prune belly syndrome

1. Prune belly syndrome

2. • Triad of bilateral cryptorchidism, deficiency of the abdominal wall musculature, and a dilated, dysmorphic
urinary tract.
• William Osler is credited with giving the syndrome its name “prune belly” in 1901
• Eagle-Barrett syndrome, urethral obstruction malformation complex, the triad syndrome, abdominal muscle
deficiency syndrome, and mesenchymal dysplasia syndrome.
• Prune belly morphology is not confined to the male gender. Females constitute about 3%–5% (pseudo
prune).
• 1/29,000 to 1/40,000 live births.
• The majority are sporadic.

4. Classification

6. Theories
•Fetal Outlet Obstruction.
•Theory of Mesodermal Arrest.
•The Yolk Sac Theory.

7. Fetal outlet
obstruction

8. Theory of
Mesoderma
l Arrest

9. Clinical features

10. Extra genitourinary
manifestations

11. Abdominal wall
• Wrinkled, floppy abdominal wall, deficiency of underlying muscles, difficult
sitting, difficult walking, chronic constipation, respiratory problems.
• Ventral and lateral muscles: transversus abdominis, rectus abdominis
below the umbilicus, internal oblique, external oblique and rectus
abdominis above the umbilicus.

14. Genitourinary symptoms

15. kidney
• Renal dysplasia, hydronephrosis.
• Renal dysplasia of the prune belly syndrome is due to a
combination of a ureteric bud and metanephric defect.
• The renal parenchyma is often well preserved despite a grossly
abnormal drainage system.
• Urinary infection rather than obstruction represents the greatest
threat to the renal parenchyma.

16. Ureters
• Elongated, tortuous, and dilated.
• The lower one third of the ureter is more profoundly affected than
the proximal portion.
• VUR: up to 85% of patients
• The upper ureter is potentially best suited for definitive
reconstruction, so no ureterostomy to be done.

19. Bladder
• Thick walled and grossly enlarged, absent trabeculations.
• A patent urachus with urethral atresia or microurethra.
• Splayed trigone.
• Laterally separated UO.
• Wide BN.
• Efficient low-pressure storage and good compliance.

21. Posterior urethra
• Posterior urethra is dilated, elongated, and tapered at the membranous urethra.
• Utricular diverticulum.
• Vas deferential reflux and small or absent verumontanum.
• True obstructive lesions at the junction of the prostate and membranous urethra
have been described in 20% of infants.
• Stenosis, true valves, atresia, diaphragms, and diverticula.
• Prostatic hypoplasia is one of the etiologies of infertility in this syndrome.

22. Anterior urethra
• Urethral atresia, megalourethra, Surviving patients with urethral
atresia or microurethra have a patent urachus.
• Scaphoid and fusiform megalourethra.
• Fusiform type is the worst.

24. Testis
• Bilateral cryptorchidism
• intra-abdominal, overlying the ectatic ureters at the pelvic inlet.
• Risk of malignancy: 30–50 times.
• The gubernaculum is normally attached proximally to the tail of the epididymis,
travels via the inguinal canal, and attaches distally at the pubic tubercle.
• Vas deferens obstruction and epididymal abnormalities associated with maldescent
affect sperm delivery and maturation.

25. Management

26. Prenatal evaluation
• As early as 11 weeks of gestation on ultrasound.
• Difficult to distinguish from other causes of obstructive uropathy like
PUV.
• In utero intervention ????

27. Neonatal evaluation
• Abdominal wall appearance is diagnostic.
• Cardiac and pulmonary evaluation is a must.
• X - ray to rule out pneumothorax and pneumomediastinum.
• 48-72 hours serum creatinine, if >1 in term infant or >1.5 in preterm
>>>> indicate renal insufficiency.
• Starting chemoprophylaxis.

28. Investigations
• Serum electrolytes.
• serum creatinine.
• Urine analysis and culture.
• Renal and bladder sonogram.
• VCUG.
• MAG 3 renogram vs DMSA.
• MRU.

29. Initial management
• Watchful waiting to immediate surgical reconstruction of the urinary tract.
• Early surgical intervention in the neonate is avoided, unless a rising creatinine or infection
occurs, requiring early vesicostomy.
• Category I: oligohydramnios, pulmonary hypoplasia, usually die , if passes need for high
urinary diversion like ureterostomy, pyelostomy.
• Category II: if urinary tract infection, vesicostomy, urinary tract reconstruction, orchiopexy,
abdominoplasty is preferred before the age of 1 year.
• Category III: chemoprophylaxis, usually no need for urinary reconstruction, urodynamics,
early orchiopexy and abdominoplasty improves the voiding and defection mechanism.

30. In utero intervention
• Vesicoamniotic shunting in patients with suspected bladder outlet
obstruction does not improve renal outcomes but would assist in
pulmonary maturation and function.

31. Anesthetic consideration
• Careful preoperative pulmonary assessment.
• An antecedent history of recurrent respiratory infections warrants
aggressive physiotherapy, postural drainage, and intermittent positive
pressure breathing treatments.
• Specific antibiotic therapy.
• Postoperative analgesics.

32. Vesicourethral dysfunction
• Internal urethrotomy: trans urethral incision
• Assessment of lower urinary tract dynamics, routine urinary flow
rates, and the amount of residual urine.
• If urinary tract infection or deteriorating renal function in the
perinatal period, a cutaneous vesicostomy is the drainage procedure
of choice.

33. Reduction cystoplasty
• Bladder volume: 3 L.
• Removal of a dilated urachal diverticulum.
• Some authors do not recommend reduction cystoplasty in light of the
effective and safe application of clean intermittent catheterization
except for the removal of a urachal pseudodiverticulum.

36. Anterior urethra
• Micro urethra: need for regular dilatation and hydrodistension.
• Megalourethra: hypospadias repair.

37. Upper urinary tract
• VUR 85%.
• Vesicoureteral obstruction.
• Ureteral valve.
• Decreasing renal function, urinary tract infection.
• Reduction and reimplantation.

38. Orchiopexy
• Open or laparoscopic.
• Early before the age of 1 year.
• Microvascular testicular auto transplantation, The spermatic vessels
are anastomosed to the inferior epigastric vessels, with microsurgical
vascular techniques.

39. Abdominal wall reconstruction
• Improve voiding, defecation and sensation of bladder fullness.
• Preservation of the lateral and upper parts of the abdominal wall, the sites of most normal
musculature.
• Ehrlich and Monfort techniques: Abdominal wall reconstruction, utilizing an ellipsoid midline
incision with extensive subcutaneous dissection, and a pants over vest closure.
• This technique preserves the umbilicus, uses the full thickness of abdominal wall, and
provides narrowing at the waist.
• Randolph technique.

45. Comprehensive reconstruction
• Reduction cystoplasty, resection of the distal ureter and bilateral
ureteral tapered reimplantation.
• Combined with the performance of the abdominoplasty and bilateral
orchiopexy, before the age of 2 years.
• Antibiotic prophylaxis.

49. Renal transplantation
• Bilateral nephroureterectomies.
• Urodynamic assessment of the lower urinary tract is recommended to
insure absence of obstruction and balanced voiding.
• The use of CIC to empty the decompensated bladder is not a
contraindication to renal transplantation.
• Antibiotic prophylaxis.
• Allograft torsion is an unusual complication, graft loss, was a result of
lack of abdominal wall tone, Nephropexy is recommended.