BJUI Surgery Illustrated – Surgical AtlasPHILLIPS and GEARHART

PHILLIPS and GEARHART

Surgery Illustrated – Surgical Atlas

BJUI BJU INTERNATIONAL
Primary closure of bladder exstrophy
Timothy M. Phillips and John P. Gearhart
Paediatric Urology, Brady Urological Institute, Johns Hopkins Hospital, Baltimore, MD, USA

ILLUSTRATIONS by STEPHAN SPITZER, [Link]

PLANNING AND PREPARATION

a

Before undertaking closure of primary

exstrophy the surgeon must decide whether a a

formal osteotomy will be required, and if so,

ensure that there is appropriate orthopaedic b
b

support for this. Also, the anaesthesia staff,

nursing staff and parents need to be prepared
to support a prolonged admission while the
child is supine and in traction after surgery.

Supplies include:

• Interfragmentary pins, external fixator, and

orthopaedic instruments (osteotomy, if
required);
• Supplies for Buck’s or Bryant’s traction (as
needed);
• 3- and 5-F paediatric feeding tubes
(ureteric stents);
• 10 F Malecot drain (suprapubic tube);
• 2–0 silk ties (umbilical vessels);
• 3–0 and 5–0 polydioxanone sutures
(bladder and urethral closure);
• 3–0 nylon, 4–0 chromic, 3–0 polyglactin
(secure drains);
• No. 2 nylon sutures (intrapubic stitch);
• Paediatric Van Buren sounds.

INDICATIONS

Whilst other methods of treatment of the

newborn with bladder exstrophy have been
offered, we routinely use a modern staged
reconstruction of bladder exstrophy. This
includes: bladder and abdominal wall closure,
and urethral closure onto the penis, in the
newborn period, with bilateral innominate
and vertical iliac osteotomy, if indicated
(Stage 1); epispadias repair at 0.5–1 year old

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TABLE 1 The initial presentation and management of exstrophy of the bladder

Age Problem Possible solution

Initial presentation
0–72 h Classic exstrophy with reasonable capacity and moderate I: Midline closure of bladder, fascia, and symphysis to level of posterior
symphyseal separation; long urethral groove; mild dorsal urethra; no osteotomy. In very selected cases combined bladder
chordee closure and epispadias repair
0–72 h Above findings with short urethra and severe dorsal chordee II: Close as in I, adding lengthening of dorsal urethral groove by para-
exstrophy skin (cautiously)
0–72 h Above findings with very wide separation of symphysis or late Osteotomy (combined anterior and vertical iliac) and closure as in I or II
presentation (>72 h, up to 1–3 years) for initial treatment
0–2 weeks Very small, non-distensible bladder patch Prove by examination under anaesthesia, then non-operative expectant
treatment awaiting internal or external diversion or delayed closure if
bladder plate grows

(Stage 2); and bladder neck reconstruction capacity of ≥5 mL, and has elasticity and should be removed, the bladder surface
along with antireflux procedure at age 4–5 contractility, can be expected to develop to a irrigated with sterile saline, and clean plastic
years, when the child has achieved an useful size and capacity after successful wrap or dressing placed over the bladder
adequate bladder capacity for bladder neck bladder, posterior urethra and abdominal wall surface area. The surgeon should ensure that
reconstruction and is motivated to participate closure with early epispadias repair. Table 1 any serious coexisting medical problems
in a postoperative voiding programme (Stage provides a guideline for managing bladder that would prevent bladder closure and
3). Primary bladder, abdominal wall, and closure according to variations in initial subsequent immobilization are stabilized
proximal urethral closure are outlined here presentation. before taking the child to the operating room.
(Stage 1). Other methods of primary bladder Before and during the procedure, the patient
exstrophy closure, such as the complete is given broad-spectrum antibiotics. The
primary repair of exstrophy or Kelly repair can SPECIFIC PATIENT PREPARATION procedure is performed under a combined
be used, but the basic surgical principles of general and tunnelled caudal epidural
closure are similar. At birth, although the bladder mucosa is anaesthetic technique.
usually smooth, pink and intact, it is also
sensitive and easily denuded. In the delivery
PATIENT SELECTION room the umbilical cord should be tied with SPECIFIC PATIENT POSITIONING
2–0 silk close to the abdominal wall, so that
The size and the functional capacity of the the umbilical clamp does not traumatise the For the entire procedure, including anterior
detrusor muscle are important considerations delicate mucosa and cause excoriation of the osteotomy (if indicated) the patient is placed
for the eventual success of functional closure. bladder surface. The bladder can then be supine with the body circumferentially
Sometimes a good portion of previously covered with a non-adherent film of plastic prepared, draped and exposed from the
unappreciated bladder can be discovered wrap (e.g. Saran wrap) or a hydrated gel nipples distally. A small towel is placed
behind the fascia, under examination with dressing, to prevent sticking of the bladder beneath the sacrum and the bladder is
anaesthesia. The exstrophied bladder that is mucosa to clothing or diapers. In addition, covered with soft absorbent gauze during the
estimated at the time of birth to have a each time the diaper is changed the dressing time of osteotomy (if indicated).

