Prune Belly Syndrome

PRUNE BELLY SYNDROME
What is prune-belly syndrome?

Prune-Belly syndrome, also known as Eagle-Barrett syndrome, is a rare
disorder characterized by partial or complete absence of the stomach
(abdominal) muscles, failure of both testes to descend into the scrotum
(bilateral cryptorchidism), and/or urinary tract malformations.
Other names: 1. triad syndrome, 2. Eagle-Barrett syndrome, 3.abdominal musculation

Syndrome 4.Obrinsky syndrome, 5. Frohlich syndrome
INTRODUCTION
A constellation of anomalies with variable degrees of severity.

 Three characteristic findings:
1. a deficiency of abdominal musculature,
2. bilateral intra-abdominal testes, and
3. an anomalous urinary tract.
 Single most important determinant of long-term survival severity of the urinary tract
anomaly, in particular, degree of renal dysplasia
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
Symptoms
 Poorly developed and/or absent abdominal muscles
 Undescended testicles in males (cryptorchidism)
 Urinary tract problems such enlarged or blocked ureters (tubes that carry urine from
the kidneys to the bladder)
 Enlarged bladder
 Enlarged kidney (hydronephrosis)
CLINICAL FEATURES
Kidneys:
 Spectrum of anomalies range from normal renal parenchyma to
dysplasia.
 Dysplasia in 50%.
 Severe degree of renal collecting system dilatation- characteristic.
 Calyceal morphology well-preserved.
CLINICAL FEATURES
 Severely dysplastic or dilated kidney.
 Non-obstructive hydronephrosis.
 Primary or secondary UPJO (ureteropelvic junction obstruction)
Kidneys
CLINICAL FEATURES
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
Bladder
CLINICAL FEATURES
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.

CLINICAL FEATURES
 Poorly attached epididymis to testis.
 Abdominal undescended testis(Most common).
 Lack of continuity b/w efferent ductules & rete testis.
 Retrograde ejaculation
Prostate & accessory sex organs
CLINICAL FEATURES
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.
Anterior Urethra
CLINICAL FEATURES
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.
Nonurologic-Associated Abnormalities:
• Most commonly cardiac,
pulmonary, & orthopedic
defect(75%)
• Abdominal wall defect(Mostly
inferior & medial segments
affected)
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
EVALUATION AND MANAGEMENT
Initial Management:
1. Chest radiograph to evaluate for pulmonary condition
2. Baseline assessment of renal function
3. Voiding cystourethrogram (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
(mercapto acetyl tri glycine) & DMSA (dimercaptosuccinic acid)
respectively
 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
Surgical Management
Urinary tract:
1.Cutaneous vesicostomy / PCN
2.Internal Urethrotomy
3.Reduction cystoplasty
4. Anterior Urethral reconstruction
Surgical Management
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

Prune Belly Syndrome

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PRUNE BELLY SYNDROME

What is prune-belly syndrome?

Other names: 1. triad syndrome, 2. Eagle-Barrett syndrome, 3.abdominal musculation

A constellation of anomalies with variable degrees of severity.

 Unknown cause: Most common sporadic.

 Abdominal wall reconstruction

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