Bladder Extrophy

Dr Reward E.M
Bladder Extrophy

Bladder Exstrophy:
A rare congenital anomaly characterized by an exposed bladder
mucosa through a lower abdominal wall defect, due to failure of the
anterior abdominal wall and bladder to close during embryogenesis.

Key Feature: The bladder is open and exposed on the abdominal

wall, with associated pelvic bone abnormalities and often other
genitourinary defects.

Epidemiology
A rare congenital anomaly seen in I: 50,000 births
Male: Female = 4 : 1
Bladder Extrophy

Risk Factors:
• Family history (rare, but increased risk if present).
• Associated with other congenital anomalies (e.g., epispadias, cloacal exstrophy).
• May be associated with maternal tobacco use (J Urol 2008;179:1539)

Aetiopathogenesis
• This occurs due to failure of development of lower abdominal wall and anterior wall of the
urinary bladder.
• As a result, the posterior bladder wall is seen protruding out below the umbilicus. Hence, it
is exostrophy of the bladder.
Bladder Extrophy

Risk Factors:
• Family history (rare, but increased risk if present).
• Associated with other congenital anomalies (e.g., epispadias, cloacal exstrophy).
• May be associated with maternal tobacco use (J Urol 2008;179:1539)

Aetiopathogenesis
• This occurs due to failure of development of lower abdominal wall and anterior wall of the
urinary bladder.
• As a result, the posterior bladder wall is seen protruding out below the umbilicus. Hence, it
is exostrophy of the bladder.

Types
1. Complete: Pubic symphysis is not formed, complete epispadias in male or bifid clitoris in
female.
2. Incomplete: Pubic symphysis, penis or clitoris are normal.
Bladder Extrophy

Anatomical Features:
• Bladder mucosa exposed through a midline defect
below the umbilicus.
• Pubic symphysis diastasis (widened pelvic bones).
• Epispadias (urethra opens on dorsal penis or clitoris).
• Abnormal genitalia (e.g., short penis, bifid clitoris).

Associated Anomalies:
• Epispadias (almost always present).
• Pelvic bone abnormalities -(pubic diastasis).
• Inguinal hernias -(common).
• Cloacal exstrophy -(rare, includes hindgut exposure).
• Vesicoureteral reflux (post-repair).
Bladder Extrophy

Clinical Presentation
Neonatal Period:
• Visible red bladder mucosa protruding through a midline lower abdominal defect.
• Urine leaking from exposed bladder. Constant dribbling of urine outside-therefore, they smell
of urine.
• Widened pubic bones (palpable or visible gap).
• Epispadias: Urethra opens on dorsal penis (males) or bifid clitoris (females).
• Abnormal genitalia: Short penis, splayed labia.
• Inguinal hernias (bilateral, common).

Associated Signs:
• Risk of infection or irritation of exposed bladder mucosa.
• Signs of associated anomalies (e.g., cloacal exstrophy with imperforate anus).
Bladder Extrophy

Diagnosis
Prenatal Diagnosis
• Ultrasound: Findings include absent bladder filling, low umbilical cord insertion, widened
pubic bones, and external genitalia abnormalities (as early as 15–20 weeks).
• Amniocentesis for chromosomal anomalies (rarely needed).

Postnatal Diagnosis:
• Physical Examination: Exposed bladder mucosa, epispadias, pubic diastasis.
• Imaging:
• Pelvic X-ray: Confirms pubic symphysis diastasis.
• Ultrasound: Assesses kidneys and ureters for anomalies.
• MRI/CT (if available): For complex cases (e.g., cloacal exstrophy).
Labs: Monitor for infection (e.g., CBC) or renal function (creatinine, BUN).
Bladder Extrophy

Management

Initial Management (pre-referral)

Protect Bladder Mucosa: Cover with sterile, moist saline gauze or plastic wrap to prevent
drying/irritation.
Thermoregulation: Prevent hypothermia (use warmer).
IV Fluids: Maintain hydration, especially with urine loss.
Antibiotics: Prophylactic to prevent infection of exposed mucosa.
Umbilical Cord Care: Tie cord high to avoid pressure on bladder.
Refer: To a tertiary center with pediatric urology/surgery expertise (urgent).
Bladder Extrophy

Surgical Management:

Goals: Close bladder, reconstruct urethra and genitalia, achieve urinary continence, and correct
pelvic bones.
• Staged Repair (Modern Staged Repair of Exstrophy, MSRE):
• Stage 1 (Newborn Period): Bladder closure and pubic bone approximation (osteotomy if
needed).
• Stage 2 (6–12 months): Epispadias repair (urethral reconstruction).
• Stage 3 (4–5 years): Bladder neck reconstruction for continence.
• Complete Primary Repair (CPRE): Single-stage closure in some centers (bladder, urethra, and
genitalia).
• Osteotomy: For severe pubic diastasis to aid closure.
• Temporary Measures: Urinary diversion (e.g., vesicostomy) if closure delayed.