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SURGICAL STEPS

Figure 1

Children undergoing closure after 72 h of life,

those with very wide separation of the
symphysis (>4 cm), or those with a pelvis that
is not malleable, will typically undergo a
bilateral anterior transverse innominate and
vertical iliac osteotomy, performed by
orthopaedic colleagues. At our institution,
this is done by the orthopaedic team before
bladder closure. Interfragmentary pins are
placed as shown.

After soft tissue closure and completion of

the urological procedure, an external fixator is
placed to buttress the pelvis in the corrected
position. Modified Buck’s traction is applied
after surgery. Osteotomy together with
posterior urethral, bladder and abdominal
wall closure is a 5–7 h procedure in these
infants.

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Figure 2

A strip of mucosa 2 cm wide, extending

from the distal trigone to below the
verumontanum in the male (a) and to the
vaginal orifice in the female (b), is outlined
and incised for prostatic and posterior
urethral reconstruction in the male and
adequate urethral closure in the female. The
male urethral groove might be adequate, in
which case no transverse incision of the
urethral plate is needed for urethral
lengthening. We tend not to incise the
urethral plate unless the length of the
urethral groove from the verumontanum to
the urethral glans is so short that it interferes
with eventual penile length and reduces
dorsal angulation. If so, the urethral groove is a
lengthened using a modified Duckett para-
exstrophy skin-flap technique (see below).

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Figure 3

The bladder plate is then dissected off the

anterior abdominal wall as shown, with an
incision extending from just above the
umbilicus, down the junction of the bladder
and para-exstrophy skin, to the level of the
urethral plate.

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Figure 4

An appropriate plane is entered just above the

umbilicus, and a plane is established between
the rectus fascia and the bladder. The
umbilical vessels are doubly ligated (3–0
polyglactin) and incised and allowed to fall
into the pelvis. The peritoneum is taken off
the dome of the bladder at this point so that
the bladder can be placed deep into the pelvis
at the time of closure. The plane is continued
caudally down between the bladder and
rectus fascia until the urogenital diaphragm
fibres are encountered bilaterally.

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Figure 5

The pubis is encountered with continued

caudal dissection. Gentle traction on the glans
at this point shows the insertion of the
corporal body on the lateral inferior aspect of
the pubis. The penis is lengthened by exposing
the corpora cavernosa bilaterally and freeing
the corpora from their attachments to the
suspensory ligaments on the anterior part of
the inferior pubic rami.

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Figure 6

A double-pronged skin hook can be inserted

into the bone at this time and pulled laterally
to accentuate the urogenital diaphragm
fibres, and to help the surgeon radically incise
these fibres between the bladder neck,
posterior urethra and pubic bone. The
visualized urogenital diaphragm fibres are
taken down sharply with electrocautery to
the levator hiatus in their entirety. If this
manoeuvre is inadequate the posterior
urethra and bladder will not be placed deeply
into the pelvis, and when the pubic bones are
brought together, the posterior vesico-
urethral unit will be brought anteriorly
into an unsatisfactory position for later
reconstruction. Ureteric catheters (3–5 F) are
placed bilaterally, as well as a 10 F Malecot
catheter being placed through the dome of
the bladder for suprapubic drainage. The
ureteric catheters will be passed through the
anterior bladder wall at the time of closure.
The ureteric catheters are secured directly to
the ureteric orifice with 4–0 chromic sutures.
The suprapubic tube is secured to the outside
of the bladder with two sutures of 3–0
polyglactin, then at the skin with two sutures
of 3–0 nylon. The ureteric stents are steri-
stripped to the suprapubic tube at the skin.
The corporal bodies are not brought together
at this juncture, because later Cantwell-
Ransley epispadias repair will require the
urethral plate to be brought beneath the
corporal bodies. If the urethral plate is left in
continuity, it must be mobilized up to the level
of the prostate to create as much additional
urethral and penile length as possible. Further
urethral lengthening can be done at the
time of epispadias repair. The wide band of
fibres and muscular tissue representing
the urogenital diaphragm is detached
subperiosteally from the pubis bilaterally.
Reluctance to free the bladder neck and
urethra from the inferior ramus of the pubis
moves the neobladder opening cephalad
should any separation of the pubis occur
during healing, increasing the chance of
bladder prolapse.

