Agenesis of Inferior Vena Cava with Cavernous

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D
Journal of US-China Medical Science 13 (2016) 19-23
doi: 10.17265/1548-6648/2016.01.003
DAVID
PUBLISHING
Agenesis of Inferior Vena Cava with Cavernous
Transformation and Hypoplastic Infrarenal Part of IVC,
Rare Congenital Vascular Anomalies: A Case Series
Rub Nawaz Baloch1, Shazia Kadri2, Naveed Ahmed2, Sami U Din2, Arifuz Zaman2 and Tariq Mahmood2
1. Department of Cyber Knife Radiosurgery, Jinnah Postgraduate Medical Centre, Karachi, Sindh 72500, Pakistan
2. Department of Radiology, Jinnah Postgraduate Medical Centre, Karachi, Sindh 72500, Pakistan
Abstract: Congenital vascular anomalies are although rare but may cause serious complications. Vascular pathologies are best
demonstrated by contrast enhanced computed tomography, with the advent of multidetector computed tomography and applying
multiplanar reformation techniques. However, ultrasound color Doppler and catheter angiography are accessory imaging modalities
utilized to rule out vascular anomalies. We present here a case series of 2 patients aged 19 years and 54 years, diagnosed with congenital
vascular anomalies (agenesis of inferior vena cava and hypo plastic infra renal segment of inferior vena cava). The development of
inferior vena cava (IVC) includes early embryogenesis stages including formation, confluence and involution of three primary
longitudinal venous systems. Agenesis of inferior vena cava or hypoplasia of segment of inferior vena cava leads to formation of
collaterals to maintain the venous flow. Contrast enhanced computed tomography with multiplanar reformation provides ulterior
spatial and contrast resolution in cases of recognized vascular abnormalities. Inferior vena cava obstruction due to thrombosis or
congenital vascular anomaly leads to extensive collateralization accompanied by varicose veins. In our case series, we found contrast
enhanced computed tomography as an ulterior modality in setting ultimate diagnosis with accuracy and maximum detail.
Key words: IVC, hypoplastic, azygous vein, cavernous transformation, varicose.
1. Introduction
Anomalies of inferior vena cava are present in
0.3%-0.5% of otherwise healthy individuals and in
0.6%-2% of patients with other cardiovascular defects
[1, 2]. The prevalence of deep venous thrombosis
(DVT) varies according to age being 10 times lower in
20-40 years old individuals than in older age groups [3,
4]. In young patients, its etiology is frequently
associated with risk factors such as congenital and
acquired thrombophilia, autoimmune diseases,
pregnancy and peurperium, use of oral contraceptives,
neoplasms,
surgical
procedures,
prolonged
immobilization, and trauma [5-7]. In adults, absent
inferior vena cava (AIVC) can cause diagnostic
problems in the paravertebral regions because of their
tumor like appearance [8, 9].
Corresponding Author: Rub Nawaz Baloch, B.S (MIT,
RT), reasearch fields: dosimetry, radiosurgery and radiology.
2. Materials and Methodology
This case series was held at Jinnah Postgraduate
Medical center Karachi, Pakistan. Both the patients
were referred from out patients department (OPD) for
computed tomography (CT). Axial post contrast
(Iopamiro 370) imaging was performed for abdomen
pelvis, with 2 mm slice thickness, with an interspace of
2 mm, 120 KVp, 300 mA, axial images were
reconstructed into coronal and sagittal planes.
3. Case Report 1
A 19 year old male, who came to outpatient
department (OPD) with complain of lower limb
varicose veins, and generalized abdominal pain. Patient
was sent to radiology department for abdominal
imaging. Abdominal ultrasound, a venous duplex scan
of both legs was performed. On duplex both legs were
20
Agenesis of Inferior Vena Cava with Cavernous Transformation and Hypoplastic
Infrarenal Part of IVC, Rare Congenital Vascular Anomalies: A Case Series
edematous with varicosities without any evidence of
deep venous thrombosis and deep or superficial venous
incompetence. On abdominal sonogram, we revealed
dilated anterior abdominal wall veins while
nohepatomegalia/splenomegaly,
nor
portal
hypertension was observed. A post contrast CT
abdomen was suggested following abdominal
sonography, CT scan revealed absent inferior vena
cava (AIVC) (Fig. 1) with cavernous transformation,
enlarged azygous and hemiazygous veins with a cluster
of collaterals in the pelvic region as well as anterior
abdominal wall (Fig. 2). CT scan revealed no venous
thrombosis.