Long-Term Management:
- Monitor for continence, vesicoureteral reflux, and renal function.
- Psychological support for body image (due to genital abnormalities).
- Urological follow-up for recurrent UTIs or incontinence.
Bladder Extrophy

Complications
Short-Term:
- Infection of exposed bladder mucosa.
- Dehydration from urine loss.
- Hypothermia.
- Bladder dehiscence (post-surgical).
Long-Term:
- Urinary incontinence (50–70% achieve continence with repair).
- Vesicoureteral reflux (VUR).
- Recurrent urinary tract infections (UTIs).
- Renal damage (from VUR or obstruction).
- Sexual dysfunction or infertility (due to genital abnormalities).
- Psychological issues (body image, stigma).
Bladder Extrophy

Complications
Short-Term:
- Infection of exposed bladder mucosa.
- Dehydration from urine loss.
- Hypothermia.
- Bladder dehiscence (post-surgical).
Long-Term:
- Urinary incontinence (50–70% achieve continence with repair).
- Vesicoureteral reflux (VUR).
- Recurrent urinary tract infections (UTIs).
- Renal damage (from VUR or obstruction).
- Sexual dysfunction or infertility (due to genital abnormalities).
- Psychological issues (body image, stigma).

Prognosis
Outcome: Good with early surgical intervention in specialized centers; continence achieved in 50–
70% with staged repair.
Bladder Extrophy

Challenges:
• Complex repairs require multiple surgeries.
• Long-term urological and psychological issues.
• Limited access to pediatric urologists in Tanzania increases risk of complications.
• Early referral to tertiary centers (e.g., Muhimbili National Hospital) is critical due to limited local
expertise.

Differential Diagnosis
• Omphalocele: Midline defect with sac, may include bladder exstrophy (e.g., OEIS complex:
Omphalocele, Exstrophy, Imperforate anus, Spinal defects).
• Cloacal Exstrophy: More severe, includes hindgut and bladder exposure.
• Patent Urachus: Urine leakage from umbilicus, no exposed bladder.
• Epispadias Alone: Urethral defect without bladder exstrophy.
Bladder Extrophy

Summary

1. Bladder exstrophy = exposed bladder mucosa, epispadias, pubic diastasis.

2. Part of exstrophy-epispadias complex; associated with inguinal hernias.
3. Prenatal ultrasound and postnatal exam are diagnostic; pelvic X-ray confirms diastasis.
4. Initial management: Protect mucosa, prevent hypothermia, refer to tertiary center.
5. Staged repair (bladder closure, epispadias repair, continence surgery) is standard.
6. Complications: Incontinence, recurrent UTIs, renal damage.
7. Tanzania Context: Early referral critical due to limited pediatric urology services.
Questions

A newborn presents with a midline lower abdominal defect exposing red bladder mucosa, urine
leakage, and a dorsally open urethra (epispadias). Pelvic X-ray shows widened pubic bones.
a) What is the most likely diagnosis?
b) What are the initial management steps?
c) Name two potential long-term complications.

A 1-day-old male infant is noted to have an exposed bladder through a lower abdominal defect, a
short penis with dorsal urethral opening, and palpable separation of pubic bones. Prenatal
ultrasound showed no other major anomalies.
a) What is the diagnosis and its key anatomical features?
b) What is the definitive treatment approach?
c) What is one critical step to take before transport to a tertiary center?
References

- NACTVET Curriculum for Clinical Officers (Pediatric Surgery Section).

- Nelson Textbook of Pediatrics (Bladder Exstrophy and Epispadias).
- WHO Guidelines on Neonatal Surgery in Low-Resource Settings.
- Tanzania MOHCDGEC Guidelines for Pediatric Surgical Emergencies
Thank you!