Which anomalies the of inferior vena caval are associated with inferior vena caval thrombosis (IVCT)?

Updated: Jun 12, 2018
  • Author: Luis G Fernandez, MD, FACS, FASAS, FCCP, FCCM, FICS, KHS, KCOEG; Chief Editor: John Geibel, MD, DSc, MSc, AGAF  more...
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Answer

Anomalies of the IVC have been described more frequently (0.6-2%) in those with other cardiovascular defects [19] and less so in otherwise healthy individuals. Various abnormalities of the IVC have been described, including complete absence, partial absence, or presence of bilateral IVC. [20]

Absent IVC is an extremely rare anomaly that is associated with idiopathic DVT, particularly in the young. Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is the result of perinatal IVC thrombosis causing regression and disappearance of the once present IVC. [21]

A case report has described an absent IVC and left renal hypoplasia and a right hypertrophic kidney. [22] A more commonly recognized association is right renal aplasia, as suggested in a review by Gayer et al, in which all nine patients with complete absence of the IVC had an absent or very small right kidney. [23]

The association of an absent or hypoplastic kidney is related (or may contribute to an absent IVC) to perinatal renal vein thrombosis. [24] Veen et al proposed naming this condition KILT (kidney and IVC abnormalities with leg thromboses) syndrome (when associated with DVT). [22]

It is estimated that DVT occurs at a rate of 1 case per 1000 patient-years [25] ; in as many as 80% of patients who are affected, a risk factor can be identified. Ruggeri et al presented four cases of absent IVC over a 5-year period that presented with idiopathic DVT in patients younger than 30 years, [26] representing an estimated 5% of cases of idiopathic DVT in young people.

Chee et al similarly noted that as many as 5% of 20- to 40-year-old patients presenting with DVT had an IVC anomaly (four in total, of which three had a complete absence of IVC). [27] This was much higher than the expected 0.5%.


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