My Baby Has Bladder Exstrophy
Medically reviewed by Dr. Michael Okonkwo, MD, FAAP · Board-Certified Neonatologist
Content reviewed against published NIH, AAP, Children's Hospital guidelines
Last reviewed:
If your baby has been diagnosed with or you suspect has bladder exstrophy, here is what the evidence says.
The short answer
Bladder exstrophy is a rare birth defect (occurring in about 1 in 30,000-50,000 births, more common in boys) where the bladder is open and exposed on the outside of the abdomen rather than being enclosed inside the body. It is typically associated with epispadias (malformation of the urethra) and a widened pubic bone. Surgical repair begins in the first days of life and may require multiple staged procedures. The goal of treatment is to achieve a functional bladder with continence, good kidney function, and acceptable cosmetic appearance. Outcomes have improved significantly with experienced surgical centers.
Key takeaways
- Bladder exstrophy is a rare birth defect (occurring in about 1 in 30,000-50,000 births, more common in boys) where the bladder is open and exposed on the outside of the abdomen rather than being enclosed inside the body. It is typically associated with epispadias (malformation of the urethra) and a widened pubic bone. Surgical repair begins in the first days of life and may require multiple staged procedures. The goal of treatment is to achieve a functional bladder with continence, good kidney function, and acceptable cosmetic appearance. Outcomes have improved significantly with experienced surgical centers.
- Usually normal when: Your baby's bladder closure was successful and the wound is healing well
- Call your doctor if: Your baby develops wound separation (dehiscence) or prolapse of the bladder through the closure site - this is a surgical emergency
- Varies by age — see the age-by-age breakdown below
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What Parents Should Know
According to NIH, AAP, Children's Hospital guidelines, bladder exstrophy is a rare birth defect (occurring in about 1 in 30,000-50,000 births, more common in boys) where the bladder is open and exposed on the outside of the abdomen rather than being enclosed inside the body. It is typically associated with epispadias (malformation of the urethra) and a widened pubic bone. Surgical repair begins in the first days of life and may require multiple staged procedures. The goal of treatment is to achieve a functional bladder with continence, good kidney function, and acceptable cosmetic appearance. Outcomes have improved significantly with experienced surgical centers. At Prenatal, bladder exstrophy may be suspected on prenatal ultrasound when the bladder is not visualized in its normal position, or it may not be detected until birth. If diagnosed prenatally, you will be referred to a pediatric urology center with expertise in exstrophy repair. Delivery should be planned at or near this center. Vaginal delivery is generally safe. Connecting with the Association for the Bladder Exstrophy Community (A-BE-C) or similar organizations can provide invaluable peer support and information before your baby arrives. It is generally considered normal when your baby's bladder closure was successful and the wound is healing well. However, you should contact your pediatrician promptly if your baby develops wound separation (dehiscence) or prolapse of the bladder through the closure site - this is a surgical emergency.
Normal vs. Concerning
When to Seek Immediate Care
- Your baby develops wound separation (dehiscence) or prolapse of the bladder through the closure site - this is a surgical emergency
- Your baby has signs of UTI: fever, foul-smelling urine, irritability, or poor feeding - UTIs need prompt treatment to protect the kidneys
- Your baby has significantly decreased urine output or no urine output, which may indicate obstruction
- You notice signs of infection at the surgical site: increasing redness, swelling, warmth, drainage, or fever
- Your child has sudden inability to urinate, severe abdominal pain, or distension
By Age
What to expect by age
Prenatal
Bladder exstrophy may be suspected on prenatal ultrasound when the bladder is not visualized in its normal position, or it may not be detected until birth. If diagnosed prenatally, you will be referred to a pediatric urology center with expertise in exstrophy repair. Delivery should be planned at or near this center. Vaginal delivery is generally safe. Connecting with the Association for the Bladder Exstrophy Community (A-BE-C) or similar organizations can provide invaluable peer support and information before your baby arrives.
0-72 hours
At birth, the exposed bladder plate is visible on the lower abdomen. The bladder is kept moist with plastic wrap or moist dressings. Two main surgical approaches exist: modern staged repair of exstrophy (MSRE), where initial closure is done within the first 72 hours, or complete primary repair of exstrophy (CPRE), where the entire reconstruction is attempted in a single early operation. Initial closure involves placing the bladder back inside the pelvis and closing the abdominal wall, often with pelvic osteotomies (cuts in the pelvic bones) to bring the pubic bones together. Your baby will be cared for in the NICU.
1-6 months
After initial closure, your baby recovers in the hospital for several weeks. The legs may be immobilized temporarily to protect the pelvic repair. A catheter drains urine while the bladder heals. After discharge, your baby is monitored for bladder growth and kidney health with regular ultrasounds. Urine cultures are checked if there are signs of UTI. The goal during this phase is for the closed bladder to gradually increase in capacity. Your baby will be followed closely by the pediatric urology team.
6 months - 5 years
In the staged repair approach, additional surgeries are performed during this period. Epispadias repair is typically done around 6-12 months to reconstruct the urethra and penis (in boys) or urethra (in girls). Bladder neck reconstruction (to achieve urinary continence) is usually done later, typically when the child is 4-5 years old and developmentally ready for toilet training, and when the bladder has grown to adequate capacity. Regular follow-up monitors bladder capacity, kidney function, and overall development.
