Open Journal of Urology, 2013, 3, 206-209
http://dx.doi.org/10.4236/oju.2013.35038 Published Online September 2013 (http://www.scirp.org/journal/oju)
Case Presentation of Preureteral Vena Cava and Review
of the Literature
Nexhmi Hyseni*, Sadik Llullaku, Murat Berisha, Ardian Shefkiu, Salih Grajqevci, Hysni Jashari,
Defrim Koqinaj, Fjolla Hyseni, Islam Bytyci, Fehim Muqolli
Department of Pediatric Surgery, University Clinical Center, Prishtina, Kosovo
Received July 24, 2013; revised August 21, 2013; accepted August 26, 2013
Copyright © 2013 Nexhmi Hyseni et al. This is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Retrocaval ureter, terms are anatomically descriptive but misleading in regard to development and results from altered
vascular development. This anomaly is relatively uncommon, although it has clinical relevance. The ureter typically
deviates medially behind the inferior vena cava, winding about and crossing in front of it from a medial to a lateral di-
rection, to resume a normal course, distally, to the bladder. The renal pelvis and upper ureter typically appear elon-
gated and dilated in a “J” or fishhook shape before passing behind the vena cava. Diagnoses were confirmed with in-
travenous urography and patient had an open surgical repair of the anomaly. The anomaly predominantly involves the
right ureter, as was observed in these reported cases. Treatment is surgical allowing for correction of the anomaly with
resolution of symptoms
Keywords: Preuretral Vena Cava; Pediatric Urology
This anomaly is commonly known as circumcaval or
retrocaval ureter. This variety of vascular lesion can cau-
se ureteral obstruction. The term preurteral vena cava
emphasizes that the circumcaval ureter results from al-
tered vascular, r a ther than ur etral, de ve lopm e nt .
It was initially considered as aberration in ureteric de-
velopment; however several studies in embryology have
led to it being considered as an aberration in the devel-
opment of the inferior vena cava [2-4].
This disorder invo lves the right ureter, which typically
deviates medially behind (dorsal to) the inferior vena
cava, winding about and crossing in front of it from a
medial to lateral direction, to resume a normal course,
distally, to the b ladder. The renal pelvis and upper ureter
are typically elongated and dilated in a J or fishhook
shape before passing behind the vena cava .
2. Case Report
A 5 year-old boy since four months, he admitted having
had occasional sharp transient dull and intermittent pain
in the right flank.
Physical examination was normal. Complete labora-
tory evaluation including urinalysis, complete blood pic-
ture, urea, creatinine and electrolytes were within normal
limits. KUB ultrasound showed a moderate hydronephro-
sis. Left kidney, left ureter and urinary bladder were
normal. An intravenous pyelogram showed prompt bilat-
eral excretion from both kidneys and a normal left upper
urinary tract. On the right side a moderate hydronephro-
sis associated with caliectasis was observed and it was
noted that the upper ureter was S-shaped and was kinked
medially towards the midline at the level of the trans-
verse process of the third lumbar vertebra. The ureter
could not be visualized beyond that point Figure 1. Ret-
rograde ureteropyelography demonstrates and S curve to
the point of obstruction, with the retrocaval segment ly-
ing at the level of L3 or L4 suggesting the presence of a
retrocaval ureter (Figure 2). The right ureter was ex-
plored through a right-flank incision. On exploration,
proximal ureter was curved medially then posterior to
IVC. Finally curved anteromedially to IVC and took a
downward course (Figure 3). Surgical correction in-
volves ureteral division, with relocation and ureterouret-
eral reanastomosis Figure 4. A simple ureteral stent was
inserted in an antegrade manner during operation. An
intravenous pyelography, and renal ultrasonography were
performed 3 months postoperatively, showed regression
*Corresponding a uthor.
opyright © 2013 SciRes. OJU
N. HYSENI ET AL. 207
Figure 1. Intravenous pyelogram showed that the upper
ureter was S-shaped and was kinked medially towards the
midline at the level of the transverse process of the third
Figure 2. Retrograde ureteropyelography demonstrates an
S curve to the point of obstruction, with the retrocaval seg-
ment lying at the level of L3 or L4 suggesting the presence
of a retrocaval ureter.
of hydronephrosis and hydroureter with no ureteric ob-
The first observed case of retrocaval ureters was de-
scribed by Hochstetter in 1893 . Though initially
thought of as an anomaly of ureteric development studies
in embryology has revealed an anomaly related to the
development of the inferior vena cava [7-9].
