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Retrocaval Ureter - Types, Symptoms, Diagnosis, and Treatment

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The retrocaval ureter is an occasional anomaly of the inferior vena cava where the ureter lies behind it. Read the article for further details.

Written byDr. Antara Mukherjee

Medically reviewed byDr. Madhav Tiwari

Published At December 9, 2022
Reviewed AtOctober 15, 2024

Introduction:

The retrocaval ureter is an uncommon anomaly that has been present since birth. It is also known as circumcaval ureter or pre-ureteral vena cava. A vascular abnormality is usually found on the right side. This anomaly was first described in 1893 in a dead body. Harrill was the first person to describe the condition clinically.

At first, it was thought to be an anomaly in the development of the ureter but was later found to be caused by abnormal development of the vena cava. Males are three times more prone to the condition than females.

The anomaly's occurrence rate is around one in one thousand individuals. It has been associated with Goldenhar syndrome (a syndrome characterized by craniofacial abnormalities along with vertebral, cardiac, renal, and central nervous system defects) and inferior vena cava duplication (a congenital vascular anomaly where the vena cava is doubled). The first case was reported in Ghana. Although it is extremely rare, the retrocaval ureter is one of the most common defects present since birth, resulting in obstruction in the ureters.

What Is the Definition of Retrocaval Ureter?

Retrocaval ureter or circumcaval ureter is a scarce congenital variation of the ureter. This tube transports urine from the kidney to the bladder, where the ureter crosses the IVC (inferior vena cava) instead of lying anterior to it. Such positioning may result in ureteral obstruction, hydronephrosis, and its related complications, which include pain, hematuria, and recurrent urinary tract infections. It is typically diagnosed with ultrasound, CT, or MRI and may call for surgical intervention to correct the blockage and allow urine to flow freely.

What Is the Pathophysiology of Retrocaval Ureter?

The ureter, a tubular structure that connects the kidneys with the urinary bladder, passes from behind the inferior vena cava (a large blood vessel that carries blood from the lower extremities and organs to the heart). Retrocaval ureter usually has no symptoms and is diagnosed incidentally.

This abnormality takes place around the fourth and sixth week of pregnancy. During this period, the inferior vena cava forms from the sub cardinal vein (a blood vessel located on either side of the abdomen) instead of the supra cardinal vein (blood vessels located on either side of the main blood vessel that carries blood from the heart to different body parts).

When it develops from the subcardinal vein, the ureter gets trapped at the back, leading to the formation of the retrocaval ureter. In case progressive kidney damage occurs, early diagnosis with radiological imaging and surgical correction is needed to save the remaining kidney.

How Many Types of Retrocaval Ureters Are There?

There are two types of retrocaval ureters. Bateson and Atkinson reported these.

  • Type l (Low Loop) - This is the most common type of retrocaval ureter. In this type, the ureter goes behind the inferior vena cava, forming a fish hook or reverse J shape. It is also associated with hydroureteronephrosis.

  • Type ll (High Loop) - In this type, the ureter goes behind the inferior vena cava, giving a sickle-shaped look. It is associated with a milder version of hydroureteronephrosis.

What Are the Signs and Symptoms of Retrocaval Ureter?

The retrocaval ureter is usually asymptomatic, but in some instances, it might give rise to symptoms such as.

  • Pain in the right side or flank pain.

  • Nephrolithiasis - These are kidney stones made up of minerals and salts.

  • Pyelonephritis - In this condition, there is inflammation of the kidneys due to bacterial infection.

  • Hematuria - It is the presence of blood in the urine.

  • Urine infections.

  • Hydronephrosis - It is the swelling of the kidneys due to the accumulation of urine. This occurs when urine cannot drain out of the kidneys due to a blockage or stricture anywhere in the urinary system.

  • Loss of function in the right kidney.

How Is Retrocaval Ureter Diagnosed?

The retrocaval ureter is usually diagnosed when hydronephrosis progresses to a stage where it becomes symptomatic during adulthood because the inferior vena cava squeezes the ureter against the muscle in the lower region of the spine or because a part of the ureter twists. In sporadic cases, kids have also been diagnosed with the condition.

The retrocaval ureter is diagnosed based on radiological findings.

  • Intravenous Urography - This is an examination of the urinary tract with the help of X-rays. This helps the physician to examine the kidneys, bladder, and ureters.

  • Retrograde Pyelogram - This technique helps diagnose various obstructions that cannot be seen in other modalities.

  • CT Urogram - A computed tomography (CT) urogram is carried out by injecting contrast into the veins. This process can diagnose the positioning of the ureter concerning the inferior vena cava and help rule out the presence of any mass or fibrosis behind the membrane lining the abdominal cavity.

  • Magnetic Resonance Urography - This diagnostic procedure uses magnetic resonance to create images of the kidneys, bladder, and ureters.

How Is Retrocaval Ureter Treated?

The treatment of the retrocaval ureter depends on the clinical and radiological findings.

  • Surgery - Surgical interventions are a standard treatment modality in case of complications. Surgical intervention is required for type I retrocaval ureters with hydronephrosis, loss of kidney function, and recurrent urine infections. During surgery, the part of the ureter behind the inferior vena cava is removed and stitched in front of it. Surgical removal of the ureter is sometimes required to prevent continuous obstruction.

