Isolated female hypospadias

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Overview

Isolated female hypospadias is a rare birth defect affecting the urinary and genital anatomy in girls and women. In this condition, the opening of the urethra (the tube that carries urine out of the body) is not in its normal position. Instead of being located at the typical spot, the urethral opening may be found on the front wall of the vagina or in another abnormal location. The term 'hypospadias' is much more commonly associated with boys, where the urethral opening is on the underside of the penis, but it can also occur in females, though it is far less recognized and studied. Girls born with this condition may experience symptoms such as an abnormal urine stream that sprays or is difficult to direct, recurrent urinary tract infections, and sometimes difficulty with hygiene. In some cases, the condition may not be noticed until later in childhood or even adolescence. The severity can vary widely from mild displacement that causes few problems to more significant misplacement that affects daily comfort and urinary function. Treatment typically involves surgical correction to reposition the urethral opening to its normal location. The surgery is usually performed by a pediatric urologist and is often done in early childhood when possible. Outcomes after surgery are generally good, and most girls go on to have normal urinary function. Because this condition is isolated, meaning it occurs without other birth defects or syndromes, the overall health outlook is usually very favorable once the anatomical issue is corrected.

Key symptoms:

Abnormal position of the urinary openingUrine stream that sprays or goes in an unexpected directionRecurrent urinary tract infectionsDifficulty with toilet trainingDiscomfort or irritation in the genital areaDifficulty keeping the area clean and dryAbnormal appearance of the external genitaliaVaginal irritation or inflammation

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Isolated female hypospadias.

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No actively recruiting trials found for Isolated female hypospadias at this time.

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Search ClinicalTrials.gov ↗Join the Isolated female hypospadias community →

No specialists are currently listed for Isolated female hypospadias.

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 Isolated female hypospadias.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is my child's hypospadias, and does it require surgical correction?,What is the best age for surgery, and what surgical technique do you recommend?,Are there any additional tests needed to rule out other conditions or syndromes?,What are the risks and expected outcomes of the surgery?,How can we reduce the risk of urinary tract infections before and after treatment?,Will this condition affect my child's future reproductive health?,Is there any chance this condition could be inherited or occur again in future children?

Common questions about Isolated female hypospadias

What is Isolated female hypospadias?

Isolated female hypospadias is a rare birth defect affecting the urinary and genital anatomy in girls and women. In this condition, the opening of the urethra (the tube that carries urine out of the body) is not in its normal position. Instead of being located at the typical spot, the urethral opening may be found on the front wall of the vagina or in another abnormal location. The term 'hypospadias' is much more commonly associated with boys, where the urethral opening is on the underside of the penis, but it can also occur in females, though it is far less recognized and studied. Girls born

At what age does Isolated female hypospadias typically begin?

Typical onset of Isolated female hypospadias is neonatal. Age of onset can vary across affected individuals.