This anomaly is commonly known as circum-caval or
retrocaval ureter . The terms of cicumcaval ureter is
preferred, because rarely a ureter may lie behind (dorsal
to) the vena cava for some portion of its lumbar course,
forming a “siphon” capable of causing urinary obstruc-
tion. The anomaly predominantly involves the right
ureter, as was observed in these our reported cases. If it
involves the left ureter then it is usually associated with
Figure 3. On exploration, proximal ureter was curved me-
dially then posterior to IVC and finally curved anterome-
dially to IVC and took a downwa r d course.
Figure 4. Following the confirmation of obstruction, sur-
gery was indicated in the form of pyelic sectioning and ure-
teral trans positioning of the retrocaval segment.
Copyright © 2013 SciRes. OJU
N. HYSENI ET AL.
either partial or complete situs inversus or duplication of
the inferior vena cava (IVC) [10,11]. Duplication of the
IVC (D-IVC): This is a relatively uncommon congenital
anomaly with a reported incidence of 0.2% - 3%. A ma-
jority of the cases are clinically silent and they are diag-
nosed incidentally during imaging studies which are done
for other reasons . Retrocaval ureter results from
altered vascular, rather than ureteral, development. Bate-
son and Atkinson distinguished the two types of retro-
caval ureters according to the radiological appearance
and the site of the ureteral narrowing. These are:
Type I: The ureter crosses behind the IVC, at the level
of the L3 vertebra and it exhibits an “S-shaped” deform-
Type II: The renal pelvis and the upper ureter lie hori-
zontally. The retrocaval segment of the ureter is at the
same level as that of the renal pelvis and it exhibits a
“sickle shaped” deformity .
The retrocaval ureter which was observed in our case
classified into the Type I of the given classification. The
incidence of preuretral vena cava at autopsy is about one
in 1500 cadavers, although the lesion is congenital, most
patients do not present until the third or fourth decade of
life . Clinically, may present with symptoms of flank
or abdominal pain or infection or the disorder may be
discovered incidentally during other radiologic tests. This
disorder can cause varying degrees of ureteral obstruc-
tion. In order to reduce irradiation, the scintigraphy scan
is likely to replace IV urography, CT urography and diu-
retic renography. Excretory urography often fails to vis-
ualize the portion of the ureter beyond the J hook, but
retrograde ureteropyelography demonstrates an S curve
to the point of obstruction with the reterocaval ureter ly-
ing at the level of L3 or L4 .
In our cases Intravenous pyelogram showed that the
upper ureter was S-shaped and was kinked medially to-
wards the midline at the lev el of the transverse process of
the third lumbar vertebra. Also we perform the retrograd e
ureteropyelography and demonstrate the S curve of ret-
rocaval ureter. MRI can demonstrate the course of a
preureteral vena cava, and may be a more detailed and
less invasive imaging procedure, compared with CT and
retrograde Pyelography . Surgical repair is indicated
only when symptoms are present or significant obstruc-
tion exist that have repercussion in renal function. Surgi-
cal correction involves ureteral divisions, with relocation
and ureteroureteral or ureteropelvic reanastomosis [16,
17]. Laparoscopic and robotic minimally invasive repair
of the ureter has been described by a trans or retroperito-
neal approach and should be considered before open
surgery [18,19]. In our cases following the confirmation
of obstruction, surgery was indicated in the form of pro-
ximal ureteric sectioning and ureteral transpositioning of
the retrocaval segment.
Retrocaval ureter should be suspected in any case of
pyelectasis and proximal ureterectasis respectively of the
upper third ureter on the right side.
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IVC: Inferior Vena Cava
D-IVC: Duplication of the IVC
KUB: Kidney Urinary Bladder