Description, Causes and Risk Factors:
In urography, the medial deviation of the right ureter in the rare circumstance in which it passes behind the inferior vena cava before entering the pelvis.
Retrocaval ureter also referred to as circumcaval ureter or pre-ureteral vena cava is a rare congenital anomaly with the ureters passing posterior to the inferior vena cava. The ureter classically course medially behind the inferior vena cava winding around it and then passes laterally in front of it to then course distally to the bladder. Though it is a congenital anomaly, patients do not normally present with symptoms until the 3rd and 4th decades of life, from a resulting hydronephrosis. The hydronephrosis may be due to kinking of the ureter, a ureteric segment that is adynamic or compression against the psoas muscle. It was initially considered as aberration in ureteric development; however current studies in Embryology have led to it being considered as an aberration in the development of the inferior vena cava. Hence it is being suggested that the anomaly be referred to as a pre-ureteral vena cava
Retrocaval ureter is a rare congenital anomaly occurring with incidence of about 1 in 150000 people with a three to four times male predominance in autopsy studies. Though similar male to female ratios are seen in Clinical practice, few clinical cases have been reported world wide.
Majority of patients presenting with symptoms, present with flank or abdominal pain that can be intermittent, dull and aching and is commonly due to ureteric obstruction and associated hydronephrosis. Some patients may present with recurrent urinary tract infection and hematuria. Renal calculi and pyonephrosis may complicate the condition. Some cases are found incidentally during radiographic imaging for other conditions.
Imaging studies are usually accurate and sufficient for diagnosis of the disorder. Ultrasonography is a noninvasive method to demonstrate the anatomy of the retrocaval ureter and follow-up patients for hydronephrosis, parenchymal atrophy, and nephrolithiasis. IVU (intravenous urography) in the early stage of ureteral stenosis only shows dilatation of the renal pelvis, calyces, and upper ureter above the site of obstruction. AP and RP is helpful in revealing the characteristic "S" shape and midline deviation of the ureter. Retrograde pyelography combined with inferior venacavography can clearly confirm the diagnosis but is invasive. Enhanced CT scan with ureteral catheterization can demonstrate the opacified catheter posterior to IVC. New imaging studies such as spiral CT scan and magnetic resonance imaging (MRI) are of great help in delineating the anatomy non-invasively. An isotope renal scan can reveal the degree of obstruction, and differential renal function to help decide the therapeutic modalities.
Treatment is surgical and involves division of the ureter and re-positioning it anterior to the inferior vena cava. This may be achieved through an anastomosis between the renal pelvis and the ureter or a ureteroureteral anastomosis over a double-J stent. The segment behind the inferior vena cava which may be aperistaltic is either excised or left in situ. Surgical intervention is for symptomatic cases. Patients with minimal caliceal dilatation and no significant symptoms do not need surgery but need to be followed up. Transperitoneal and retroperitoneal laparoscopic repair of the retrocaval ureter has been described offering advantages of a shorter hospital stay and early recovery. Important differential diagnosis includes retroperitoneal fibrosis and retro peritoneal masses displacing the ureters from its normal course.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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