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Figure 7

If the decision is made at this point that the

urethral groove is to be transected, caudal
glabrous para-exstrophy skin at the
inferolateral borders of the bladder template
are outlined and incised (Fig. 7a). A V-incision
is made transversely through the urethral
plate 1 cm distal to the verumontanum, and
full-thickness para-exstrophy skin flaps are
mobilized (Fig. 7b).

The corporeal bodies are further freed from

the pubic bone by incising the suspensory
ligaments (Fig. 7c). Again, complete and
radical transection of the urogenital fibres
is a critical step to allow placement of the
posterior vesico-urethral unit deep into the a
pelvis (Fig. 7d).

Dissection is carried out cephalad and medial

behind the prostate and lower aspect of the
bladder (Fig. 7e). The para-exstrophy skin flaps
are sutured to the lateral aspect of the
prostatic urethra (5–0 polydioxanone). They
extend to the inferior border of the bladder
to effectively widen the plate used for
tubularization (Fig. 7f).

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Figure 8

The mucosa and muscle of the bladder are

closed in single layer with interrupted 3–0
polydioxanone figure-of-eight sutures.
Starting at the vesico-urethral junction, the
urethra is then closed well onto the penis in
the midline anteriorly with full thickness,
interrupted 5–0 polydioxanone. The urethra
should accommodate a 10–12 F sound
comfortably. The size of the opening should
allow enough resistance to aid in bladder
adaptation and to prevent prolapse, but not
enough outlet resistance to cause upper tract
changes. The posterior urethra and bladder
neck are buttressed with a second layer of
local tissue if possible. The urethra is not
stented, to avoid necrosis with accumulation
of secretions in the neourethra.

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Figure 9

When the bladder and urethra have been

closed and the drainage tubes placed,
pressure over the greater trochanters
bilaterally allows the pubic bones to be
approximated in the midline. Horizontal
mattress sutures are placed in the pubis and
tied with a knot away from the neourethra
(Fig. 9a). Often, we are able to use another
stitch of no. 2 nylon at the most caudal
insertion of the rectus fascia onto the pubic
bone. This manoeuvre adds to the security of
the pubic closure. The neourethra can then be a
matured to the surrounding lower abdominal
and penile skin with interrupted 5–0
polyglactin. A V-shaped flap of abdominal
skin at a point corresponding to the normal
position of the umbilicus is tacked down to
the abdominal fascia with 5–0 polyglactin,
and the drainage tubes exit this orifice
(Fig. 9b).

