Overview
Anterior urethral valve (AUV) is a rare birth defect in boys where an abnormal flap of tissue forms inside the urethra — the tube that carries urine out of the body. This flap acts like a one-way valve that partially or completely blocks the flow of urine. Unlike the more common posterior urethral valve, which forms at the back of the urethra near the bladder, an anterior urethral valve forms closer to the outside of the body, usually in the middle or front section of the urethra. AUV is sometimes also called anterior urethral obstruction or anterior urethral diverticulum when a small pouch forms alongside the valve. Because urine cannot drain properly, pressure builds up in the bladder and can back up into the kidneys over time. This can cause the bladder to become enlarged and the kidneys to swell — a condition called hydronephrosis. If left untreated, the kidneys can be permanently damaged. Boys with AUV may have trouble urinating, a weak urine stream, or may wet themselves without meaning to. The good news is that AUV can often be treated successfully with surgery. A pediatric urologist can remove or destroy the abnormal valve tissue using a small camera inserted into the urethra, a procedure called endoscopic valve ablation. Early diagnosis and treatment are very important to protect kidney function. With prompt care, many boys go on to live healthy lives, though some may need long-term follow-up to monitor their kidneys and bladder.
Key symptoms:
Weak or dribbling urine streamDifficulty starting to urinateSwollen or enlarged bladder that can be felt in the bellySwollen kidneys (hydronephrosis) seen on ultrasoundUrinary tract infections that keep coming backBedwetting or daytime accidents in older boysStraining or pushing hard to urinateUrine leaking or dribbling after urinatingPoor weight gain or feeding problems in newbornsSwelling of the urethra visible on the underside of the penis
Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Anterior urethral valve.
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Specialists
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Anterior urethral valve.
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Caregiver Resources
NORD Caregiver Resources
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Has my son's kidney function been affected, and how will we monitor it over time?,What type of surgery is recommended, and what are the risks and expected recovery?,Will my son need preventive antibiotics, and for how long?,What signs should I watch for at home that would mean I need to seek emergency care?,Are there any long-term effects on the bladder, and will my son need bladder retraining?,How often will follow-up appointments and tests be needed after surgery?,Could this condition affect my son's fertility or urinary function as an adult?
Common questions about Anterior urethral valve
What is Anterior urethral valve?
Anterior urethral valve (AUV) is a rare birth defect in boys where an abnormal flap of tissue forms inside the urethra — the tube that carries urine out of the body. This flap acts like a one-way valve that partially or completely blocks the flow of urine. Unlike the more common posterior urethral valve, which forms at the back of the urethra near the bladder, an anterior urethral valve forms closer to the outside of the body, usually in the middle or front section of the urethra. AUV is sometimes also called anterior urethral obstruction or anterior urethral diverticulum when a small pouch fo
How is Anterior urethral valve inherited?
Anterior urethral valve follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Anterior urethral valve typically begin?
Typical onset of Anterior urethral valve is neonatal. Age of onset can vary across affected individuals.