Prune belly syndrome is rare disease of child

1. PRUNE BELLY SYNDROME

2. Other names: 1. triad syndrome, 2. Eagle-Barrett syndrome, 3.abdominal musculation
Syndrome 4.Obrinsky syndrome, 5. Frohlich syndrome

4. Etiology
 Unknown cause: Most common sporadic.
 Genetics: Turner syndrome, Monosomy 16, Trisomy 13, Trisomy 18.
 Embryological abnormality:
 Early in utero posterior urethral obstruction resulting in severe dilation of urinary
tract and possible fetal ascites and oligohydramnios
 Primary defect in the lateral plate of mesoderm, which is the precursor of the
ureters, bladder, prostate, urethra.
 Intrinsic defect of the urinary tract leading to ureteral dilation and fetal ascites
 Yolk sac defect

5. Clinical Features

6. Kidney:
 Renal dysplasia most common.
 Mainly potter type II & IV varieties.
 Hydroureteronephrosis
 Calyceal morphology may be well preserved.
 Vesicoureteral reflux(75 %)
 Poor peristalsis

7. Bladder:
 Massively enlarged.
 Pseudodiverticulum at the urachus.
 Patent urachus(25-30%).
 Increased collagen to muscle fiber ratio.
 Trigone displaced laterally & superiorly.
 In UDS: Large capacity bladder, Normal compliance, Significant post void volume,

8. Prostate & accessory sex organs:
 Prostatic hypoplasia Dilated prostatic urethra.
 Obstructive lesions in distal posterior urethra(Urethral
atresia, Valves, Urethral stenosis, Urethral membrane &
urethral diverticulum)
 Angulation of urethra(Type IV Valves)
 Vas deferens & seminal vesical atresia, Dilated, or
thickened.
 Poorly attached epididymis to testis.
 Abdominal undescended testis(Most common).
 Lack of continuity b/w efferent ductules & rete testis.
 Retrograde ejaculation

9. Anterior Urethra:
 Urethral atresia & Megalourethra(Most common).
 Two variation in megalourethra: 1. Fusiform type 2.
Scaphoid type.
 In fusiform type is a deficiency of corpus cavernosum
& corpus spongiosum with entire phallus dilatation
during voiding.
 In scaphoid type is a deficiency of corpus
spongiosum with ventral urethra dilatation during
voiding.

10. Testes:
 Bilateral intra-abdominal testes lying over the iliac vessels and adjacent to the
dilated ureters are the most typical findings.
 No difference in germ cell counts, Ad spermatogonia, and Leydig cells between
PBS testes and non-PBS intra-abdominal testes.

11. Nonurologic-Associated Abnormalities:
 Most commonly cardiac, pulmonary, & orthopedic defect(75%)
 Abdominal wall defect(Mostly inferior & medial segments affected).

12. Spectrum Of Disease:
 CATEGORY CHARACTERISTICS
I Renal dysplasia
Oligohydramnios
Pulmonary hypoplasia
Potter features
Urethral atresia
II Full triad features
Minimal or unilateral renal dysplasia
No pulmonary hypoplasia
May progress to renal failure
III Incomplete or mild triad features
Mild to moderate uropathy
No renal dysplasia
Stable renal function
No pulmonary hypoplasia

13. EVALUATION AND MANAGEMENT
 Initial Management:
1. Chest radiograph to evaluate for pulmonary condition
2. Baseline assessment of renal function
3. Voiding cyst urethrogram (VCUG) is indicated in the neonatal period if there is
renal insufficiency or evidence of bladder outlet obstruction
4. Circumcision is advisable in the absence of a structural penile abnormality
5. Early intervention is indicated for evidence of bladder outlet obstruction and
preferably with a percutaneous suprapubic tube.
6.Renal function & structure in neonate best assessed with MAG3 & DMSA
respectively.

14. EVALUATION AND MANAGEMENT
 Category I PBS patients: Supportive care.
 Category III patients: Rarely require urologic intervention for the urinary tract
because minimal abnormality present.
 Category II patients: Require individualization of evaluation and management on
the basis of the fact

15. Surgical Management
 Urinary tract:
1.Cutaneous vesicostomy / PCN
2.Internal Urethrotomy
3.Reduction cystoplasty
4. Anterior Urethral reconstruction
 Orchidopexy:
1.Transabdominal better option(67% success rate)
2.Age of operation- 6 months
3.Fowler stephens orchidopexy(77% success rate)
4.Miocrovascular autotransplantation
 Abdominal wall reconstruction

16. For Megalourethra:
A, The prepuce is reduced,
B, The penis is degloved along the subdartos plane.
C, The involved segment of the urethra is opened
longitudinally,
D,The redundant urethral wall is excised
E, The urethra is closed
F, The penile skin is brought forward,

17. Abdominal wall reconstruction
 Benefits : 1. Cosmetics
2.Improves bladder, bowel, & pulmonary function.
 Age of operation: After 6 months of age
 Techniques: 1.Randolph techniques
2. Ehrlich Technique
3.Monfort Technique(Best cosmetics & function results)

18. Monfort Technique
A, Delineation of redundancy by tenting up abdominal
wall.
B, Elliptically skin incision
C, Skin (epidermis and dermis only) is excised with
electrocautery.
D, Abdominal wall central plate is incised at the lateral
border of the rectus muscle on either side, from the
superior epigastric to the inferior epigastric vessels,
creating a central musculofascial plate.
E, Adequate exposure is provided for concomitant
transperitoneal genitourinary procedures.