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Paediatric / developmentalPediatric Urology

Prune Belly Syndrome

Prune belly (Eagle–Barrett) syndrome is the triad of deficient abdominal-wall musculature, bilateral undescended testes, and a dilated urinary tract — management centres on protecting renal function and treating infection, with surgery individualised rather than reflexive.

Triad
wall · undescended testes · dilated tract
+
Tract
usually non-obstructive
+
Priority
renal preservation
Orientation

The big picture

Prune belly syndrome (Eagle–Barrett syndrome), almost exclusively in males, is defined by a classic triad: deficient/absent anterior abdominal-wall muscles (giving the wrinkled 'prune' appearance), bilateral undescended (intra-abdominal) testes, and a dilated urinary tract (large hypotonic bladder, dilated tortuous ureters, often with reflux). Severity ranges widely; the most severe have renal dysplasia and, if associated with oligohydramnios, pulmonary hypoplasia that dominates the neonatal course.

Golden rule

Prune belly = abdominal-wall deficiency + bilateral undescended testes + dilated (usually non-obstructive) urinary tract — manage function-first (renal preservation, infection control), individualising surgery rather than reflexively correcting dilation.

Presentation

Symptom sorter

Recognise it.

Deficient/absent anterior abdominal-wall muscles ('prune' appearance)Bilateral undescended (intra-abdominal) testesDilated urinary tract (large bladder, tortuous ureters, often reflux)Almost exclusively male
Work-up

Diagnostic algorithm

Each step answers one question. Tap to expand.

Management

Treatment ladder

Function-led care: protect renal function, control infection and ensure emptying, with individualised surgery rather than reflexive correction of dilation.

1
Recognise + assess function/pulmonary status
2
Function-first (renal/infection/emptying)
3
Orchidopexy + abdominoplasty (individualised)
4
Selective urinary reconstruction for genuine obstruction/severe reflux
5
Lifelong renal and cancer-awareness follow-up
Safety

Complications

Disease complications
  • Chronic kidney disease/renal failure, recurrent UTIs, infertility and testicular cancer risk, respiratory issues (pulmonary hypoplasia)
Treatment complications
  • Surgical complications of reconstruction/orchidopexy/abdominoplasty
How to prevent
  • Function-first management, infection control, judicious surgery
How to manage
  • Treat genuine obstruction/reflux; renal support; lifelong follow-up
Surveillance

Follow-up

What to monitor
  • Renal function and upper tracts
  • UTIs and bladder emptying
  • Testicular position (cancer awareness), abdominal-wall function
Timing
  • Lifelong surveillance, especially renal
Success looks like
  • Preserved renal function, controlled infections, adequate emptying
Failure looks like
  • Renal deterioration, recurrent UTIs, genuine obstruction
When to image
  • Surveillance and on deterioration
Long-term issues
  • Chronic kidney disease, infertility/cancer risk (undescended testes), abdominal-wall and respiratory issues
Recall

Memory hooks

Triad: prune wall + undescended testes + dilated tract.

Dilation is usually non-obstructive.

Function-first: kidneys, infection, emptying.

Surgery individualised, not reflexive.

Almost always male.

Exam

Board traps

Prune belly dilation is usually non-obstructive.

Triad: abdominal-wall deficiency, bilateral undescended testes, dilated urinary tract.

Pulmonary hypoplasia (oligohydramnios) can dominate the neonatal course.

Apply

Clinical cases

Case 1

A male neonate has a lax, wrinkled abdominal wall, impalpable testes, and markedly dilated ureters and bladder on ultrasound. A renogram shows no significant obstruction.

What is the diagnosis and the management principle?

Test yourself

Quiz

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