Congenital malformation in the urinary tract

Ureteral Duplication, Ureterocele, and Ectopic Ureter

Development of the urinary organs before birth prepares the body to void urine. Sometimes those organs do not develop correctly, perhaps because of heredity or related to an unknown influence. The result is different anatomy that exists at birth—called congenital malformation.

Congenital malformations of the urinary tract include:

  • Ureteral duplication
  • Ureterocele
  • Ectopic ureter

These conditions will be defined and discussed here.

Treatment overview

If symptoms are mild, the doctor might prefer to monitor the condition and treat symptoms such as urinary tract infection and backward flow of urine. Surgical treatments include endoscopic decompression and partial nephrectomy.

Choice of treatment depends on the patient’s condition:

  • Patient age
  • Overall health
  • Kidney function
  • Problems with urine flow
  • Blockage of urine by a ureterocele
  • Parents’ and surgeon’s preferences

Diagnosis

Definition

Treatment

Ureteral duplication A kidney has two ureters connecting it to the bladder instead of one Monitoring and treatment of symptoms if mild
Ureterocele A pouch-like enlargement of the ureter at the end where it connects to the bladder
  • Endoscopic decompression to puncture the ureterocele surgically, which reduces its size and unblocks urinary flow
  • Partial nephrectomy to remove nonworking tissue from the kidney
Ectopic ureter A ureter that connects the kidney to a site other than the bladder—for example, to the urethra or the vagina
  • Partial nephrectomy to remove nonworking tissue from the kidney
  • Surgical reconstruction of the urinary organs
Fig. 1: Treatment decision making for congenital malformations in the urinary tract.
Fig. 1: Treatment decision making for congenital malformations in the urinary tract.

This information was produced by the EAU Patient Information Working Group, March 2018.

  • Dr. Michele Innocenzi, Rome (IT)
  • Dr. Martin Kubát, Prague (CZ)
  • Dr. Antonin Prouza, Prague (CZ)