Prune belly syndrome
Key points
AKA Eagle Barrett syndrome
95% of patients are male
Possible autosomal dominant/X-linked heredity
Pathophysiology
Urinarfy tract obstruction + mesodermal defect in week 6-10
Triad of:
• Ectasia of urinary tract
• Bilateral undescended testes
• Absent abdominal wall musculature
Other abnormalities:
Generalised prostatic hypoplasia
Patent urachus - 25-30%
Very large smooth walled bladders with pseudodiverticulum at urachus
Potter sequence (Oligohydramnios causing pulmonary hypoplasia, clubbed feet, cranial abnormalities)
Associated with:
Malrotation
Cardiac anomalies
Spinal anomalies
GI atresias
Trisomy 13, 18, 21
Categories:
1. Severe Pulmonary hypoplasia - 100% mortality in first few days
2. Classic features
3. Mild features
Outcomes
Retrograde ejaculation
30% progress to renal failure
Abdominal wall reconstruction
Advocates for abdominoplasty for persistent prune belly suggest it can improve coughing, constipation, bladder emptying
UK practice is to not reconstruct the abdominal wall during childhood
Example is the Montfort technique:
Elliptical midline incision - preserve umbilicus
Mobilise muscle and fascia - lateral fasciotomies - bring to midline
Standard scenario
Neonate with PBS
Concern is for renal tract obstruction and renal function
Ensure resuscitation - treatment for pulmonary hypoplasia
Pass non-balloon catheter and ensure drainage
Prophylactic antibiotics
Antenatal history:
Potters sequence - oligohydramnios
BL HUN, echogenic kidneys
Cardiovascular abnormalities
Examination:
Confirm diagnosis -
Abdominal wall
Palpate kidneys
BL impalpable UDT
System by system examination
Chromosomal anomalies
Spine + anus
Categorise:
1. Severe Pulmonary hypoplasia - 100% mortality in first few days - palliate
2. Classic features
3. Mild features
Investigations:
Echo
USS KUB + MCUG with antibiotic cover at 48h
Delayed film if VUR to check for obstructing refluxing megaureter
Define level of urinary tract obstruction
Management:
Prophylactic antibiotics
Manage urinary tract obstruction in neonatal period:
PUV - ablate
BL VUJO - JJ stents (nephrostomy backup) + serial U+E until creatinine stable + repeat USS in 3 months - Kaefer procedures if worsening dilatation after stent removal
BL PUJO - JJ stents (nephrostomy backup) + serial U+E until creatinine stable + repeat USS in 3 months - remove stents
Serial creatinine until stable
1st stage Orchidopexies at 1 year
UK practice is not to reconstruct abdominal wall unless necessary as adults
References
Snider Novitski, Katie, et al. "Prune Belly Syndrome." Pediatric Surgery NaT, American Pediatric Surgical Association, 2023. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829694/all/Prune Belly Syndrome.
Essentials of Pediatric Urology, 3rd edition, 2022, Chapter 24 Adolescent Urology