Retrocaval ureter is a rare anomaly causing upper urinary tract obstruction, usually presented later in life as recurrent flank or abdominal pain and recurrent urinary tract infection. There may be associated abnormalities of other organ systems as well. We present a rare case of retrocaval ureter with contralateral renal agenesis with renal failure. Surgical correction with ureteropyelostomy was successful.
Dheeraj Kumar Gupta, Vishwajeet Singh, Rahul Janak Sinha
Submitted January 3, 2012 - Accepted for Publication February 10, 2012
KEYWORDS: Retrocaval ureter, ureteropyelostomy, surgical cause of renal failure
CORRESPONDENCE: Vishwajeet Singh, MS, MCh (Urology), Chhatrapati Sahuji Maharaj Medical University (Formerly KGMC), Lucknow, Uttar Pradesh, India ().
CITATION: UroToday Int J. 2012 June;5(3):art 27. http://dx.doi.org/10.3834/uij.1944-5784.2012.06.14
Retrocaval ureter is a rare congenital anomaly associated with upper urinary tract obstruction due to the passage of the ureter posterior to the inferior vena cava (IVC). Many times it is associated with abnormalities of other organ systems as well. This anomaly is typically diagnosed by the fishhook-shaped or the sickle-shaped appearance of the upper ureter on intravenous urogram (IVU) and can be confirmed by a CT scan/MRI. We present a rare case of retrocaval ureter and contralateral renal agenesis with renal failure.
A 17-year-old female presented with intermittent right flank pain, dysuria, and intermittent fever for 2 months. Her physical examination was unremarkable except for a palpable, mildly tender right flank lump. Urine analysis showed 12 to 15 pus cells/HPF, and the urine culture/ sensitivity showed E. coli, sensitive to amikacin and ciprofloxacin. The serum creatinine was 2.9 mg/dl. The renal ultrasound showed right gross hydronephrosis with internal echoes, a mildly dilated upper ureter, and empty left renal fossa (Figure 1). Initially, the patient was managed with right percutaneous nephrostomy. The serum creatinine became normal 1 week following nephrostomy. The nephrostogram showed a grossly dilated right pelvicalyceal system with a curved upper ureter and abrupt cutoff at the L3 level (Figure 2[a]). The retrograde ureteropyelogram showed a medial deviation of the right upper ureter with a curved ureter at the L3 vertebra, from the medial to the lateral side, and narrow lumen (Figure 2[b]). The magnetic resonance (MR) urogram was suggestive of right retrocaval ureter. The DTPA scan showed right subrenal obstruction and an absent left kidney.
The patient was subjected to midline laparotomy. Prior to that, cystoscopy and right ureteric catheterization was done. On exploration, the right upper ureter was retrocaval in position (Figure 3). The retrocaval portion of ureter was atretic, it was excised, and the proximal end of the ureter was spatulated. End-to-end ureteropyelostomy, over 6 Fr/26 cm double-J stent, was performed. Her postoperative period was uneventful. The histopathology of the excised atretic segment of the ureter showed chronic inflammation and fibrosis. The double-J stent was removed after 6 weeks. The DTPA scan 4 weeks following stent removal showed no subrenal obstruction. The patient is doing well in her last 1 year of follow-up.
Retrocaval ureter was first reported by Hochstetter in 1893 . Its incidence is about 1 in 1 100 with a 2.8:1 male-female ratio . Retrocaval ureter almost invariably involves the right side, but in patients with situs inversus, it can be on the left side.
Although the lesion is congenital, it is usually present by the third or fourth decade of life . Retrocaval ureter results from altered vascular, rather than ureteric, development. Persistence of the posterior cardinal vein as the infrarenal IVC is the cause of the development of this anomaly .
The reported literature revealed that nearly 20% of retrocaval ureter cases present with concomitant abnormalities with preponderance from the cardiovascular system and the genitourinary tract. Various associated anomalies include congenital variations of inferior vena cava, brachial arch syndrome, horseshoe kidney, myelomeningocele, hypospadias, Turner syndrome, esophageal atresia, an abnormal left kidney (agenetic, ectopic, or malrotated), and cardiovascular anomalies such as situs inversus, supernumerary lumbar vertebra syndactyly, and yolk sac tumor .
Patients with retrocaval ureter usually present with recurrent flank pain, recurrent urinary tract infection, and nephrolithiasis. This entity may be totally asymptomatic, too . Based on the intravenous urogram (IVU) findings, Bateson and Atkinson classified retrocaval ureter into 2 clinical types: the more common, type I, has moderate-severe hydronephrosis and fishhook-shaped deformities of the upper ureter. The less common, type II, has less angulated “sickle-shaped” deformities with milder/no hydronephrosis . This anomaly is typically diagnosed by a fishhook-shaped or a sickle-shaped appearance of the upper ureter on IVU and can be confirmed by a CT scan/MRI. A spiral CT scan of the abdomen is the investigation of choice.
Surgical correction involves uretero-ureteral or ureteropelvic anastomosis with excision or a bypass of the retrocaval segment, either by an open or laparoscopic approach.
In our case, the patient had a solitary right kidney with retrocaval ureter and renal failure, initially managed by percutaneous nephrostomy, and, subsequently, a ureteropyelostomy was done. Based on our findings of retrograde ureteropyelogram and MR urogram, it was a type 1 retrocaval ureter. The renal failure in our patient was most likely was due to the infection and debris in the pelvicalyceal system, which would have aggravated the obstruction in an already obstructed (retrocaval segment of the ureter) system. The percutaneous nephrostomy was done to relieve the obstruction, which must have prevented the irreversible tubular damage. This is the most probable reason for the reversal of renal failure and stable serum creatinine over 12 months of follow-up.
Retrocaval ureter with contralateral renal agenesis presenting with renal failure is a rare condition. Initial percutaneous nephrostomy followed by ureteropyelostomy should be the treatment of choice.
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