Duplication of urethra

Other Names: Urethral duplication

Duplication of urethra is a very rare condition where there is an extra urethra, instead of the normal one. The urethra is the tube that connects the urinary bladder to the genitals for the removal of fluids from the body. In men, the urethra is a long tube that runs through the penis. In women, the urethra is shorter and emerges above the vaginal opening.

Duplicated ureter

Classification

Urethral duplications can be classified into three types: incomplete urethral duplication (type 1), complete urethral duplication (type 2), and urethral duplication as a result of two bladders

Cause

The exact cause of this condition is unknown; but it is probably due to a defect during the development of the embryo.

Signs and symptoms

There are 3 different types of duplication of urethra. In type 1, the extra urethra arises from the primary urethra and may or may not extend to the external genitals. In type 2, the extra urethra either arises from another opening in the bladder or connects with the primary ureter at the opening of the bladder and then extends to the external genitals. The urethra can also connect with the primary urethra at the opening of the bladder and extend into the perineum (also called the Y-type). Type 3 is when there are two bladders that each give rise to a separate urethra that exits through the external genitals. The clinical features of this abnormality are variable. People with complete urethral duplication can be asymptomatic or can present with a double stream of urine, incontinence, recurrent infections, or outflow obstructions. A double stream is the most common complaint and can be annoying, depending on where the extra opening in the genitals is located.

Diagnosis

Prenatally diagnosed hydronephrosis (fluid-filled kidneys) suggest post-natal follow-up examination. The strongest neo-natal presentation is urinary tract infection. A hydronephrotic kidney may present as a palpable abdominal mass in the newborn, and may suggest an ectopic ureter or ureterocele. In older children, ureteral duplication may present as: Urinary tract infection - most commonly due to vesicoureteral reflux (flow of urine from the bladder into the ureter, rather than vice versa). Urinary incontinence in females occurs in cases of ectopic ureter entering the vagina, urethra or vestibule.

Treatment

Treatment of this condition depends on the anatomy of the duplication and its clinical features. Some people with mild symptoms do not require surgery. However, sugery might be considered for disturbing symptoms, such as an annoying double stream or incontinence, or for cosmetic reasons.

NIH genetic and rare disease info

• NIH info on Duplication of urethra

Duplication of urethra is a rare disease.

Duplication of urethra Resources
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