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Paediatric / developmentalPediatric Urology

Bladder Exstrophy

Bladder exstrophy is a rare midline closure defect where the bladder is open on the lower abdominal wall (part of the exstrophy–epispadias complex) — it requires staged or complete primary surgical reconstruction in specialist centres, aiming for secure abdominal/bladder closure, continence and acceptable genital reconstruction.

Defect
open bladder, midline
+
Spectrum
exstrophy–epispadias complex
+
Care
specialist centre
Orientation

The big picture

Bladder exstrophy is a midline ventral defect in which the bladder fails to close and is everted onto the lower abdominal wall, with associated epispadias, a widened pubic symphysis (pelvic diastasis), and abnormal pelvic/abdominal-wall anatomy. It sits on a spectrum — the exstrophy–epispadias complex — ranging from isolated epispadias through classic bladder exstrophy to the most severe cloacal exstrophy (with bowel involvement). It is obvious at birth.

Golden rule

Bladder exstrophy is a midline exstrophy–epispadias-complex defect needing specialist staged/complete reconstruction — protect the bladder plate, refer to a specialist centre, and aim for closure, continence and genital reconstruction.

Presentation

Symptom sorter

Recognise it.

Open bladder everted on the lower abdominal wallEpispadiasWidened pubic symphysis (pelvic diastasis)Abnormal abdominal-wall/pelvic anatomy
Work-up

Diagnostic algorithm

Each step answers one question. Tap to expand.

Management

Treatment ladder

Specialist, staged or complete reconstruction aiming for secure closure, continence, acceptable genitalia and renal protection.

1
Recognise + protect plate
2
Specialist referral
3
Closure (± osteotomy)
4
Epispadias/bladder-neck reconstruction for continence
5
Lifelong surveillance (continence, renal, malignancy risk)
Safety

Complications

Disease complications
  • Incontinence, renal impairment, sexual/fertility and psychological issues, long-term bladder malignancy risk
Treatment complications
  • Closure failure/dehiscence, complications of osteotomy and reconstruction
How to prevent
  • Specialist, high-volume care; secure closure; surveillance
How to manage
  • Redo/continence surgery; lifelong follow-up
Surveillance

Follow-up

What to monitor
  • Continence, renal function and upper tracts
  • Bladder capacity/function
  • Long-term bladder malignancy risk; genital/cosmetic and psychological outcomes
Timing
  • Lifelong specialist follow-up
Success looks like
  • Secure closure, acceptable continence, protected kidneys, acceptable genitalia
Failure looks like
  • Failed closure, incontinence, renal deterioration
When to image
  • Surveillance and on clinical change
Long-term issues
  • Incontinence needing further surgery, renal issues, malignancy risk, sexual/fertility and psychological considerations
Recall

Memory hooks

Open bladder on the abdominal wall = exstrophy.

Part of the exstrophy–epispadias complex.

Protect the plate; refer to a specialist centre.

Goals: closure, continence, genital reconstruction.

Lifelong surveillance (malignancy risk).

Exam

Board traps

Exstrophy is part of the exstrophy–epispadias complex.

Protect the plate and refer to a specialist centre.

Reconstruction goals: closure, continence, genital reconstruction.

Apply

Clinical cases

Case 1

A newborn is born with the bladder mucosa visible and everted on the lower abdominal wall, with epispadias and a widened pubic symphysis.

What is the diagnosis and the immediate priority?

Test yourself

Quiz

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