Azygos continuation of the inferior vena cava

Orpha code: 99121OMIM code:

Definition

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.

Disease data
Classification

Morphological anomaly

Synonyms
Azygos continuation of the IVC
Kontynuacja IVC przez żyłę nieparzystą
Nieparzysty przebieg żyły głównej dolnej
Azygos continuation of the inferior caval vein
Inferior vena cava interruption with azygos continuation
ORPHA code
99121
OMIM code
-
ICD10 code
Q26.8
ICD11 code
LA86.Y

No additional description.

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