MITROFANOFF

The Mitrofanoff principle offered a major advancement to continent urinary reconstruction

for children and young adults. Earlier innovations—including clean intermittent
catheterization (CIC), bladder augmentation, and a variety of bladder neck tightening
procedures—made continent bladder reconstruction possible. The technically difficult
problem was creating a bladder outlet that was tight enough to ensure continence but wide
enough to allow reliable catheterization over a lifetime. The concept of a continent
catheterizable abdominal channel was introduced by Paul Mitrofanoff in 1980 (11) and has
since been adopted as the ideal alternate urinary continence mechanism in most pediatric
centers worldwide. This principle involves creation of a flap valve continence mechanism
for a conduit that is tunneled into a low-pressure urinary reservoir that can then be
catheterized and emptied via an abdominal stoma (Fig. 112-1).
FIG. 112-1. Umbilical Mitrofanoff stoma allowing catheterization in the sitting or standing
position.
There are multiple surgical options for creating the Mitrofanoff channel.
Appendicovesicostomy has by tradition been used due to the reliable blood supply,
adequate lumen for catheterization, and supple muscular wall. Long-term follow-up has
shown that appendicovesicostomy provides a durable channel with minimal late
complications (8,9). In the absence of a suitable appendix, or in conditions where the
appendix is used for an alternate procedure [such as for a Malone antegrade continence
enema (MACE channel)], there are multiple options that have been described. The most
reliable alternatives have been the Monti–Yang ileovesicostomy, ureterovesicostomy,
and continent bladder tube.
DIAGNOSIS
The Mitrofanoff procedure can be performed with essentially any underlying bladder
pathology. The most frequent diagnosis for children undergoing the procedure is
neuropathic bladder, usually due to myelomeningocele. This procedure has also been
described for reconstruction in patients with exstrophy-epispadias, cloacal anomalies, prune
belly syndrome, posterior urethral valves, and other conditions. Although many of these
children will require a bladder outlet procedure to provide urinary continence,
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some will have an intact and continent bladder outlet and the Mitrofanoff channel will
provide an alternate to urethral catheterization. This is especially useful in wheelchair-
bound patients who cannot access their perineum independently and also in patients with
normal urethral sensation where catheterization can be traumatic both physically and
psychologically.
INDICATIONS FOR SURGERY
In the past, the primary indication for bladder reconstruction was for upper urinary tract
preservation. In the era of aggressive use of anticholinergics and intermittent catheterization
in young patients, the more common indication for a Mitrofanoff channel is for urinary
continence and convenient, independent bladder management for the patient. All patients
should undergo a trial of CIC to demonstrate that they are reliable and able to comply with
a daily routine prior to bladder reconstruction.
ALTERNATIVE THERAPY
The most common alternative to continent bladder reconstruction with a Mitrofanoff stoma
is anticholinergic therapy with clean intermittent urethral catheterization. With careful
attention to catheterization schedules and fluid intake, social dryness can be achieved in
many patients with neuropathic bladder and other underlying bladder pathology.
Less frequently used alternatives are long-term incontinent vesicostomy and conduit
urinary diversion. Although these are both considered suboptimal in the era of continent
urinary reconstruction, there will be a subset of patients who are unable to care for
themselves because of physical, mental, or psychosocial problems, and the incontinent
diversion provides a safer long-term option for these patients.
In the rare patient with a completely nonusable bladder, a continent urinary reservoir with a
continent catheterizable channel is another alternative.
SURGICAL TECHNIQUE
All patients are admitted the day before surgery for intravenous antibiotics to sterilize the
urinary tract and for a mechanical and antibiotic bowel preparation. This is in particular
important for patients with ventriculoperitoneal shunts, who have a risk of shunt infection.
Potential sites for stomal location should be determined preoperatively, with the patient in
the sitting and supine position.
Surgical exposure is usually obtained through a midline transabdominal incision that is
carried around the umbilicus to leave enough fascia to close the abdomen without
compromising an umbilical stoma. A lower transverse Pfannenstiel incision will also allow
adequate exposure for both bladder augmentation and the Mitrofanoff stoma in thin
patients. Laparoscopic mobilization of the right colon and isolation of the appendix has
been described, which allows a smaller lower-abdominal incision without the concern of
inadequate exposure.
Appendicovesicostomy
The right colon is mobilized beyond the hepatic flexure to allow maximal freedom of the
appendiceal mesentery. If the appendix is retrocecal in location it is mobilized carefully
from the cecal attachments with extra caution to avoid injuring the appendiceal artery,
which is a branch of the ileocolic artery (Fig. 112-2). In some
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cases there is significant inflammation due to the presence of a ventriculoperitoneal shunt,