5 years+
After bladder neck reconstruction, many children achieve daytime continence, though this can take time and patience. Some children need additional procedures such as bladder augmentation (using a segment of bowel to increase bladder size) or continent diversion procedures (like a Mitrofanoff) if continence is not achieved. Clean intermittent catheterization may be needed to empty the bladder. Long-term follow-up with urology continues through adolescence and into adulthood. Sexual and reproductive function is addressed as children mature. Experienced exstrophy centers achieve the best outcomes.
What to Tell Your Pediatrician
- Describe when you first noticed has bladder exstrophy and how it has changed over time.
- Note your baby's current age and which age-specific patterns you are seeing.
- Mention if you are uncertain about the surgical plan or timing of the next procedure.
- Mention if your child is having difficulty achieving urinary continence after reconstruction.
- Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
- Bring a list of any questions or observations you want to discuss at the appointment.
What Should You Do?
When to take action
- Your baby's bladder closure was successful and the wound is healing well
- Follow-up ultrasounds show good bladder growth and normal kidney function
- Your child is progressing through the staged surgical plan as expected
- Your child is achieving continence (even if slowly) after bladder neck reconstruction
- You are being followed at a center experienced in exstrophy management
- You are uncertain about the surgical plan or timing of the next procedure
- Your child is having difficulty achieving urinary continence after reconstruction
- You have questions about your child's long-term urinary, sexual, or reproductive function
- You want to connect with other families affected by bladder exstrophy
- Your baby develops wound separation (dehiscence) or prolapse of the bladder through the closure site - this is a surgical emergency
- Your baby has signs of UTI: fever, foul-smelling urine, irritability, or poor feeding - UTIs need prompt treatment to protect the kidneys
- Your baby has significantly decreased urine output or no urine output, which may indicate obstruction
- You notice signs of infection at the surgical site: increasing redness, swelling, warmth, drainage, or fever
- Your child has sudden inability to urinate, severe abdominal pain, or distension
What You Can Do at Home
- Keep track of when you notice has bladder exstrophy — noting the time of day, duration, and any triggers can help your pediatrician.
- Remember that your baby's bladder closure was successful and the wound is healing well — this is generally within the range of normal.
- At Prenatal, focus on observation rather than intervention unless your pediatrician advises otherwise.
- Follow any care instructions from your pediatrician. Keep a written log of symptoms to bring to appointments.
- While monitoring at home, seek immediate care if your baby develops wound separation (dehiscence) or prolapse of the bladder through the closure site - this is a surgical emergency.
Related Conditions
Related Resources
Frequently asked questions
Is has bladder exstrophy normal?
When should I call the doctor about has bladder exstrophy?
When is has bladder exstrophy normal?
What causes has bladder exstrophy?
What should I mention to my pediatrician about has bladder exstrophy?
Is has bladder exstrophy normal at Prenatal?
Is has bladder exstrophy normal at 0-72 hours?
Should I go to the ER for has bladder exstrophy?
Does has bladder exstrophy go away on its own?
References
- [1]National Institutes of Health. Bladder Exstrophy. Genetic and Rare Diseases Information Center (GARD), 2024. NIH
- [2]Urology Care Foundation. Bladder Exstrophy. American Urological Association, 2024. AAP
- [3]Johns Hopkins Medicine. Bladder Exstrophy. Children's Hospital
Doctor Visit Checklist
Bring this checklist to your next pediatrician visit to discuss My Baby Has Bladder Exstrophy.
Things to mention
- Describe when you first noticed has bladder exstrophy and how it has changed over time.
- Note your baby's current age and which age-specific patterns you are seeing.
- Mention if you are uncertain about the surgical plan or timing of the next procedure.
- Mention if your child is having difficulty achieving urinary continence after reconstruction.
- Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
- Bring a list of any questions or observations you want to discuss at the appointment.
Observations to share
- You are uncertain about the surgical plan or timing of the next procedure
- Your child is having difficulty achieving urinary continence after reconstruction
- You have questions about your child's long-term urinary, sexual, or reproductive function
Urgent signs to report immediately
- Your baby develops wound separation (dehiscence) or prolapse of the bladder through the closure site - this is a surgical emergency
- Your baby has signs of UTI: fever, foul-smelling urine, irritability, or poor feeding - UTIs need prompt treatment to protect the kidneys
- Your baby has significantly decreased urine output or no urine output, which may indicate obstruction
My notes
From ismybabyalright.com — free, evidence-based baby health guides
All content follows our editorial policy and is reviewed against published clinical guidelines.
2,705 evidence-based guides6 authoritative medical sources5 medical advisory board members
Related Resources
Bottom line
Most cases of has bladder exstrophy are normal. Talk to your pediatrician if your baby develops wound separation (dehiscence) or prolapse of the bladder through the closure site - this is a surgical emergency.
Trust your instincts. If something feels wrong, reach out to your pediatrician. Worrying about your baby means you care — that is a good thing.
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How to Advocate for Your Child's Needs
You know your child better than anyone, and your observations matter. If you feel something is not right with your child's development or health, you have every right to ask questions, request evaluations, and seek second opinions. Advocating for your child is not being difficult - it is being a good parent.