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Diagnosis and Treatment of a Retrocaval Ureter

Definition of Retrocaval Ureter

A retrocaval ureter, also called a circumcaval ureter, is a very rare congenital anomaly of the inferior vena cava (IVC). The right ureter passes posterior to the IVC and may be compressed [fig. schematic drawing] (Zhang et al., 1990).

Schematic drawing of a retrocaval ureter.

Epidemiology

1:1500

Etiology:

During embryologic development, the ureter lies between the following fetal veins: dorsally, the supracardinal vein and posterior cardinal vein; ventrally, the subcardinal vein. The infrarenal portion of the IVC normally develops from the right supracardinal vein, so the right ureter lies anterior to the IVC. If the IVC develops from the ventrally located subcardinal vein, the ureter remains posterior and may be compressed between the IVC and the spine [fig. schematic drawing].

Signs and symptoms:

Possible findings include right-sided flank pain, nephrolithiasis, pyelonephritis, and hydronephrosis. Loss of right renal function may also occur.

Diagnosis:

Ultrasonography:

Renal ultrasonography often shows right-sided hydronephrosis with dilation of the proximal ureter. The distal ureter is usually not dilated.

CT or MRI urography:

CT or MRI urography allows definitive diagnosis and identification of associated vascular anomalies.

Intravenous urography:

The right mid-ureter turns abruptly medially; a change in caliber may also be seen. Distal to the kink, contrast is often no longer visible, giving the ureter a "J"-shaped appearance.

Retrograde pyelography:

Shows a spiral (S-shaped) course of the ureter around the IVC [fig. retrocaval ureter].

Renal function testing:

Renal function testing determines split renal function and the degree of obstruction.

Right-sided retrocaval ureter: In retrograde pyelography, the ureter follows a spiral course around the inferior vena cava, with hydronephrosis. See also fig. laparoscopic ureteroureterostomy in the same patient. With kind permission, Prof. Dr. R. Harzmann, Augsburg.

Therapy:

Surgical treatment is indicated only for complications, such as intermittent flank pain, nephrolithiasis, recurrent infections, or significant hydronephrosis with loss of kidney function. It involves mobilization of the ureter, lysis of adhesions between the ureter and IVC, transection of the ureter with resection of strictured segments, transposition of the ureter anterior to the IVC, and ureteroureterostomy of the mobilized ends. Surgery may be performed by an open, laparoscopic, or robot-assisted approach. The technical challenge is dissection near the IVC, with the associated risk of vascular injury and bleeding. If fibrotic adhesions between the ureter and IVC are severe, the atretic ureteral segment may be left posterior to the IVC [fig. laparoscopic ureteroureterostomy]. Open surgery requires a substantial retroperitoneal or transperitoneal approach because of the central position of the IVC in the abdominal cavity. Laparoscopy can reduce morbidity. Rarely, nephrectomy is required when the kidney is nonfunctioning.

References

Zhang, X. D.; Hou, S. K.; Zhu, J. H.; Wang, X. F.; Meng, G. D. & Qu, X. K. Diagnosis and treatment of retrocaval ureter.
Eur Urol, 1990, 18, 207-210

  Deutsche Version: Retrokavaler Ureter

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