  • Ureteroureterostomy - This surgery connects the ends of the excised ureter. It is also known as van hook surgery.

  • Nephrectomy - Nephrectomy, or the surgical removal of the kidney, is usually indicated in case of severe hydronephrosis, loss of kidney function, or severe infection.

  • Conservative Treatment - This is done in individuals without hydronephrosis, infection, or kidney stone formation.

  • Laparoscopy - This minimally invasive keyhole surgery is done without significantly cutting the skin.

Open surgery is successful but requires significant cuts on the skin, leading to a lot of pain after the surgery with a prolonged healing period. Laparoscopic surgery is done because of its minimally invasive technique and the lesser time required for healing.

Conclusion:

The retrocaval ureter is a rare congenital condition that is caused by an abnormal path of the ureter that encircles the inferior vena cava. It is usually asymptomatic and gives rise to symptoms only when the obstruction in the ureters causes an accumulation of urine in the kidneys or hydronephrosis, which progresses to a great extent. Open surgery is preferred over laparoscopic procedures. The prognosis after the procedure is acceptable. Early diagnosis and treatment might help preserve the remaining kidney function, thus reducing the mortality risk. Long-term follow-up is needed after surgery.

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Frequently Asked Questions

It is a rare congenital anomaly of the urinary system. The anomaly in which the ureter is abnormally present behind the inferior vena cava. It results in the narrowing of the ureter, leading to the obstruction of the flow of urine and accumulation of urine in the kidneys. The condition is known as hydronephrosis.
The retrocaval is present behind the inferior vena cava. The inferior vena cava is a large vein that carries deoxygenated blood from the body to the heart. In the retrocaval ureter, the ureter is located behind the inferior vena cava, leading to obstruction of the ureter. The retrocaval is located at the level of the renal pelvis. It is the area where urine is collected from the kidney.
The retrocaval ureter is also known as the circumcaval ureter. The rare congenital disorder of the urinary system in which the ureter is present behind the inferior vena cava. The term circumcaval ureter is a condition in which the ureter creates a loop around the inferior vena cava and leads to circumferential obstruction.
 
The retrocaval ureter is located on the right side but can be present on the left side. The abnormality is considered to be a bilateral embryonic defect that affects the development of the ureter. It can also result in the abnormal positioning of the inferior vena cava. Retrocaval ureter on the left side is more uncommon than on the right side. 
The treatment depends on the severity and degree of ureter obstruction. The doctor may recommend surgical intervention to relieve the ureteral obstruction.  The choice of treatment depends on the individual case. The surgery also prevents further complications such as urinary tract infections and kidney damage. The surgery can be open surgery or minimally invasive techniques.
 
Retro means backward or behind and caval refers to the inferior vena cava. The retrocaval is present behind the inferior vena cava. The rare congenital anomaly in which the ureter is located behind the inferior vena cava leads to obstruction of the ureter. It can cause kidney damage and urinary tract infections.
The various issues are as follows:
- The retrocaval ureter can compress the ureter, leading to urinary obstruction.
- The condition can lead to repeated urinary tract infections.
- The accumulation of urine may lead to swelling of the kidneys. 
- It may increase the risk of kidney stones.
- If left untreated may lead to chronic kidney disease.
The ureter is at risk in various locations. The common areas where the ureter can be at risk are as follows:
- Retrocaval Region: The congenital anomaly in which the ureter is present behind the inferior vena cava, leading to ureter obstruction.
- Ureteropelvic Junction: The junction where the ureter meets the renal pelvis, leading to obstruction.
- Ureterovesical Junction: The junction where the ureter meets the bladder, leading to obstruction and backflow of the urine to the kidneys.
 
The most common location of ureter stones is the lower part of the ureter. The area is narrow and the stones become stuck and cause obstruction. The stones can also form in the other areas of the ureter such as the upper or mid ureter. Ureter stones vary in shape and size. The stones consist of different materials such as uric acid, calcium oxalate, and struvite.
The ureter cannot repair itself after an injury. There may be a formation of scar tissue or partial-thickness defects. The scarring depends on the location and severity of the injury. Small injuries may heal without scarring. Large injuries with full-thickness defects may lead to more scarring and narrowing of the ureter. If left untreated may lead to kidney damage.
The treatment depends on the cause and severity of the condition. The condition can be cured by medical and surgical interventions. The infections such as urinary tract infections can be treated by anti-inflammatories and antibiotics. Kidney stones in the ureter can be treated with shock wave therapy. In case the ureter is obstructed or damaged can be treated with surgical intervention.
The risks and complications of ureter surgery are as follows:
- Bleeding.
- Infection.
- Injury to adjacent organs.
- Chronic pain.
- Loss of bladder control.
- Scarring and narrowing of the ureter.
The consequences of ureter damage are as follows:
- Urine leakage.
- Obstruction of urine flow.
- Infections.
- Kidney damage.
- Chronic pain.
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Dr. Madhav Tiwari from iCliniq
Dr. Madhav Tiwari

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