American Journal of Neuroradiology 27:593-595, March 2006
© 2006 American Society of Neuroradiology
Case Report
SPINE
Low Back Pain as the Initial Symptom of Inferior Vena Cava Agenesis
H. Yigita,
B. Yagmurlub,
N. Yigita,
S. Fitozb and
P. Kosara
a Department of Radiology, Ankara Training and Research Hospital, Ankara University School of Medicine, Ankara, Turkey
b Department of Radiology, Ibn-i Sina Hospital, Ankara University School of Medicine, Ankara, Turkey
Address correspondence to Hasan Yigit, MD, Department of Radiology, Ankara Training and Research Hospital, Cebeci 06590, Ankara, Turkey
 |
Abstract
|
|---|
Summary: Inferior vena cava (IVC) agenesis is a rare anomaly
that is usually an incidental finding in radiologic work-up
or it can rarely be symptomatic due to deep venous thrombosis
of iliac veins. In this report, we present a case of IVC agenesis
detected on lumbar spinal MR imaging scans by extensive epidural-paravertebral
collateral vessels compressing the thecal sac and causing low
back pain in a child.
|
Introduction
|
|---|
Estimated prevalence of the anomalies of inferior vena cava
(IVC) in the general population is about 0.07%–8.7%.
1 It may be totally asymptomatic or may be complicated by venous
thrombosis, alerting the clinician to the diagnosis; however,
low back pain is not an expected outcome of this condition.
|
Case Report
|
|---|
A 13-year-old girl with a 5–6-month history of low back
pain worsening during lifting was referred to our institution,
and a lumbar spinal MR imaging examination was performed as
part of the diagnostic evaluation of her symptoms. MR imaging
scans showed tubular structures located at the anterior epidural
space along the lumbar segments of the spinal column, with low
signal intensity on both T1- (spin-echo sagittal; TR/TE, 400/14
milliseconds; section thickness, 4.0 mm; matrix, 224
x 256)
and T2-weighted (fast spin-echo sagittal and axial; TR/TE, 3000/96
milliseconds; section thickness, [sagittal] 4.0 mm/[axial] 5.0
mm; matrix, 192
x 256) series, which happened to compress the
thecal sac and were in communication with paravertebral tubulonudular
structures by means of neural foraminae on both sides having
similar MR signal intensity. Scalloping of the L3 vertebral
body at its posterior aspect secondary to compression was also
noted (
Figs 1 and
2). Signal intensity loss and location along
the site of basivertebral plexus and the left paravertebral
structures in continuity with another larger diameter, para-aortic
fusiform structure were suggestive of the vascular nature of
the lesions. Additional cranial MR imaging with gadolinium,
as well as an abdomen-oriented, contrast-enhanced dynamic MR
angiography (MRA), was performed to define the extent of the
disease. For the angiographic examination we used a 3D-fast
spoiled gradient-recalled sequence with an injection of 0.2
mmol/kg gadolinium followed by 3 sets of acquisitions within
separate breath holds (TR/TE, 6/1.2 milliseconds; section thickness,
3.0 mm; matrix, 128
x 256; flip angle, 20°; NEX, 1). MRA
and the upper-spinal MR imaging revealed the enlarged internal
and external venous plexus veins at the anterior epidural and
paravertebral space, respectively, with an extent to Th10 level
and communicating with the azygos-hemiazygos veins. The infrahepatic
segment of IVC could not be defined as a separate structure,
though suprahepatic segment was formed with normal caliber.
Common iliac veins were seen up to the conjunction level and
communicated with both vertebral and epigastric veins afterward,
because this seemed the only drainage pathway other than IVC.
Renal and ovarian veins also drained into the azygos-hemiazygos
system (
Fig 3). With these findings, the patient was diagnosed
with IVC agenesis associated with enlarged collateral pathways,
including vertebral-paravertebral plexus causing low back pain
due to thecal sac and neural foraminal compression. The patient
was discharged after palliative treatment of her symptoms, and
she was taken into follow-up regardless of whether the symptoms
worsened.
View larger version (97K):
[in this window]
[in a new window]
|
Fig 1. A, Axial T2-weighted spinal MR image at the level of L4–5 shows enlarged epidural veins characterized by signal void, causing a mass effect and displacing the thecal sac posteriorly. B, More cranial image depicts the scallopping of the posterior margin of L3 vertebra (open arrows) and the continuity of the epidural veins with paravertebral venous plexus via neural foraminae bilaterally (arrows).
| |
View larger version (158K):
[in this window]
[in a new window]
|
Fig 2. A, Midsagittal T2-weighted scan shows the prominent epidural venous plexus below level L2 (arrowheads). B, Sagittal T2-weighted image shows the enlarged veins obliterating the neural foraminae of level L2–3 (arrows).
| |
View larger version (145K):
[in this window]
[in a new window]
|
Fig 3. Venous phase of the 3D contrast-enhanced MR angiographic study, on a coronal MIP (maximum intensity projection) images.
A, Enlarged vertebral venous plexus (VVPx) consisting of epidural and paravertebral veins (arrows) extending up to the renal hilus level and consequently draining into the azygos-hemiazygous veins (open arrow).