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POSTOPERATIVE CARE ensure adequate bony healing and callus achieve appropriate postoperative voiding
formation before the external fixator is dynamics. They are taught to relax the pelvic
The modern staged reconstruction of bladder removed and the child allowed to bear weight floor and use the pelvic floor muscles
exstrophy converts a patient with exstrophy and mobilize. If the pubic diastasis is not properly. This is accomplished by a team
into one with incontinent proximal shaft completely corrected initially, it can be of senior nursing staff using one-to-one
epispadias. In males, the second stage gradually corrected at the bedside in coaching and modern biofeedback
epispadias repair is at 0.5–1 year old, with succeeding days by gradual approximation of equipment.
bladder neck reconstruction and ureteric the inferior fixator bars. These fixation devices
reimplantation at age 4–5 years, when the are removed at the bedside or clinic using a FROM SURGEON TO SURGEON
child has achieved an adequate bladder mild sedative at 6 weeks after surgery in
capacity (>100 mL) and is motivated to children who are ≥2 years old. Children aged The difficult case: Small bladders that are of
participate in a postoperative voiding <2 years might have the devices removed as questionable size for closure are a special
programme. early as 4 weeks after surgery in a similar challenge. For example, a small, fibrotic
manner. bladder patch that is stretched between the
Ensure all catheters are well secured and a edges of the small triangular fascial defect
sterile surgical dressing of choice is used. Newborns undergoing closure without without elasticity or contractility cannot be
osteotomy in the first 48–72 h of life are selected for the usual closure procedure.
Adequate postoperative pain control immobilized in modified Bryant’s traction Examination with the patient under
is essential in maintaining pelvic while supine, where the hips have 90° of anaesthesia might sometimes be required to
immobilization. Epidural analgesia is flexion. When modified Bryant’s traction is assess the bladder adequately, particularly if
maintained with a continuous infusion of used, it is maintained for 4 weeks. Spica casts considerable oedema, excoriation, and polyp
1 mg/mL lidocaine at 0.8 mg/kg/h. Epidural and ‘mummy wraps’ are all inadequate for formation have developed between birth and
infusion rates are proactively adjusted to proper immobilization and are associated the time of assessment. Decisions on the
maintain serum lidocaine levels at 5 mg/L. It is with an increased failure rate. suitability of bladder closure, or the need for
desirable to maintain epidural catheters for waiting (delayed primary closure) should be
≥3 weeks. If analgesia remains inadequate, CATHETER/STENT HANDLING made only by surgeons with extensive
catheters are removed and i.v. or oral opioids experience of the exstrophy condition.
are administered. In addition to epidural The bladder is drained by a suprapubic non- Some conditions preclude primary closure,
analgesia, infants receive enteral latex Malecot catheter for 4 weeks. Before including penoscrotal duplication, ectopic
acetaminophen 10–15 mg/kg every 4–6 h removing the suprapubic catheter the bladder bowel within the extruded bladder (a relative
around the clock and i.v. or enteral diazepam outlet is calibrated with a urethral catheter or contraindication), a hypoplastic bladder, and
0.1 mg/kg, every 4–6 h, as needed. Diazepam sound, to ensure free drainage. Also, residual significant bilateral hydronephrosis. Ideally,
also assists with controlling bladder spasms. urine is estimated by clamping the suprapubic waiting for the bladder template to grow for
All children who receive benzodiazepines tube, and specimens for culture are obtained 4–6 months in the child with a small bladder
and/or opioids for >2 weeks are considered before the patient leaves the hospital and is not as risky as submitting a small bladder
drug-dependent and are, at the conclusion of before tube removal. Should bladder outlet template to closure in an inappropriate
their hospital stay, weaned slowly (10–20% of resistance be such that urine is retained setting, resulting in dehiscence and allowing
their total daily dose per day) to prevent within the bladder, then reflux and ureteric the fate of the bladder to be sealed at that
symptoms of withdrawal. dilation could develop, with a subsequent risk point. If the bladder does not grow to
of urinary infection. In these cases, it might be sufficient size after 4–6 months, other options
All children are given oral oxybutynin necessary to dilate/incise the urethra or begin include excision of the bladder and
(0.1–0.2 mg/kg) three times daily until all intermittent catheterization. Ureteric stents a nonrefluxing colon conduit or
catheters are removed. Also, appropriate provide drainage during the first 10–14 days ureterosigmoidostomy. Another alternative
continuous prophylactic antibiotics should be after closure, as swelling caused by the involves urinary diversion with a colon
continued, as all children with bladder pressure of closure of a small bladder conduit and placing the small bladder inside,
exstrophy have VUR. can obstruct the ureters and give rise to to be used later for the posterior urethra in an
obstruction and transient hypotension. If Arap-type procedure. Lastly, if the bladder is
there are no problems with the stents during small and the presentation is for late primary
PATIENT INSTRUCTION (ACTIVATION/ healing, we leave them in for up to 3–4 weeks. closure, bladder augmentation, ureteric
IMMOBILIZATION) A complete ultrasonography examination is reimplantation, and an outlet procedure, in
obtained to ascertain the status of the renal addition to a continent urinary stoma, can be
All patients who receive pelvic osteotomy are pelves and ureters after catheter removal. considered.
immobilized after surgery, using modified
Buck’s traction and external fixation. The ADJUVANT THERAPY THINGS TO MAKE LIFE EASIER
patient is placed in minimal skin traction in a
nearly horizontal position for ≈4 weeks for While there is no specific adjuvant therapy in We have found that having a paediatric
infants and 6–8 weeks for older children, neonatal exstrophy closure, all older children orthopaedic surgeon with an interest in pelvic
depending on patient age and amount of undergo a structured voiding-improvement osteotomy and the orthopaedic care of
healing. Pelvic radiographs are taken to course before bladder neck reconstruction, to children with bladder exstrophy is essential to

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successful closure. Along those lines, the been placing a ≈2 × 4-cm patch of human The single most important aspect when
tunnelled epidural catheter, as described acellular dermis (HAD, Alloderm, LifeCell encountering intraoperative problems is
previously, has proved crucial in ensuring Corporation, Branchburg, NJ, USA) between having previous experience with closure of a
long-term postoperative pain control and the posterior urethral/bladder neck and the wide variety of exstrophy variants.
immobilization. Performing osteotomy on a pubic closure to avoid these potential
child without a clear plan for immobilization complications. The HAD is secured to the pelvic Correspondence: Timothy M. Phillips,
is doomed to failure. floor and bladder with interrupted 4–0 Paediatric Urology, Brady Urological Institute,
polyglactin before pubic bone apposition. The Johns Hopkins Hospital, 600 N. Wolfe Street,
Also, in an effort to decrease the incidence of pubic bone is then closed over the HAD adjunct Baltimore, MD 21287, USA.
vesicocutaneous fistulae and intrapubic and urethral/bladder neck closures. The long- e-mail: tphill24@[Link]
suture erosion after surgery, we have recently term results of this technique are pending.

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