Patient was suggested to avoid prolonged
immobilization maintain lower limb elevation while
taking on bed, as those patients with agenesis of IVC
were found to be at a greater risk of developing deep
venous thrombosis.
(A)
4. Case Report 2
A 54 year old female came to outpatient department
(OPD) complaining of lower limb pain was sent to
radiology department for CT scan abdomen post
contrast study. Post contrast axial images were
obtained and were reformatted into coronal and sagittal
(B)
(C)
Fig. 2 Post contrast axial Computed Tomography images
showing (A) dilated azygos and hemiazygos, (B) extensive
patent anterior abdominal wall collaterals and dilated
paravertebral veins and (C) collateral vessels in the pelvic
region.
Fig. 1 Post contrast computed tomography MPR coronal
image showing complete absence of inferior vena cava.
planes. Findings were, absent/hypoplastic infra renal
part of IVC not showing any contrast enhancement
associated with dilated gonadal veins (Fig. 3) on both
Agenesis of Inferior Vena Cava with Cavernous Transformation and Hypoplastic
Infrarenal Part of IVC, Rare Congenital Vascular Anomalies: A Case Series
sides, predominantly on left side throughout its course.
Lumbar and azygous veins were also prominent
consistent with collateralization (Fig. 3).
5. Discussion
The IVC is developed as a result of a complex
embryologic process between the sixth and eighth
weeks of gestation. Three pairs of primitive veins
(postcardinal, subcardinal, and supracardinal) appear in
this order and give rise to the four segments of the adult
IVC: hepatic, suprarenal, renal, and infrarenal (Fig. 4)
21
[3, 4, 9, 10]. AIVC is often used to describe three
different entities:
(1) Absence of the suprarenal IVC results from
failure to form the right subcardinal vein. The hepatic
segment drains directly into the right atrium, and the
blood from the infrarenal IVC returns to the heart
through the azygos and hemiazygos veins [8, 10].
There is association with other cardiac and visceral
anomalies, such as dextrocardia, atrial septal defect,
atrioventricular canal, situsinversus, polysplenia,or
asplenia [9, 11, 12].
(A)
(B)
Fig. 3 Contrast Enhanced CT shows absent infra renal part of IVC with prominent azygous and hemi azygous vein and
dilated bilateral gonadal veins.
Fig. 4 Embryologic deviation of the IVC from 7 weeks of gestation (A) to the adult (B).
22
Agenesis of Inferior Vena Cava with Cavernous Transformation and Hypoplastic
Infrarenal Part of IVC, Rare Congenital Vascular Anomalies: A Case Series
(2) Absence of the infrarenal IVC with preservation
of the suprarenal segment implies a failure of the
development of the right supracardinal vein [10].
(3) Absence of the entire IVC, as in our patient’s
case, suggests that all three paired vein systems failed
to develop properly [10], but it has no relation to the
other congenital anomalies described previously [11].
The reasons for the developmental failure are
unclear. One hypothesis is embryonic dysontogenesis
[1, 11, 13], but some authors suggest that it is the result
of an intrauterine or perinatal thrombosis [9]. AIVC
could be present with DVT. An inadequate blood
return through collaterals may increase the venous
blood pressure in the veins of the legs, thus facilitating
deep venous thrombosis (DVT) [9, 13]. The dysgenesis
of the IVC has been described in coincidence with
that it is the result of an intrauterineor perinatal
thrombosis [10]. But in our case, patient did not
presented with DVT despite of dysgenesis of IVC.
6. Conclusion
In our case series, we found that congenital IVC
abnormalities may not present clinically for a long time
due to collateral function. Peripheral venous
thrombosis or varicose veins are often first symptoms
of congenital anomaly of IVC. So in case of DVT or
varicose veins, a potential cause of IVC abnormality
must always be excluded with the help of contrast
enhanced computed tomography.
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Normally, the IVC is formed by the junction of the
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