PRUNE BELLY SYNDROME

Background

 The syndrome is named for the mass of

wrinkled skin that is often (but not always)
present on the abdomens of those with the
disorder.
 Other names for the syndrome include
Abdominal Muscle Deficiency Syndrome,
Congenital Absence of the Abdominal Muscles,
Eagle-Barrett Syndrome, Obrinsky Syndrome,
Fröhlich Syndrome, or rarely, Triad Syndrome.
Background
 Prune belly syndrome is a group of birth
defects that involve three main problems:

1. A partial or complete lack of abdominal muscles.

There may be wrinkly folds of skin covering the
abdomen.
2. Undescended testicles in males (cryptorchidism)
Crytorchidism
3. Urinary tract
abnormality such as
unusually large ureters,
distended bladder,
accumulation and
backflow of urine from
the bladder to the
ureters and the kidneys
Background
 Prune belly syndrome is an uncommon birth
defect occurring in about one in 40,000 births.
 In 95 percent of the cases, it occurs in males.
 It may occur if there is a urethral obstruction during
fetal development preventing any urine from
flowing through the urinary tract. The urethra is the
tube that drains urine from the bladder to the
outside of the body for elimination. If there is
obstruction, urine can reverse flow and cause an
expanded bladder
 Prune belly syndrome is associated
with trisomy 18 and 21. Patients with prune
belly syndrome also have an increased
incidence of tetralogy of Fallot (TF) and
ventriculoseptal defects.
Diagnosis: Prenatal

 A woman who is pregnant with a baby who

has prune belly syndrome may not have
enough amniotic fluid (oligohydramnios). This
can cause the infant to have lung problems.
 An ultrasound done during pregnancy may
show that the baby has a swollen bladder or
enlarged kidney.
The abdomen of an infant with prune belly syndrome shows marked
distention of the abdomen and bulging flanks secondary to a large urinary
system and the absence of abdominal wall musculature.
Diagnosis: Postnatal

 Weak abdominal muscles can cause:

 "Little Buddha" appearance
 Constipation
 Delay in sitting and walking
 Difficulties coughing
 Urinary tract problems can cause difficulty
urinating.
Diagnostic/Laboratory Tests

 Renal ultrasound - a diagnostic imaging technique that uses

high-frequency sound waves and a computer to create images
of blood vessels, tissues, and organs. Ultrasounds are used to
view internal organs as they function, and to assess blood flow
through various vessels.

 Voiding cystourethrogram (VCUG) - a specific x-ray that

examines the urinary tract. A catheter (hollow tube) is placed in
the urethra (tube that drains urine from the bladder to the
outside of the body) and the bladder is filled with a liquid dye. X-
ray images will be taken as the bladder fills and empties. The
images will show if there is any reverse flow of urine into the
ureters and kidneys.
 Intravenous pyelogram (IVP) - a diagnostic
imaging technique which uses an x-ray to
view the structures of the urinary tract. An
intravenous contrast of dye is given so that
the structures can be seen on film. An IVP
also reveals the rate and path of urine flow
through the urinary tract.
 Blood tests (to determine how well the
kidneys may be functioning).
Prognosis
 Prune belly syndrome is a serious and often
life-threatening problem.
 Many infants with prune belly syndrome are
either stillborn or die within the first few
weeks of life from severe lung or kidney
problems, or a combination of birth
problems.
 Some newborns survive but continue to have
problems.
Treatment: Medical Therapy

Antibiotics
o Surgical intervention is not warranted in these
patients as long as they remain free of infection.
Once infections start occurring frequently, the
reflux should be corrected.
Management of the undescended testis with
human chorionic gonadotropin (hCG) is not
warranted in these cases.
Treatment: Surgical therapy

