Congenital stenosis of the inferior vena…

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Congenital stenosis of the inferior vena cava

ORPHA:99122Morphological anomaly

Also called Congenital stenosis of the IVC · Congenital stenosis of the inferior caval vein

What it is

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.

Key facts

Classified as
Morphological anomaly

ICD-10 codes

Q26.0filed under a broader ICD-10 category

Cross-references

MONDO 0020451UMLS C0265934

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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