and the peritoneal incision must be carried medially to adequately mobilize the appendix.
When the length of appendix is inadequate, it can be extended by incorporating a segment
of distal cecum as described by Cromie et al. (5). Prior to detaching the appendix, the
bladder is mobilized to ensure that the bladder and appendix can easily reach the chosen
site for the abdominal stoma without tension. The bladder is then opened to the left of the
midline for a right lower-quadrant appendicovesicostomy or in a wide U-shaped anterior
bladder incision for an umbilical stoma. The appendix is detached from the cecum either
sharply or with a stapling device, and the cecum is closed with absorbable and permanent
sutures. The mesentery to the appendiceal artery can be freed from the cecal mesentery to
allow complete mobilization of the appendix if needed (Fig. 112-3). The terminal end of the
appendix is then opened, and a 12 Fr catheter is passed to ensure that the appendix has an
adequate lumen. If necessary the appendix can be gently dilated with serial sounds. If
bladder augmentation is to be performed, the segment of bowel is isolated, harvested, and
reconfigured appropriately. If an additional segment of intestine is required for the
Mitrofanoff (e.g., Monti–Yang) channel it can be harvested simultaneously. The site of
the bladder hiatus is then selected, again ensuring that it can easily reach the posterior
abdominal wall fascia without tension. The site of the hiatus is opened wide enough to
allow the appendix to pass without any tension and a vessel loop is passed through the
hiatus for traction. A submucosal bladder tunnel is then created using sharp dissection.
Placing several traction sutures on the bladder to flatten out the posterior bladder wall
facilitates this dissection. The orientation of the tunnel should be directed away from the
bladder outlet and trigone to prevent painful catheterization postoperatively. The tunnel
length should be at least 2.5 cm in length. It is sometimes helpful to inject 1:200,000
epinephrine along the path of the submucosal tunnel to facilitate the dissection and
minimize bleeding. The terminal end of the appendix is then passed through the bladder
hiatus and submucosal tunnel. The appendix is spatulated and secured distally with two 4-0
absorbable sutures incorporating full-thickness bites of the appendix and detrusor muscle
and mucosa. The remainder of the anastomosis is completed using 4-0 or 5-0 absorbable
sutures, securing the bladder mucosa to the appendix. The appendix is also secured at the
level of the bladder hiatus using several 4-0 absorbable sutures. The channel is catheterized
with a 12 or 14 Fr catheter to ensure that it passes easily across the hiatus and submucosal
tunnel. The stomal site is then selected, taking care to ensure that the bladder hiatus can
reach the posterior fascia without tension. A U-shaped (umbilical) or V-shaped skin
incision is made at the stomal site, and the flap is freed sharply to the level of the fascia. A
cruciate incision is made in the fascia and widened to allow passage of an index finger. The
appendix is then brought through the fascial opening and the appendiceal/bladder hiatus is
secured to the posterior fascial wall using 3-0 absorbable sutures, taking care to not
angulate or compress the appendiceal mesentery (Fig. 112-4). This maneuver ensures a
short, straight extravesical appendix channel. The cecal end of the appendix is then
spatulated on the antimesenteric side, and if there is redundant appendix it is amputated.
The stomal anastomosis is secured using interrupted 4-0 absorbable sutures (Fig. 112-5).
The stoma is then catheterized multiple times with the bladder both distended and empty to
ensure that there is no angulation in the channel. A 12 Fr catheter is left indwelling for 3
weeks before initiating intermittent catheterization