What is ectopic ureter?

Ureters are the two funnel-shaped tubes that carry urine from the kidneys to the bladder. A ureter that does not connect properly to the bladder and drains somewhere outside of the bladder is called an ectopic ureter. In boys the ectopic ureter usually drains into the urethra closer to the prostate. In girls the ectopic ureter usually drains into the reproductive organs or urethra.

Ectopic ureters are commonly associated with a duplex kidney, where one kidney has two separate ureters; one of the ureters drains normally into the bladder, but the second is ectopic.

Signs and symptoms

• Hydronephrosis: When the ectopic ureter connects outside the bladder, the insertion is usually narrow, creating a level of obstruction. The urine that is draining from the kidneys backs up and causes the kidney and ureter to dilate (hydronephrosis).
• Urinary Tract Infection (UTI): Since there is some level of obstruction associated with an ectopic ureter, poor drainage of urine may increase the chances of a UTI. Also, because of the abnormal insertion of the ureter, children with an ectopic ureter may have vesicoureteral reflux (VUR) (urine travels back up the ureter and into the kidney), this can also increase the chance of a UTI.
• Incontinence: Girls with an ectopic ureter may present with continuously damp underwear, since the ureter is connected outside the bladder, below the sphincter mechanism (the “hold on“ muscle). This condition is usually apparent after toilet training. These children differ from children who have intermittent wetting because girls with an ectopic ureter have a constant dampness due to urine dripping rather than occasional loss of bladder control.

Testing and diagnosis

Your child may undergo a variety of tests to help confirm his diagnosis and evaluate the extent of his condition.

• Renal Bladder ultrasound: This procedure uses sound waves to outline the kidneys and bladder. If ureteral dilation is present, it will enable us to see the degree of dilation.
• Voiding cystourethrogram (VCUG): A catheter (tube) is inserted through your child’s urethra into the bladder. The tube will be used to slowly fill the bladder with a solution. While the bladder is being filled, a special machine (fluoroscopy) is used to take pictures. The radiologist looks to see if any of the solution is going back up into the kidneys, which confirms the diagnosis of VUR.
• MAG III renal scan: This study will be performed to determine how each kidney is functioning and will determine the degree of blockage, if any. This test involves the placement of an intravenous line (IV). This IV is used to inject a special solution called an isotope into the veins. The isotope makes it possible to see the kidneys clearly. Pictures of the kidneys will be taken will a large X-ray machine that rotates around your child.
• MRI/MRU: MRI is a radiation-free diagnostic procedure that uses a combination of a large magnet, radiofrequencies and a computer to produce detailed images of the body. Magnetic resonance urography (MRU) creates detailed pictures of the kidneys, ureters and bladder. These tests can give detailed images of where the ectopic ureter inserts.

Treatment

Surgical options

Cutaneous distal ureterostomy: This may be necessary in a newborn with massive ureteral dilation. The ureter is surgically brought to the surface of the skin to allow it to drain urine freely into the diaper. This allows the affected kidney and ureter to decompress. Around 18 months of age, the ureter is then reimplanted into the correct position in the bladder.

Ureteral reimplantation: Under general anesthesia and through a lower abdominal incision, the ureter is reimplanted into the correct position where it joins the bladder. At CHOP this procedure can be done through a minimally invasive surgery (MIS) using a laparoscopic or robotic approach. The minimally invasive approach is accomplished through three small incisions, the size of a grain of rice. The advantages are smaller, less visible incisions and overall, a quicker recovery period. The minimally invasive technique is not appropriate for all children. Each year, approximately 50 ureteral reimplantations are done at CHOP. Your provider will work with you and your family to determine what kind of intervention is best.

Partial nephrectomy: If the ectopic ureter is associated with a duplex kidney and the upper pole of the kidney does not function well, it might be recommended to remove that portion of the kidney. At CHOP a partial nephrectomy can be done through a minimally invasive surgery (MIS).

Ureteropyelostomy: If the ectopic ureter is associated with a duplex kidney, the upper pole of the duplex kidney can be divided and connected to the normal lower part of the kidney. This allows the urine from the upper part of the kidney to drain normally. This procedure can also be done through a minimally invasive surgery (MIS).