Congenital anomalies of IVC

By Dr M.Ajay. K & Dr Eranna Palled
Please visit www.jssmcradiology.com for more cases

There are Spectrum of Congenital Anomalies of the Inferior Vena
Cava and are usually seen in asymptomatic patients .
TYPES
I. Left IVC
II. Double IVC
III. Azygos continuation of the IVC
IV. Circumaortic left renal vein
V. Retroaortic left renal vein
VI. Circumcaval ureter
VII. Double IVC with Retroaortic Right Renal Vein and Hemiazygos
Continuation of the IVC
VIII. Double IVC with Retroaortic Left Renal Vein and Azygos
Continuation of the IVC
IX. Absent Infrarenal IVC with Preservation
of the Suprarenal Segment

I. Left IVC
 It is seen as a result from regression of the right
supracardinal vein with persistence of the left
supracardinal vein.
 Prevalence is 0.2%–0.5%.
 Here left IVC joins the left renal vein, which crosses
anterior to the aorta in the normal fashion, uniting with the
right renal vein to form a normal right-sided prerenal IVC

Schematic shows a left IVC terminating at the left renal vein.

Left IVC inferior to the renal veins. Left IVC joins the left renal vein

Left IVC crossing over aorta before
uniting with right renal vein
Left IVC shifted towards right
side and in close relation
with small pseudocyst

Left IVC shifted towards right side compressed by
large pseudocystPlease visit www.jssmcradiology.com for more cases

Hepatic veins draining into IVC

II Double IVC : the left IVC typically ends at the left renal vein,
which crosses anterior to the aorta to join the right IVC
III Azygos continuation of the IVC : the prerenal IVC passes
posterior to the diaphragmatic crura to enter the thorax as
the azygos vein
IV Circumaortic left renal vein : one left renal vein crosses
anterior to the aorta and another crosses posterior to the
aorta.
V Retroaortic left renal vein : As with circumaortic left renal
vein, a retroaortic left renal vein results from persistence of
the dorsal arch of the renal collar

VI Circumcaval ureter
VII Double IVC with Retroaortic Right Renal Vein and
Hemiazygos Continuation of the IVC.
VIII Double IVC with Retroaortic Left Renal Vein and
Azygos Continuation of the IVC
IX Absent Infrarenal IVC with Preservation
of the Suprarenal Segment

 The infrahepatic IVC develops between the 6th and 8th
weeks of embryonic life as a composite structure
formed from the continuous appearance and regression
of three paired embryonic veins.
 In order of appearance, they are the posterior cardinal,
the subcardinal, and the supracardinal veins .
 Initially, all blood return from the body wall caudal to
the heart proceeds through the posterior cardinal veins

 Blood return from the viscera is conveyed by the vitelline veins ,
which drain the yolk sac.
 Subsequently, the subcardinal veins develop ventromedial to the
posterior cardinal veins and ventrolateral to the aorta.
 The intersubcardinal anastomosis forms between the paired
subcardinal veins anterior to the aorta and caudal to the superior
mesenteric artery.
 Anastomoses between the posterior cardinal and subcardinal
veins develop on each side at approximately the level of the
intersubcardinal anastomosis.

 At the same time, union occurs between the right
subcardinal vein and the hepatic segment of the IVC, which
forms from the vitelline vein.
 As the cranial portions of the posterior cardinal veins begin
to atrophy,blood return from the lower extremities is
shunted through the postsubcardinal anastomosis, then
through the subcardinal-hepatic anastomosis to the hepatic
segment of the IVC.
This process establishes the prerenal division of the IVC.

 The next major development is the appearance of the paired
supracardinal veins,which lie dorsomedial to the posterior cardinal veins
and dorsolateral to the aorta.
 Initially, multiple anastomoses form between the posterior and
supracardinal veins.
 On each side, a suprasubcardinal anastomosis develops from union of the
postsupracardinal and the postsubcardinal anastomoses.
 In addition, intersupracardinal anastomoses develop dorsal to the aorta.
 The supracardinal veins then separate into cranial (azygos) and caudal
(lumbar) ends.

 Meanwhile, inferiorly, anastomoses develop between the two posterior
cardinal veins and between the posterior and lumbar supracardinal
veins.
 With further atrophy of the posterior cardinal veins, blood return from
the lower extremities is shunted through the supracardinal system to the
suprasubcardinal anastomosis, then to the prerenal division of the IVC.
 In addition, blood return from the left side of the body is shunted to the
right across the intersupracardinal and interpostcardinal anastomoses.
 Finally, the left supracardinal vein is one of the last veins to disappear,
although Huntington and McLure (4state that the vessel does not so
much atrophy as become incorporated into the right supracardinal vein
by coalescence of the multiple anastomoses.

 In summary, the normal IVC is composed of four segments:
hepatic, suprarenal, renal, and infrarenal.
 The hepatic segment is derived from the vitelline vein.
 The right subcardinal vein develops into the suprarenal
segment by formation of the subcardinal-hepatic anastomosis.

 The renal segment develops from the right suprasubcardinal and
postsubcardinal anastomoses.
 It is generally accepted that the infrarenal segment derives from
the right supracardinal vein, although this idea is somewhat
controversial .
 In the thoracic region, the supracardinal veins give rise to the
azygos and hemiazygos veins.
 In the abdomen, the postcardinal veins are progressively replaced
by the subcardinal and supracardinal veins but persist in the pelvis
as the common iliac veins.

 Since the embryonic ureter passes posterior to the posterior cardinal veins and
anterolateral to the supracardinal vein, formation of the postsupracardinal
anastomosis inferiorly and the suprasubcardinal anastomosis at the level of the
kidney allows development of a potential periureteric venous ring.
 The renal collar is formed from the intersupracardinal anastomosis dorsally, the
intersubcardinal anastomosis and postsubcardinal anastomoses ventrally, and the
suprasubcardinal anastomosis laterally. The embryonic kidneys are initially drained
by paired ventral and dorsal limbs.
 Ordinarily, both dorsal limbs regress.
 On the right side, the ventral limb is incorporated into the lateral wall of the
renal segment of the IVC.
 On the left side, the ventral limb and the anterior limb of the renal collar form
the normal adult left renal vein.

THANK YOU

Congenital anomalies of IVC

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