syndromes has been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Azygos continuation of the inferior vena cava | Azygos continuation of the IVC · Azygos continuation of the inferior caval vein · Inferior vena cava interruption with azygos continuation |
| fr | Continuation azygos de la veine cave inférieure | Interruption de la veine cave inférieure avec continuation azygos |
| de | Azygoskontinuität der Vena cava inferior | Azygos-Kontinuitätssyndrom · Azygoskontinuität der Vena cava inferior · Azygoskontinuität der unteren Hohlvene · Unterbrechung der Vena cava inferior mit Azygoskontinuität |
| es | Continuación ácigos de la vena cava inferior | Interrupción de la VCI con continuación por la vena ácigos |
| it | Continuazione della vena azygos nella vena cava inferiore | |
| nl | Azygoscontinuatie van vena cava inferior | Azygoscontinuatie van VCI · Azygoscontinuatie van onderste holle ader · Interruptie van vena cava inferior met continuatie van vena azygos · Onderbreking van onderste holle ader met azygoscontinuatie |
| pt | VCI, prolongamento da veia azigos | |
| pl | Kontynuacja żyły głównej dolnej przez żyłę nieparzystą | Kontynuacja IVC przez żyłę nieparzystą · Nieparzysty przebieg żyły głównej dolnej |
| cs | Dolní dutá žíla pokračující venou azygos | Azygos pokračování VCI |
| uk | Продовження нижньої порожнистої вени в непарну вену (в складі синдрому гетеротаксії) | Азиготне продовження нижньої порожнистої вени · Азиготське продовження IVC · Переривання нижньої порожнистої вени з азиготним продовженням |
| zh | 下腔静脉与奇静脉延续 | 下腔静脉阻断伴奇静脉延续 |
| tr | İnferior vena kavanın azigos devamı | IVC'nin azigos devamlığı · Alt kaval venin azigos devamlığı · Azigos devamı ile inferior vena kava kesintisi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q26.8 | NTBT | inclusion | validated |
| ICD-11 | LA86.Y | NTBT | index | validated |
| MONDO | 0020450 | E | validated | |
| UMLS | C1859772 | 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/99121
- This record as JSON: /data/d/99121.json