Congenitally absent Inferior Vena Cava: A rare cause of recurrent DVT and non-healing leg ulcers

Case 12672 Congenitally absent IVC: A rare cause of recurrent DVT andnon-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:[email protected]

King Saud Medical City, Riyadh Saudi Arabia

Abdominal Imaging Section: 2015, May. 14 Published:

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.

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

Figure 1 CT abdomen and pelvis

Axial section through lower chest and mediastinum. Note the prominent azygous andhemiazygous veins. Dilated superficial veins in anterior abdominal wall also noted withgross 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 areprominent.




Thick oedematous abdominal wall with dilated tortuous veins. Note the dilatedintraabdominal veins.





Para-aortic dilated and prominent veins. © Muhammad Asim Rana



Axial cuts through the pelvis. Note the gross oedema of the back and anterior abdominalwall with tortuous dilated veins. Clusters of dilated veins are visible in pelvis in the cavityand near the wall.




Grossly oedematous abdominal wall with dilated tortuous veins. Note the oedema on theback.





Coronal section CT abdomen. Dilated and prominent mesenteric veins are seen. Grosslyoedematous abdominal wall with prominent veins.




Coronal section CT abdomen. Prominent and dilated renal veins. © Muhammad Asim Rana



Coronal section CT abdomen. Note the para-aortic venous dilatation. Note the absence ofinferior vena cava.




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] 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:[email protected] (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

Congenitally absent Inferior Vena Cava: A rare cause of recurrent DVT and non-healing leg ulcers

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Transcript of Congenitally absent Inferior Vena Cava: A rare cause of recurrent DVT and non-healing leg ulcers