BLADDER EXSTROPHY:

CURRENT MANAGEMENT
AND POSTOPERATIVE IMAGING
(

ABSTRACT
Bladder exstrophy is a rare malformation
Incomplete closure of the bladder with mucosa continuous with
the abdominal wall, epispadias, and alterations in the pelvic
bones and muscles.
More common in boys

EMBRYOLOGY
The most popular theory explaining the exstrophyepispadias
defect describes an overgrowth of the cloacal membrane
that prevents medial migration of the mesenchymal tissue.

ANATOMICAL DEFECTS
Children with bladder exstrophy have an everted
bladder,epispadias, wide diastasis of the pubic symphysis,
and pelvic muscular defects that result in anterior
displacement of the anus and occasionally rectal prolapse.

The defects result in an open book configuration of the

pelvis in which the pubic bone is 30% deficient and the pelvic
bones are externally rotated, resulting in an average diastasis
of the pubic symphysis of 4 cm (Fig. 4)

The pelvic floor anatomy is also altered. The puborectal sling is

flatter (compared to its more normal conical shape) and wider with
divergence of the levator ani muscles. Compared to the normally
centered position of the rectum within the levator muscles, the
rectum is positioned in the anterior third of the pelvic floor (Fig. 5)

The ureters insert low into the bladder and have a J-shape
configuration because of the enlarged pouch of Douglas, which
forces the distal ureters downward and laterally. These children
invariably have vesicoureteral reflux following exstrophy closure
unless ureteral reimplantation is performed (Fig. 6)

INITIAL SURGICAL REPAIR OF BLADDER EXSTROPHY

Two main approaches to bladder exstrophy repair:
Modern staged repair of exstrophy (MSRE)
Complete primary repair of exstrophy (CPRE).

MODERN STAGED REPAIR OF BLADDER EXSTROPHY (MSRE)

Stage I, performed within 4872 h after birth, the bladder and the abdominal
wall defect are closed in both boys and girls, with concomitant epispadias
repair in girls only. Iliac osteotomies can be performed at this time.
Stage II, closure of the urethra in boys (epispadias repair), is typically
performed when children are 612 months of age.
Stage III (boys and girls) consists of bladder neck reconstruction, typically
with bilateral ureteral reimplantation, and is performed when the child
can participate in a voiding program, most commonly 45 years of age

STAGE I

STAGE II

STAGE III

COMPLETE PRIMARY REPAIR OF EXSTROPHY (CPRE)

The objective of CPRE is to combine bladder closure and formal
epispadias repair at the initial reconstruction.
The CPRE approach has traditionally been performed within 72
h after birth; however there has been a recent shift in this
practice by some surgeons, with repair now delayed until 2
3 months of age.

ILIAC OSTEOTOMY

Several types of pelvic osteotomies have been developed. The first

described was the bilateral posterior iliac osteotomy.
Pelvic osteotomies reduce pubic diastasis, which has many benefits.
In some cases iliac osteotomy is not performed, typically in infants
younger than 72 h with pubic diastasis less than 4 cm

AUGMENTATION CYSTOPLASTY
Augmentation cystoplasty can be considered in children who are not
appropriate candidates for bladder neck reconstruction on the basis of
poor bladder capacity or poor bladder compliance, and in those who fail to
achieve continence or safebladder storage characteristics following
bladder neck reconstruction. Augmentation cystoplasty can be combined
with a catheterizable conduit.

CATHETERIZABLE CONDUITS
Mitrofanof
Appendicovesicostomy
YangMonti ileovesicostomy
ileovesicostomy

HISTORICAL PROCEDURES FOR BLADDER EXSTROPHY TREATMENT

Ureterosigmoidostomy was the first method of diversion used
for patients with bladder exstrophy.
The ureters are divided at or below the common iliac arteries
and each ureter is reimplanted into the teniae coli of the
sigmoid colon using an antireflux technique
This method has fallen out of favor because of complications
including stone formation and increased risk of malignancy.

CLINICAL OUTCOMES FOLLOWING EXSTROPHY REPAIR

Continence, defined as dry intervals of 3 or more hours, is one
of the primary goals of repair.
Clinical outcomes in children following exstrophy repair vary
across institutions depending on a number of factors
including institution volume, surgeon experience, type of
repair and definition of continence, making accurate
comparison of clinical outcomes in these children
challenging.

IMAGING THE RECONSTRUCTED URINARY TRACT

Because so few children are born with bladder
exstrophy,guidelines for preoperative and postoperative
imaging are not standardized.
After the child is born, preoperative imaging can include a
radiograph of the kidneys, ureters and bladder to assess the
diastasis of the pubic symphysis and renal US examination
to assess the kidneys,

After primary repair, voiding cystourethrograms (VCUGs)

assess for reflux and complications of surgery.
Ultrasonography is useful immediately after surgery and at
6-month and 1-year follow-up examinations to monitor for
significant upper tract dilatation.

MRI is useful for further definition of anatomy both

preoperatively and postoperatively, and it is used
experimentally in measuring pelvic angles to predict
continence.
Urodynamic testing at 3 years of age is useful for assessing
bladder capacity and compliance. If there is concern for
bladder perforation, US can first detect free fluid and then
the defect is best visualized by CT or conventional
cystograms.

FLUOROSCOPY
VCUG is the mainstay of post-operative evaluation following
bladder exstrophy repair. VCUG allows the assessment and
surveillance of bladder capacity, the evaluation of
complications and, in conjunction with a retrograde
urethrogram, the evaluation of urethral integrity and
patency. The grade of vesicoureteral reflux can also be
assessed.

OSSEOUS STRUCTURES

NORMAL VCUG FINDINGS

FOLLOWING BLADDER CLOSURE
AND BLADDER NECK
Immediately following exstrophy repair, the typical findings are
RECONSTRUCTION
a small-capacity bladder with an irregular contour and
vesicoureteral reflux into J-shape ureters.
Over time the bladder contour may become smooth.
The bladder capacity is typically smaller than age-predicted
capacity.

ULTRASOUND
Kidneys
Bladder
Augmented bladder

MRI
MRI is not commonly used to image postoperative
complications of bladder exstrophy repair. Complications
secondary to iliac osteotomy rarely occur. MRI, as stated in
previous sections, is utilized primarily in a research capacity
to evaluate pelvic anatomy changes following repair.

IMAGING OF COMPLICATIONS FOLLOWING BLADDER EXSTROPHY REPAIR

Fluoroscopy
Urethrocutaneous fistula
Urethral stricture
Bladder rupture/perforation
Complications of bladder augmentation
Ultrasound
Renal scarring
Stone formation

MISCELLANEOUS COMPLICATIONS
Bladder malignancy

Bladder malignancy, most commonly

adenocarcinoma, in children with bladder
exstrophy occurs rarely in the present day of
early primary repair. Squamous cell carcinoma
is the second most common pathology and is
thought to occur on a background of
squamous metaplasia.

CONCLUSION
Bladder exstrophy is a rare congenital abnormality with a
complex surgical repair regardless of the approach.
Imaging plays a key role in the follow-up of these patients.
Therefore an understanding of the surgical techniques and the
norma postoperative appearances of bladder exstrophy
repair and familiarity with the imaging appearance of
complications are essential for the pediatric radiologist and
pediatric urologist.

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