Case Presentation of Preureteral Vena Cava and Review of the Literature

doi:10.4236/oju.2013.35038

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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

Email: *[email protected]

Received July 24, 2013; revised August 21, 2013; accepted August 26, 2013

which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

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

1. Introduction

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 [1].

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 [5].

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.

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

lumbar vertebra.

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-

struction.

3. Discussion

The first observed case of retrocaval ureters was de-

scribed by Hochstetter in 1893 [6]. 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 [5]. 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.

N. HYSENI ET AL.

208

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 [12]. 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-

ity.

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 [13].

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 [5]. 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 [14].

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 [15]. 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.

4. Conclusion

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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Abbreviations

IVC: Inferior Vena Cava

D-IVC: Duplication of the IVC

KUB: Kidney Urinary Bladder