Spina bifida-hypospadias syndrome

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Overview

Spina bifida-hypospadias syndrome is an extremely rare condition that combines two birth defects: spina bifida and hypospadias. Spina bifida is a defect in which the bones of the spine (vertebrae) do not fully close around the spinal cord during early development in the womb. This can range from mild forms with few symptoms to more severe forms where the spinal cord and its coverings push through an opening in the back. Hypospadias is a condition in males where the opening of the urethra (the tube that carries urine out of the body) is located on the underside of the penis rather than at the tip. Children born with this syndrome may experience problems related to both conditions, including difficulties with walking and movement, bladder and bowel control issues, and abnormalities of the urinary and reproductive systems. The severity can vary widely from person to person. Some individuals may have mild spinal defects with minimal impact, while others may have more significant neurological problems affecting the legs and lower body. Treatment is typically focused on managing each component of the syndrome. Spina bifida may require surgical repair shortly after birth, along with ongoing care for any neurological complications. Hypospadias is usually corrected through surgery, often performed in infancy or early childhood. A team of specialists including neurosurgeons, urologists, and rehabilitation doctors typically work together to provide comprehensive care. The exact genetic cause of this syndrome is not fully understood, and research into this condition remains limited due to its rarity.

Key symptoms:

Opening in the spine (spina bifida)Abnormal position of the urinary opening on the penis (hypospadias)Weakness or paralysis in the legsBladder control problemsBowel control problemsDifficulty walkingAbnormal curvature of the spineClubfoot or other foot deformitiesHydrocephalus (fluid buildup in the brain)Urinary tract infectionsReduced sensation in the lower bodyLearning difficulties in some cases

Clinical phenotype terms (3)— hover any for plain English
Spinal dysraphismHP:0010301
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 Spina bifida-hypospadias syndrome.

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No actively recruiting trials found for Spina bifida-hypospadias syndrome at this time.

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No specialists are currently listed for Spina bifida-hypospadias syndrome.

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 Spina bifida-hypospadias syndrome.

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Community

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is my child's spinal defect, and what does this mean for their development?,Will my child need surgery for the spina bifida and/or hypospadias, and when should it be done?,What kind of bladder and bowel management will my child need?,Should we have genetic testing done, and what would it tell us about the risk for future children?,What therapies (physical, occupational) should we start, and how often?,Are there signs of hydrocephalus, and will a shunt be needed?,What specialists should be part of my child's care team, and how often should we see them?

Common questions about Spina bifida-hypospadias syndrome

What is Spina bifida-hypospadias syndrome?

Spina bifida-hypospadias syndrome is an extremely rare condition that combines two birth defects: spina bifida and hypospadias. Spina bifida is a defect in which the bones of the spine (vertebrae) do not fully close around the spinal cord during early development in the womb. This can range from mild forms with few symptoms to more severe forms where the spinal cord and its coverings push through an opening in the back. Hypospadias is a condition in males where the opening of the urethra (the tube that carries urine out of the body) is located on the underside of the penis rather than at the t

At what age does Spina bifida-hypospadias syndrome typically begin?

Typical onset of Spina bifida-hypospadias syndrome is neonatal. Age of onset can vary across affected individuals.