In search of a cause for the so-called idiopathic Deep Vein Thrombosis (DVT), researchers have pointed towards association between recurrent DVT and absent IVC

1. Case 12672
Congenitally absent IVC: A rare cause of recurrent DVT and
non-healing leg ulcers
Muhammad Asim Rana , Abdulrahman M. Alharthy , Ahmed F. Mady , Basim Huwait , Waleed1 1 1 1
T. Aletreby , Omar E. Ramadan , Ahmed Hossam Awad1 1 2
1. King Saud Medical City, Riyadh Saudi Arabia
2. Rashid Hospital Dubai, UAE
Email:drasimrana@yahoo.com
King Saud Medical City, Riyadh Saudi Arabia
Abdominal ImagingSection:
2015, May. 14Published:
53 year(s), femalePatient:
Clinical History
The patient was referred with long-standing history of a swelling of both legs, repeated DVT and
PE. She was on life-long anticoagulation. Examination showed massive leg oedema up to the upper
third of the abdominal wall with distended veins and ulcers on both legs. CT abdomen with I.V.
contrast performed to rule out compressing nodes revealed another diagnosis.
Imaging Findings
CT abdomen and pelvis with I.V. contrast:
There was generalised oedema of the abdominal wall, worse inferiorly, which is in keeping with the
history of lymphoedema. Extensive varices at the abdominal wall were noted. There were also
intraabdominal varices at the paraaortic region. A grossly enlarged azygous vein was fed by
collateral vessels from the abdominal wall. The vena cava was absent and replaced by multiple
collateral veins around the paraaortic region. The liver, spleen, adrenals, kidneys and pancreas were
normal. No para-aortic and no pelvic masses were found. No abnormality was seen in the
abdominopelvis.

2. Discussion
Congenitally absent Inferior Vena Cava (IVC) has been associated with other cardiovascular
abnormalities [1] and includes a spectrum ranging from presence of bilateral IVC to its complete
absence [2].
In search of a cause for the so-called idiopathic Deep Vein Thrombosis (DVT), researchers have
pointed towards association between recurrent DVT and absent IVC; furthermore the absence of
IVC is associated with right renal aplasia [3]. Taking it further some investigators have linked IVC
absence and renal hypoplasia with perinatal renal vein thrombosis [4]. The condition of renal
aplasia, absent IVC and DVT has been named KILT (Kidney and IVC abnormalities with Leg
Thromboses) syndrome [5] by some investigators. Ruggeri M et al and Chee YL et al have given
special attention to the association between the so-called idiopathic DVT and IVC anomalies and
both teams have concluded that IVC anomalies with DVT are commoner than reported in the
literature [6, 7].
Absence of IVC cannot be diagnosed on ultrasound. Some clues may point towards the diagnosis
like dilated azygous and hemi-azygous veins and prominent collateral veins in abdominal wall [8].
The most reliable investigations for the diagnosis of anomalous or absent IVC are CT with I.V.
contrast or MRI. CT provides a good interpretation of retroperitoneal organs and in cases of
stenosed or absent IVC, retroperitoneal venous plexus is also well developed and helps with the
diagnosis [9]. Venography can also be used, but is helpful mainly in cases where surgical
intervention is planned.
When the association between absence or stenosed IVC and recurrent DVT is questioned, most of
the investigators have the opinion that despite development of adequate collateral channels the
venous stasis and resulting chronic venous hypertension can precipitate thrombosis.
Gayer et al advocate that all patients with IVC abnormality should undergo thrombophilia screening
as well because in their series 7 of 9 patients with IVC anomaly and DVT had a positive
thrombophilia screen [10].
Very little evidence is available on the treatment options for the symptomatic patients. One case
report documents treatment with surgical intervention with good results [4]. Life-long
anticoagulation, however, is suggested even if thrombophilia screen is negative.
Literature review suggests that any young patient with either non-healing leg ulcers or recurrent
DVT should be investigated for IVC anomalies with CT abdomen with I.V. contrast if there are no
other clues to the diagnosis. Surgical intervention has a limited role and patients should be offered
life-long anticoagulation for ongoing risks of DVT and pulmonary embolism.
Final Diagnosis
Congenitally absent IVC: A rare cause of recurrent DVT and nonhealing leg ulcers
Differential Diagnosis List
Bilateral lymphoedema in the legs, Varicose veins
Figures

