Left Preureteral Vena Cava (Retrocaval or Circumcaval Ureter) Associated with Partial Situs Inversus

Left Preureteral Vena Cava (Retrocaval or Circumcaval Ureter) Associated with Partial Situs Inversus

0022-534 7/91/1455-1047$03.00/0 VoL 145, THE JOURNAL OF UROLOGY Copyright© 1991 by AMERICAN UROLOG!ChL ASSOC!ATION, !NC. LEFT PREURETERAL VENA CAVA...

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0022-534 7/91/1455-1047$03.00/0 VoL 145,

THE JOURNAL OF UROLOGY

Copyright© 1991 by AMERICAN UROLOG!ChL ASSOC!ATION, !NC.

LEFT PREURETERAL VENA CAVA (RETROCA VAL OR CIRCUMCA VAL URETER) ASSOCIATED WITH PARTIAL SITUS INVERSUS MORIHIRO WATANABE, SHUNZO KAWAMURA, TERUHIRO NAKADA, NOBUHISA ISHII, KAZUHIKO HIRANO, KAZUO NUMASA WA AND AKIRA IMAMURA From the Department of Urology, Yamagata University School of Medicine, Shinjo Prefectural Hospital, Mitaka Kitakuchi Hospital and Shonai City Hospital, Tokyo and Yamagata, Japan

ABSTRACT

We present a rare case of left preureteral vena cava associated with partial situs inversus. A 68year-old woman was referred to our clinic for further study of left hydronephrosis on computerized tomography. The abdominal viscera were in mirror image and the heart was levocardia. The middle portion of the left ureter was dorsal to the left inferior vena cava. The left ureter was reanastomosed ventral to the inferior vena cava. To our knowledge, coexistence ofpreureteral vena cava and partial situs inversus has not been reported previously in the literature. KEY WORDS:

vena cava, ureter, situs inversus

Ureteral obstruction occasionally offers perplexing diagnostic problems to the urologist. The ureter, by virtue of its long retroperitoneal course, may participate in a host of disease processes in addition to disease of the ureter itself. Preureteral vena cava is an uncommon congenital anomaly causing ureteral obstruction by external compression. Although this condition usually is referred to as retrocaval or circumcaval ureter, its etiology generally is assumed to be due to abnormal development of the inferior vena cava and not the ureter. The term preureteral vena cava characterizes the condition more accurately.1 More than 200 cases have been reported to date and this anomaly has occurred exclusively on the right side. Situs inversus (reverse transposition of thoracic and/or abdominal viscera) is a rare congenital malformation described as the mirror image of normal, and often associated with many cardiovascular and abdominal anomalies. The incidence is 1 in 4,000 to 1 in 10,000 live births. 2 The degree of visceral transposition in this rare anomaly is divided into total and partial. We report a case of left preureteral vena cava associated with partial situs inversus. To our knowledge, this is the second case of left preureteral vena cava, following the case of Brooks, 3 and the first case of this anomaly associated with partial situs 1nversus.

vena cava (fig. 2). A retrograde pyelogram with venacavography confirmed the left preureteral vena cava (fig. 3). At operation the left ureter was dilated, and traced downward and medially, where it dipped behind the inferior vena cava. There were no abnormal vessels truncating from the inferior vena cava. The left ureter surrounding the vena cava was dissected without difficulty, and was normal on inspection and palpation. After the dilated portion of left ureter was excised, the ureter was relocated anterior to the vena cava by an endto-end anastomosis. Convalescence was uneventful. An IVP immediately after the operation revealed a normal course of the left ureter and left hydronephrosis (fig. 4), which improved during 3 years postoperatively. The patient has been well to date (4 years postoperatively) without any serious urological problems. DISCUSSION

The midline is the major determinant in the lateral symmetry of the embryo and represents a developmental field that, if

CASE REPORT

A 68-year-old Japanese woman was referred to our clinic for investigation of left hydronephrosis. Medical history was remarkable for heart disease in her youth and diabetes mellitus for the previous few years. An excretory urogram (IVP) revealed a dilated upper left ureter shifted medially in a J shape and kinking at the L3 to L4 level (fig. 1). The current illness began 2 months before hospitalization, when the patient complained of left flank pain. At physical examination a heart murmur was audible at the third left sternal border. Laboratory values demonstrated a slightly elevated blood urea nitrogen level, elevated blood sugar and proteinuria. The chest x-ray showed levocardia. Partial situs inversus was suspected and other examinations followed. A barium enema revealed that the ascending colon and descending colon were on the right side and there was no transverse colon. On computerized tomography (CT) the liver, gallbladder (with a gallstone), pancreas and lobulated spleen were located in reverse position (fig. 2). The inferior vena cava was on the left side and the left upper ureter was positioned behind the inferior Accepted for publication September 28, 1990.

