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Bladder exstrophy:

current management
and postoperative imaging

(Ketsia Pierre & Joseph Borer & Andrew Phelps & Jeanne S. Chow)
20 January 2014

Journal Reading
Oleh
dr.Mahendra

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 23 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 followup 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
reconstruction

Immediately following exstrophy repair, the


typical findings are 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.

Thank You