Duplication of urethra

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Overview

Duplication of the urethra, also called urethral duplication, is a rare birth defect where a person is born with two urethras instead of one. The urethra is the tube that carries urine from the bladder out of the body. In this condition, a second, extra urethra forms during fetal development. The two urethras can be arranged in different ways — one on top of the other, side by side, or branching off from different points along the urinary tract. This condition is far more common in males than females. The extra urethra can cause a range of urinary problems. Some people have urine leaking from an unexpected opening, difficulty controlling urination, a weak or split urine stream, or repeated urinary tract infections. In some cases, the extra urethra is completely blocked and causes no symptoms at all, while in others it connects to the bladder and causes significant problems. Treatment usually involves surgery to remove or repair the extra urethra and correct any related problems with the bladder, kidneys, or genitals. With proper surgical care, most people do very well. The outlook depends on how complex the duplication is and whether other parts of the urinary system are also affected. Early diagnosis and treatment by a specialist are key to the best possible outcome.

Key symptoms:

Two urethral openings visible on the bodyUrine leaking from an unexpected or abnormal openingWeak or split urine streamDifficulty fully emptying the bladderRepeated urinary tract infectionsDribbling of urine after urinatingInability to control urination (incontinence)Pain or discomfort when urinatingAbnormal position of the urethral opening on the penis or genitalsSwelling or blockage in the urinary tract

Clinical phenotype terms (26)— hover any for plain English
EpispadiasHP:0000039Anorectal anomalyHP:0012732ChordeeHP:0000041Septate vaginaHP:0001153GastroschisisHP:0001543Penile hypospadiasHP:0003244Uterus didelphysHP:0003762Urinary bladder wall hypertrophyHP:0008635Clitoral hypertrophyHP:0008665Distal urethral duplicationHP:0008706Coronal hypospadiasHP:0008743Anal fistulaHP:0010447Urethral strictureHP:0012227
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Duplication of urethra.

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No specialists are currently listed for Duplication of urethra.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Duplication of urethra.

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Community

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Latest news about Duplication of urethra

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What type of urethral duplication does my child have, and how does that affect the treatment plan?,Will my child need surgery, and if so, how many operations might be required?,Are there any other parts of the urinary system or other organs that are affected?,What are the risks of surgery, and what does recovery look like?,How will we monitor kidney and bladder function over the long term?,Are there any signs of complications I should watch for at home?,Should my child see any other specialists, such as a kidney doctor or geneticist?

Common questions about Duplication of urethra

What is Duplication of urethra?

Duplication of the urethra, also called urethral duplication, is a rare birth defect where a person is born with two urethras instead of one. The urethra is the tube that carries urine from the bladder out of the body. In this condition, a second, extra urethra forms during fetal development. The two urethras can be arranged in different ways — one on top of the other, side by side, or branching off from different points along the urinary tract. This condition is far more common in males than females. The extra urethra can cause a range of urinary problems. Some people have urine leaking from

How is Duplication of urethra inherited?

Duplication of urethra follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Duplication of urethra typically begin?

Typical onset of Duplication of urethra is neonatal. Age of onset can vary across affected individuals.