Prune Belly

(Eagle Barrett) Syndrome

and the Monfort Abdominoplasty

Liam Macleod
R1 General Surgery/Urology
April 2012

PBS: Epidemiology

1 in 29,000 to 40,000 live births

95% male, 5% female
Twins*, young mothers, black race
Turners, monosomy 16, trisomy 13,
trisomy 18

*twins are discordant, theory that defect due to uneven distribution of mesenchymal tissue
during development, there are also genetic theories.

Embryologic Theories:
Early in utero urethral obstruction
GU tract dilation,
oligohydramnios/ascites
Global defect in embryologic tissue
(lateral plate mesoderm, early GU
tract or yolk sac)

Clinical Features
Triad: deficient abdominal wall
musculature, dilated GU tracts, intraabdominal testes
Older
child
with rib
deformity
from
chronic
abdomina
l
wall laxity
Classic abdominal wall
appearance
(images taken from Campbell-Walsh Urology

T. Lendvay

GU anomalies most affected

system
Ureteral: dilation,
tortuosity, UPJ
obstruction, VUR

Prostatic:
hypoplasti
c
Urethral: megalourethra*, dilated
prostatic urethra, urethral
valves/membranes, urethral

Renal:
dysplasia

Cystic: UVJ obstruction,

urachal
pseudodiverticulum,
patent urachus, cystic
redundancy
Testicular: bilaterally
intra-abdominal

Extragenitourinary Anomalies
(present in 75%)
Cardiac (10%): PDA, VSD, ASD, TOF

Extragenitourinary Anomalies
(present in 75%)
Cardiac (10%): PDA, VSD, ASD, TOF
Pulmonary (55%): hypoplasia, PTX,
pneumomediastinum

Extragenitourinary Anomalies
(present in 75%)
Cardiac (10%): PDA, VSD, ASD, TOF
Pulmonary (55%): hypoplasia, PTX,
pneumomediastinum
Ortho(30-45%): pectus deformities,
sacral agenesis, club foot (talipes
equinovarus)

Extragenitourinary Anomalies
(present in 75%)
Cardiac (10%): PDA, VSD, ASD, TOF
Pulmonary (55%): hypoplasia, PTX,
pneumomediastinum
Ortho(30-45%): pectus deformities,
sacral agenesis, club foot (talipes
equinovarus)
GI (30%): atresias/malrotations,
biliary anomalies, gastroschisis,
splenic torsion

Extragenitourinary Anomalies
(present in 75%)
Cardiac (10%): PDA, VSD, ASD, TOF
Pulmonary (55%): hypoplasia, PTX,
pneumomediastinum
Ortho(30-45%): pectus deformities, sacral
agenesis, club foot (talipes equinovarus)
GI (30%): atresias/malrotations, biliary
anomalies, gastroschisis
Abdominal wall muscular defect from
which prune belly name derived

Early Management*
1st treat/support cardiac/respiratory issues
(48% require vent)
Evaluate renal function (4% will have renal
failure)
Screen for associated anomalies
Early intervention such as SPT for BOO as
well as circ and abx for UTI ppx
If patients die initial CV/pulm
complications or renal failure 2/2 obstruction
+/- infection
*%s from a 133 pt case series at Boston Childrens (Routh, 2010)

Surgical Planning

Urinary diversion/reconstruction
Abdominal wall reconstruction
Orchidopexy within first 6-12 months
Adjunct procedures based on other
findings

Abdominoplasty
Indications:
-moderate to severe laxity
-cosmetic/psychological concerns
-improvement in bowel, bladder,
pulmonary function controversial

Abdominoplasty:
Timing:
-only semi-urgent operation is
orchidopexy
-divert urine early, but operate when
child can tolerate anesthesia
-can do plasty with other procedures, but
possibly trend towards delaying to age
4-5y

Abdominoplasty:
Techniques: based on component
separation and resection of
redundant skin, fascial fixation
-Randolph (1981)
-Ehrlich (1986)
-Monfort* and modifications
(1991)

Defining
redundant
skin

Monfort:

Outer
incision

Periumbilic
al
incision

Excision
of skin

Peri-umbilical skin
preserved

Lateral border of rectus opened

Campbell-Walsh Urology, 10

Exposure of
retroperitoenum
for concomitant
upper GU tract
revision

EO sutured
to rectus
after being
pulled
medially to
overlap

Campbell-Walsh Urology, 10th

Monfort:
Scoring of
parietal
peritoneum
deep to
transversus m.