B, Infrahepatic inferior vena cava cannot be distinguished as a separate structure. Common iliac veins appear at the conjunction level just behind the common iliac arteries (arrows) and drain into the VVPx eventually (not shown here). Enlarged left ovarian vein and a large-caliber tortuous right renal vein as a secondary drainage pathway of the lower body segments, as well as the VVPx, can also be recognized (asterisk). Arrowheads indicate the enlarged azygous vein.
| |
|
Discussion
|
|---|
Low back pain in a child is quite rare, and, according to the
incidence of different causes, age constitutes an important
clue in the differential diagnosis because it is known that
congenital, neoplastic, or infectious origin is more likely
in children younger than 10 years of age, whereas disk disease
may be responsible from the symptoms in older children and or
in adolescents, most commonly because of trauma.
2 The literature
indicates that congenital disorders causing backache are almost
always detected in patients younger than 10 years of age and
that bony malformations of the spine are the most frequent cause.
2 Vascular malformations in the diagnostic spectrum of low back
pain are rare. Vascular compression may either be arterial or
venous in origin, and venous mass effect is extremely rare in
view of the whole spine. Albayram et al have described a case
of cervical epidural venous engorgement secondary to intracranial
hypotension causing cervical radiculopathy, which demonstrated
the symptomatic effect of venous pressure on nervous structures.
3 Cervical myelopathy due to varicose epidural veins was also
described by Dickman et al as an example of spinal cord compression.
4
Embryologic anomalies of the IVC and its tributaries occur infrequently, resulting from maldevelopment of lower body venous system. Variations can be classified according to the abnormal regression or abnormal persistence of various embryonic veins, the most common ones being left IVC, double IVC, azygous continuation, circumaortic or retroaortic left renal vein, and absent infrarenal IVC.5 Embryogenesis of the IVC is a complex procedure mainly involving the formation, regression, and fusion of 3 paired embryonic veins: postcardinal, subcardinal, and supracardinal. The infrahepatic segment of IVC can be examined in 3 parts—the suprarenal, renal, and infrarenal segments—where the right subcardinal vein gives rise to the suprarenal segment, the right supracardinal-subcardinal and postcardinal-subcardinal anastomoses form the renal segment, and the infrarenal segment derives from the right supracardinal vein.5–7 Absence of the entire infrahepatic IVC as in the case presented here suggests that all 3 paired venous systems failed to develop properly. Patients with IVC agenesis may present with symptoms of lower extremity venous insufficiency or idiopathic deep venous thrombosis.5 If the deep venous collateral system is sufficiently developed and drains the venous blood from the lower extremities to the heart, venous stasis secondary to inadequate blood return through collaterals (resulting in subsequent deep venous thrombosis) is likely to be prevented.1 The patient presented here had a very large collateral pool draining the lower body venous blood, which reflects the compensated long duration of the process but caused a mass effect secondary to the very large amount of blood overloading the vertebral-paravertebral venous plexus. This mass effect was typically worsening during an activity increasing the intrathoracic pressure (such as lifting), resulting in the decrease of venous return and aggravating the mass effect of the vertebral venous collaterals.
The presence of an epidural or paraspinous mass continuous within multiple spinal segments, particularly showing signal intensity void on MR images, should raise the suspicion of a vascular anomaly. Cross-sectional imaging methods facilitate the recognition of these anomalies andavoid a misdiagnosis of a true solid lesion, but MR angiographic imaging is invaluable to define the final diagnosis. Overloaded and enlarged venous structures have the potential to compress the nervous system (either the spinal nerves and spinal cord or thecal sac), eventually causing neurologic symptoms, which might be the only and initial symptom; however, venous insufficiency resulting in deep venous thrombosis is a more frequently expected consequence of IVC agenesis.
|
References
|
|---|
- Obernosterer A, Aschauer M, Schnedl W, et al. Anomalies of the inferior vena cava in patients with iliac venous thrombosis. Ann Intern Med 2002;136:37–41[Abstract/Free Full Text]
- Martinez-Lage JF, Cornejo VF, Lopez F, et al. Lumbar disc disease in early childhood: case report and literature review. Childs Nerv Syst 2003;19:258–60[Medline]
- Albayram S, Wasserman BA, Yousem DM, et al. Intracranial hypotension as a cause of radiculopathy from cervical epidural venous engorgement: case report. AJNR Am J Neuroradiol 2002;23:618–21[Abstract/Free Full Text]
- Dickman CA, Zambarmski JM, Sonntag VK, et al. Myelopathy due to epidural varicose veins of the cervicothoracic junction: case report. J Neurosurg 1988;69:940–41[Medline]
- Bass JE, Redwine MD, Kramer LA, et al. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics 2000;20:639–52[Abstract/Free Full Text]
- Bass JE, Redwine MD, Kramer LA, et al. Absence of the inferior vena cava with preservation of the suprarenal segment as revealed by CT and MR venography. AJR Am J Roentgenol 1999;172:1610–12[Free Full Text]
- Minniti S, Visenti S, Procacci C. Congenital anomalies of the venae cavae: embryological origin, imaging features and report of three new variants. Eur Radiol 2002;12:2040–55[Medline]
Received April 15, 2005;
accepted after revision May 4, 2005.
This article has been cited by other articles:
|
|
|
|
 
Arash Mohammadi Tofigh, R. Coscas, F. Koskas, and E. Kieffer
Surgical Management of Deep Venous Insufficiency Caused by Congenital Absence of the Infrarenal Inferior Vena Cava
Vascular and Endovascular Surgery,
March 1, 2008;
42(1):
58 - 61.
[Abstract]
[PDF]
|
|
|
TOP
Abstract
Introduction