 Undescended testis
 Orchiopexy
 is a surgery to move an undescended testicle into
the scrotum and permanently fix it there. It is
performed by a pediatric urologist or surgeon on
boys withcryptorchidism, typically before they reach
the age of two. Some patients remain undiagnosed
until their teenage years and undergo the surgery at
that time.
 Abdominal wall reconstruction
 Abdominal wall reconstruction is performed in
most patients with prune belly syndrome to
improve respiratory function and to improve
cosmesis.
 innovations in abdominal wall reconstruction have
been developed. The Monfort and
Ehrlich variations of the Randolph operation have
improved results, with decreased morbidity and
the ability to preserve the umbilicus.
Placement of a percutaneous
nephrostomy
 Obstruction at the ureteropelvic junction (UPJ) has
been observed in patients with prune belly
syndrome. In some cases, diagnosing this can be
difficult; however, the diagnosis can be confirmed
with the placement of a
percutaneous nephrostomy. This procedure can be
performed under ultrasound guidance with relative
ease in a dilated system and provides the
opportunity to perform a renal biopsy, which could
help with the later management of the urinary
tract.
 Placement of a percutaneous nephrostomy
provides the surgeon with several options.
The surgeon can confirm the diagnosis of UPJ
obstruction. The urinary tract can be
decompressed in an ill child or in a child who
is too small to safely undergo reconstructive
surgery at the time. Finally, the technique
also allows the surgeon to evaluate the renal
function of the obstructed unit
Standard pyeloplasty

 If conditions are right and the child is stable, a

standard pyeloplasty can be performed as
another means of treating the obstructed
UPJ. The surgeon should be meticulous in the
dissection of the upper ureter. The proximal
ureteral blood supply should be preserved as
much as possible in case ureteral tapering or
reimplantation becomes necessary later.
 Cutaneous pyelostomy still carries the risk of resultant scarring
from excessive dissection, which may be required to take down
the pyelostomy at a later stage.
 At the time of reconstruction, ureteral tapering or
reimplantation that is necessary creates a difficult situation.
Recently, these problems have been overcome with the
increasing use of percutaneous nephrostomy drainage and with
the use of vesicostomy as a means of draining the upper tracts.
 Some believe that vesicostomy can decompress the upper
tracts just as well as high diversion. Subsequently, since the
introduction of these 2 procedures, the use of high diversion
has declined significantly over the last few years, and
reconstruction in these patients has become easier.
Nephrostomy
Infravesical obstruction or
obstruction at the prostatic
urethra
 Blocksom vesicostomy performed in the early
newborn period is the simplest and best
treatment in patients with documented
obstruction. Bringing the dome of the bladder
out to the skin is essential when creating the
vesicostomy; this approach prevents the
resultant herniation of the bladder in an
improperly created vesicostomy.
 Herniation of the bladder is quite prevalent in
patients with prune belly syndrome because the
bladder is quite large and redundant. A patent
urachus can be found frequently in patients with
urethral obstruction. The patent urachus is the
means by which patients are able to survive; early
deaths usually are observed in patients with
urethral obstruction without a patent urachus. In
these patients, once a vesicostomy is performed,
the surgeon should try to identify the urachus and
ligate it at that time.
Preoperative

 Preoperatively, all patients should have stable

respiration. This is critical because postoperative
respiratory infections pose a serious risk to these
patients.
 The urine should be sterile prior to surgery.
 In patients undergoing abdominal wall
reconstruction, the presence of a peg gastrostomy
precludes the safe reconstruction of the abdominal
wall. The author prefers to not have a gastrostomy
in place before abdominal wall reconstruction.
Postoperative Details

 Postoperatively, these patients must be

monitored closely. They can easily become
fatigued and may experience respiratory
arrest. Judicious use of narcotics is critical,
and the use of ketorolac (Toradol) is
recommended for postoperative pain control.
The author has also used postoperative
epidural anesthesia, with excellent results.
Complications

 Undescended testis
 The most obvious complication of orchidopexy is
incomplete descent of the testis. Atrophy of the
testis is another possible complication. This can
occur in some cases (as many as 50%) of Fowler-
Stephens orchidopexy. For this reason, the author
does not suggest performing simultaneous
Fowler-Stephens orchidopexies.
 Abdominal wall reconstruction
 After reconstruction, persistent laxity of the
abdominal wall is a concern, regardless of the
technique used. Using the laparoscope to perform
the abdominal wall plication significantly reduces
the risk of this complication.
 The abdominal wall skin can slough when the skin
flaps are raised. Care should be taken to avoid
incisions that cut the blood supply to the flaps.
 Ureteral reimplants
 Ureteral strictures are not uncommon in tapered
reimplants of ureters in patients with prune belly
syndrome. This can be avoided using Kaliscinski-
type tapering of the ureters.