Dermis/epidermis
closed
Rectus attached
(laterally/deep) to
transversalis fascia
and lateral
abdominal muscle
layers
overlap/attached
medially on rectus

Befor
e

After

Campbell-Walsh Urology, 10th

Before

After

Campbell-Walsh Urology, 10th

P. Merguerian

P. Merguerian

P. Merguerian

P. Merguerian

P. Merguerian

P. Merguerian

P. Merguerian

P. Merguerian

T. Lendvay

Monfort Revisions:
Creation of neo-umbilicus (UNC,
2005)
Extra-peritoneal (Northwestern,
1998)
Lap assisted (Mt. Sinai, 2007)

Long Term Prognosis:

Based on damage to GU system/kidneys
Best if Cr nadir <0.7 and pyelonephritis
avoided^
Some will require renal transplant
Secondary sexual development normal
Fertility requires ICSI in all cases
29% inpatient mortality, 56% of these in
premature infants*
Monfort long term follow up lacking
^ Woodard, 2003
*Routh, 2010 Boston Childrens series but are relatively representative

Excretory Urogram
Calyces: preserved

Ureters:
dilated,
tortuous,
displaced

Campbell-Walsh Urology, 10th

Retrograde flow of urine

Urachal
diverticulum

Dilate
d

Atresia of anterior urethra

Campbell-Walsh Urology, 10th

Cryptorchidism, Megalourethra and

Hypospadias

Campbell-Walsh
Urology, 10th ED.

Rest of abd
cavity

head

Massively
dilated bladder

kidneys
Bladder
reaches
kidneys, note
hydronephrosi
s

Dilated
ureter

Urachal diverticulu

Hydronephroti
c kidneys
Bladder dilated

Dilated
prostatic
urethra
due to
urethral
atresia

Verumontanum

Cystoscopic
view of
anterior
urethral valve

T. Lendvay

References:
1. Bukowski TP, Smith CA: Monfort abdominoplasty with
neoumbilical modification. J Urol 2000, 164(5):1711-1713.
2. Monfort G, Guys JM, Bocciardi A, Coquet M, Chevallier D: A novel
technique for reconstruction of the abdominal wall in the
prune belly syndrome. J Urol 1991, 146(2 ( Pt 2)):639-640.
3. Routh JC, Huang L, Retik AB, Nelson CP: Contemporary
epidemiology and characterization of newborn males with
prune belly syndrome. Urology 2010, 76(1):44-48.
4. Furness PD, 3rd, Cheng EY, Franco I, Firlit CF: The prune-belly
syndrome: a new and simplified technique of abdominal wall
reconstruction. J Urol 1998, 160(3 Pt 2):1195-1197; discussion 1216.
5. Levine E, Taub PJ, Franco I: Laparoscopic-assisted abdominal wall
reconstruction in prune-belly syndrome. Ann Plast Surg 2007,
58(2):162-165.
6. Woodard JR: Prune-belly syndrome: a personal learning
experience. BJU Int 2003, 92 Suppl 1:10-11.
7. Parrott TS, Woodard JR: The Monfort operation for abdominal wall
reconstruction in the prune belly syndrome. J Urol 1992, 148(2
Pt 2):688-690.
8. Wein A, Kavoussi, LR, Campbell, MF, Walsh, PC (ed.): CampbellWalsh Urology. Philadelphia, PA: Elsevier Saunders; 2012.