FIG. 1. IVP shows left hydronephrosis with ureter kinking at L3 to L4 level. Middle portion of ipsilateral ureter deviates medially.

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FIG. 2. Abdominal CT scan demonstrates dilated left ureter ( U) behind left inferior vena cava ( V).

FIG. 4. Postoperative IVP reveals normal course of left ureter and dilated left collecting system. Double-J stent (Medical Engineering Corp., New York, New York) is indwelling.

Only 1 case of left preureteral vena cava has been reported; Brooks described left preureteral vena cava with total situs inversus. 3 Assuming that partial situs inversus and total situs inversus are distinguished from each other, our case probably is the first to be reported of preureteral vena cava associated with partial situs inversus. Other anomalies associated with preureteral vena cava include horseshoe kidney,7 as well as renal agenesis, hydronephrosis, renal malrotation and hypoplasia.8 Hastreiter and Rodriguez-Coronel observed that with an inversus position of the abdominal viscera the inferior vena cava is located on the left side. 9 This may explain the ontogeny of our case. REFERENCES

FIG. 3. Left retrograde pyelogram with venacavography shows left ureter ( U) dorsal to inferior vena cava ( V).

defective, may lead to an abnormal situs determination. Causal heterogeneity has been demonstrated for this developmental field defect and, as an etiology, an autosomal recessive disorder has been proved. 4 Total situs inversus (complete mirror image transposition of thoracic and abdominal viscera) is etiologically and pathogenetically different from partial situs inversus. 5 Cockayne distinguished 3 types of partial transposition of the viscera: 1) partial transposition involving thoracic and abdominal organs, 2) partial or complete transposition of the abdominal viscera without transposition of the thoracic viscera and 3) dextrocardia, with the chambers of the heart forming the mirror image of the normal. 6 The frequency of partial situs inversus is approximately 1 in 40,000 to 1 in 200,000 live births, which is approximately a tenth that of total situs inversus. Preureteral vena cava has a reported autopsy prevalence of approximately 0.9 in 1,000, with a 2.8-fold male predominance. 1 Also, this developmental failure almost always occurs on the right side.

1. Perlmutter, A. D., Retik, A. B. and Bauer, S. B.: Anomalies of the upper urinary tract. In: Campbell's Urology, 5th ed. Edited by P. C. Walsh, R. F. Gittes, A. D. Perlmutter and T. A. Stamey. Philadelphia: W. B. Saunders Co., vol. 2, sect. XII, chapt. 38, pp. 1742-1759, 1986. 2. Ruben, G. D., Templeton, J. M., Jr. and Ziegler, M. M.: Situs inversus: the complex inducing neonatal intestinal obstruction. J. Ped. Surg., 18: 751, 1983. 3. Brooks, R. E., Jr.: Left retrocaval ureter associated with situs inversus. J. Urol., 88: 484, 1962. 4. Zlotogora, J., Schimmel, M. S. and Glaser, Y.: Familial situs inversus and congenital heart defects. Amer. J. Med. Genet., 26: 181, 1987. 5. Warkany, J.: Congenital Malformations: Notes and Comments. Chicago: Year Book Medical Publishers Inc., chapt. 21, p. 183, 1971. 6. Cockayne, E. A.: The genetics of transposition of the viscera. Quart. J. Med., 7: 479, 1938. 7. Youssif, M.: Horseshoe kidney with retrocaval ureter. Eur. Urol., 11: 61, 1985. 8. Kenawi, M. M. and Williams, D. I.: Circumcaval ureter: a report of four cases in children with a review of the literature and a new classification. Brit. J. Urol., 48: 183, 1976. 9. Hastreiter, A. R. and Rodriguez-Coronel, A.: Discordant situs of thoracic and abdominal viscera. Amer. J. Cardiol., 22: 111, 1968.