Agenesis vena cava inferior associated with agenesis of the vena iliaca communis bilateral: a case report

Agenesis vena cava inferior associated with agenesis of the vena iliaca communis bilateral: a case report

EUROPEAN JOURNAL OF RADIOLOGY European Journal of Radiology 18 (1994) 101-103 Agenesis vena cava inferior associated with agenesis of the vena iliac...

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European Journal of Radiology 18 (1994) 101-103

Agenesis vena cava inferior associated with agenesis of the vena iliaca communis bilateral: a case report V. Pajkrt-Fiirstenzeller Department of Radiology, Diaconessenhuis Voorburg. Fonteynenburghlaan 5, 2275 CX Voorburg. Netherlands

(Received 25 November 1993; accepted 21 January 1994)

Key words: Magnetic


(MR); Veins; Veins, anomalies

1. Introductioa

Congenital anomaly of the inferior vena cava (IVC) is uncommon, the association with agenesis of the iliac veins is extremely rare. This report presents the case of a sportsman with varicose veins on both legs extending to the waist, without any other abnormalities. An enlarged azygos vein in the right tracheobronchial angle on chest radiograph was the first sign of increasing venous abdominal obstruction. 2. case report A 17-year-old man was admitted to the hospital with complaints of fever, fatigue, and vague intermittent muscular pain in both legs following sport. The patient’s medical history was unremarkable and his general condition was good; he had always been very active in sport. Physical examination revealed no abnormalities, all blood tests were normal. On the chest radiograph (Fig. la) a round shadow was seen in the right tracheobronchial angle simulating adenopathy. Due to the fact that the patient was asymptomatic after a few days, no clinical follow-up was done. After a few months the patient returned, complaining of large varicose veins on the outer aspect of his legs extending to the waist. A repeat chest radiograph again revealed the same shadow in the right tracheobronchial angle. On the abdominal CT scan no enlarged lymph nodes, thrombus, or tumor mass was found. The IVC from the level of the renal hilus downwards was absent. The bilateral transfemoral phlebography showed only the proximal two segments of the IVC. The other two segments and also the vena

iliaca communis bilateral were absent (Fig. 1b). Vena iliaca extema drained directly into the co-existed dilated vena lumbalis ascendens and considerably enlarged epidural veins (Fig. 2a,b), with continuation of the vena azygos and vena hemiazygos systems. The contrast medium passed through the collateral pathways into the vena iliaca intema, with retrograde filling of the vena pudendalis intema and collaterals vessels. The variation in vessel course and calibre made correct identification of these structures difficult. For further valuable information helpful in the safe planning of treatment, MRI investigation was performed (Figs. 2b, 3). 3. Diaelapsion Anomalies of the IVC, including agenesis, are often discovered incidentally because these anomalies are usually asymptomatic [1,2]. The only time they were demonstrated was during venography or cardiac catheterization with a femoral approach, procedures often associated with significant cardiac anomalies 13-51. Absence of the IVC is very rare, most of the cases described missing segment, usually the intrahepatic one [2,5,6]. There have been very few reports on similar anomalies of the venae iliacae [7,8]. The anomaly of the iliac veins is of considerable clinical significance because of the collaterals which develop to bypass the obstruction [8]. In this case, the manifestation of the external collateral insufficiency which resulted in varices on legs and waist, can be attributed to the patient’s extensive sport activity. Therapy is focused on the prevention of complications such as thrombosis. Extirpation of such veins is

0720-048X/94/307.00 0 1994 Elsevier Science Ireland Ltd. All rights reserved. SSDI 0720-048X(94)00519-1


V. Pajkrt-FiirstenzeNer

I Eur. J. Iiadiol

18 (1994) 101-103

b Fig. I. (a) Frontal radiograph. Dilated vena azygos in the right trachea-bronchial angle. (b) Contrast-enhanced venogram shows absence of the vena iliaca communis bilateral and IVC.



Fig. 2. (a) Dilated venae lumbalis ascendens and epidural veins. Large varix at the level of the vena renal is left. (b) Correlated sagittal Tlweighted MR image.

Fig. 3. Axial Tl-weighted MR image below the kidney. The WC is missing. Collateral paravertebral pathways.

V. Pajkrt-Fiirstenzeller / Eur. J. Radiol. 18 (1994) 101-103

contraindicated. The phlebography still remains a mandatory investigation for accurate visualization of the site and extent of venous dysplasia. 4. Acknowledgement

103 3

4 5

MRI was performed in the University Hospital Leiden with permission of Professor Dr J.L. Bloem.


5. References 1 Stetter G, Pallapies V, Lange S. Erweiterung der Vena azygos bei Agenesie der Vena cava inferior. Riintgenpraxis 1989; 42: 389-391. 2 Mayo J, Gray R, St Louis E et al. Anomalies of the inferior vena cava. AJR 1983; 140: 339-345.



Sonin AH, Mazer MJ, Powers TA. Obstruction of the inferior vena cava: A multiple-modality demonstration of causes, manifestations, and collateral pathways. RadioGraphics 1992; 12: 309-322. Dietz R, Reinheimer G. Beitrag zur CT-Diagnostik der Venacava-inferior-Anomalien. Radiologe 1991; 31: 352-354. Munechika H, Cohan RH, Baker ME et al. Hemiaxygos continuation of a left inferior vena cava: CT appearance. J Comput Assist Tomogr 1988; 12: 328-330. Gomes MN, Choyke PL. Assessment of major venous anomalies by computerized tomography. J Cardiovasc Surg 1990; 31: 621-628. Hawass ND, Kolawole TM, Badawi MG. Concomitant pulmonary arteriovenous and inferior vena cava malformations. Eur J Radio1 1988; 8: 102-105. Thomas ML, Posniak HV. Agenesis of the iliac veins. J Cardiovasc Surg 1984; 25: 64-66.