3. Figure 1 CT abdomen and pelvis
Axial section through lower chest and mediastinum. Note the prominent azygous and
hemiazygous veins. Dilated superficial veins in anterior abdominal wall also noted with
gross oedema of the wall.
© Muhammad Asim Rana
Area of Interest: Abdomen;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;
Axial CT abdomen. Note the dilated veins in skin and wall oedema. Intraabdominal veins are
prominent.
© Muhammad Asim Rana
Area of Interest: Abdomen;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;

4. Thick oedematous abdominal wall with dilated tortuous veins. Note the dilated
intraabdominal veins.
© Muhammad Asim Rana
Area of Interest: Abdomen;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;
Figure 2 CT abdomen and pelvis
Para-aortic dilated and prominent veins.
© Muhammad Asim Rana
Area of Interest: Abdomen;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;

5. Axial cuts through the pelvis. Note the gross oedema of the back and anterior abdominal
wall with tortuous dilated veins. Clusters of dilated veins are visible in pelvis in the cavity
and near the wall.
© Muhammad Asim Rana
Area of Interest: Abdomen;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;
Grossly oedematous abdominal wall with dilated tortuous veins. Note the oedema on the
back.
© Muhammad Asim Rana
Area of Interest: Abdomen;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;
Figure 3 CT abdomen and pelvis

6. Coronal section CT abdomen. Dilated and prominent mesenteric veins are seen. Grossly
oedematous abdominal wall with prominent veins.
© Muhammad Asim Rana
Area of Interest: Abdomen;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;
Coronal section CT abdomen. Prominent and dilated renal veins.
© Muhammad Asim Rana
Area of Interest: Abdomen;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;

7. Coronal section CT abdomen. Note the para-aortic venous dilatation. Note the absence of
inferior vena cava.
© Muhammad Asim Rana
Area of Interest: Abdomen;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;
References
[1] Anderson RC, Adams P, Burke B (1961) Anomalous inferior vena cava with azygous
continuation (infra-hepatic interruption of the inferior vena cava). The Journal of Pediatrics
59(3):370-83
[2] Bass JE, Redvine MD, Kramer LA, Huynh PT, Harris JH (2000) Spectrum of Congenital
anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics 20(3):639-52
[3] Gayer G, Zissin R, Strauss S, Hertz M (2003) IVC anomalies and right renal aplasia detected on
CT: a possible link? AbdominalImaging 28(3):395-399
[4] Dougherty MJ, Calligaro KD, DeLaurentis DA (1996) Congenitally absent inferior vena cava
presenting in adulthood with venous stasis and ulceration: A surgically treated case. J Vasc Surg
23:141-6
[5] Veen JV, Hampton KK, Makris M (2002) KILT syndrome? British Journal of Haematology
118:1199-1200
[6] Ruggeri M, Tosetto A, Castaman G, Rodighiero F (2001) Congenital absence of the inferior
vena cava: a rare risk factor for idiopathic deep-vein thrombosis. Lancet 357(9254):441
[7] Chee YL, Dominic J, Watson CG, Watson HG (2001) Inferior Vena Cava malformation as a
risk factor for deep venous thrombosis in the young. British Journal of Haematology 114:878-880

8. [8] Koc Z, Oguzkurt (2007) Interruption or congenital stenosis of the inferior vena cava:
Prevalence, imaging, and clinical findings. European Journal of Radiology 62(2)257-266
[9] Ueda J, Hara K, Kobayashi Y, Ohue S, Udrida H (1983) Anomaly of the Inferior Vena Cava
observed by CT. Computerised Radiology 7(3):145-154
[10] Gayer G, Luboshitz J, Hertz M, Zissin R, Thaler M, Lubetsky A, Bass A, Korat A, Apter S
(2003) Congenital anomalies of the inferior vena cava revealed on CT in patients with deep vein
thrombosis. Am J Roentgenology 180(3):729-32
Citation
Muhammad Asim Rana , Abdulrahman M. Alharthy , Ahmed F. Mady , Basim Huwait , Waleed1 1 1 1
T. Aletreby , Omar E. Ramadan , Ahmed Hossam Awad1 1 2
1. King Saud Medical City, Riyadh Saudi Arabia
2. Rashid Hospital Dubai, UAE
Email:drasimrana@yahoo.com (2015, May. 14)
Congenitally absent IVC: A rare cause of recurrent DVT and non-healing leg ulcers {Online}
URL: http://www.eurorad.org/case.php?id=12672