Inferior vena cava interruption without azygos continuation

Orpha code: 99123OMIM code:

Definition

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.

Disease data
Classification

Morphological anomaly

Synonyms
IVC interruption
Przerwanie IVC
Inferior caval vein interruption
ORPHA code
99123
OMIM code
-
ICD10 code
Q26.8
ICD11 code
-

No additional description.

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