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.
REFERENCES
[1] S. H. Kim, “Retrocaval Ureter,” In: K. W. Ashcraft and T.
M. Holder, Eds., Pediatric Surgery, W.B. Saunders, Phi-
ladelphia, 1993, p. 603.
[2] V. P. Chuang, C. E. Mena and P. A. Hoskins, “Congenital
Anomalies of the Inferior Vena Cava. Review of Em-
bryogenesis and Presentation of a Simplified Classifica-
tion,” British Journal of Radiology, Vol. 47, No. 556,
1974, pp. 206-213. doi:10.1259/0007-1285-47-556-206
[3] R. N. Schlussel and A. B. Retik, “Preureteral Vena
Cava,” In: L. R. Kavoussi, A. C. Novick, A. W. Partin
and C. A. Peters, Eds., Campbell-Walsh Urology, El-
Sevier Saunders, Philadelphia, 2007, pp. 3418-3420.
[4] A. Lesma, A. Bocciardi and P. Rigatti, “Circumcaval U re-
ter: Embryology,” European Urology Supplements, Vol.
5, No. 5, 2006, pp. 444-448.
[5] C. A. Peters, R. N. Schlussel and C. Mendelson, “Preuret-
eral Vena Cava,” In: A. J. Wein, L. R. Kavoussi, A. C.
Novick, A. W. Partin, C. A. Peters, Eds., Campbell-Wal-
sh Urology, El-Sevier Saunders, Philadelphia, 2012, pp.
3263-3265.
[6] R. O. Olson and G. Austen Jr., “Postcaval Ureter—Report
and Discussion of a Case with Successful Surgical Re-
pair,” The New England Journal of Medicine, Vol. 242,
No. 25, 1950, pp. 963-968.
doi:10.1056/NEJM195006222422501
[7] W. Dreyfuss, “Anomaly Simulating a Retrocaval Ureter,”
Journal of Urology, Vol. 82, 1959, pp. 630-632.
[8] I. Lerman, S. Lerman and F. Lerman, “Retrocaval Ureter:
Report of a Case,” The New England Jou rnal of Medicine,
Vol. 53, 1956, p. 74.
[9] M. R. Peisojovich and S. J. Lutz, “Retrocaval Ureter—A
Case Report and a Successful Repair with a New Surgical
Technique,” Michigan Medicine, Vol. 68, No. 21, 1969,
pp. 1137-1141.
[10] M. Watanabe, S. Ka wamura, T. Nakada, et al., “Left Pre-
ureteral Vena Cava (Retrocaval or Circumcaval Ureter)
Associated with Partial Situs Inversus,” Journal of Urol-
ogy, Vol. 145, 1991, pp. 1047-1048.
[11] I. Rubinstein, A. G. Calvacanti, A. F. Canalini, M. A.
Freitas and P. M. Accioly, “Retrocaval Ureter: Two Case
Reports,” Ghana Medical Journal, Vol. 45, No. 4, 2011,
pp. 177-180.
[12] W. T. Ng and S. S. M. Ng, “Double Inferior Vena Cava:
A Report of Three Cases,” Singapore Medical Journal,
Vol. 50, No. 6, 2009, pp. 211-213.
[13] E. Bateson and D. Atkinson, “Circumcaval Ureter: A
New Classification,” Clinical Radiology, Vol. 20, No. 2,
1969, pp. 173-177. doi:10.1016/S0009-9260(69)80166-2
[14] M. M. Kenawi and D. I. Williams, “Circumcaval Ureter:
A Report of 4 Cases in Children with a Review of the
Copyright © 2013 SciRes. OJU