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Fig. 16.18 Umbilical fl ap for
appendicostomy
20116 Gastrointestinal Stomas in Infants and Children
Fig. 16.20 Catheterized umbilical fl ap anastomosis in appendicostomy
Fig. 16.19 Laparoscopic port placement for appendicostomy
There are now a number of palliative surgical options for
Hirschsprung’s disease, most of which can be performed
without a protective colostomy in selected patients.
Historically, however, the initial approach to a child suspected
of having HD was to confi rm the diagnosis by performing a
transanal rectal wall biopsy, and then to perform a “leveling”
202 D.K. Magnuson and O.S. Soldes
Fig. 16.21 Appearance of colon in typical Hirschsprung’s disease
colostomy for temporary decompression. The modifi er
“leveling” was added to stress the importance of defi ning the
zone of transition between normal ganglionic bowel and distal aganglionic bowel, and to perform a colostomy above that
“level.” Currently, leveling colostomies are still indicated in
two groups of patients: those who present with acute stasis
enterocolitis with signifi cant physiologic compromise, and
those with a delayed diagnosis in whom the proximal colon is
chronically dilated and thick-walled – unsuitable for a pullthrough procedure in its present state and in need of a longer
period of decompression to regain a normal caliber.
Thus, a leveling colostomy is the combination of serial
seromuscular colon wall biopsies for frozen section analysis,
proceeding proximally from the peritoneal refl ection, and the
formation of a colostomy in the confi rmed ganglionic bowel
(Fig. 16.22 ). The transition zone is often characterized by a
funnel-like reduction in caliber from proximal to distal.
Although ganglion cells may be present histologically within
this tapered segment, residual neural hypertrophy often persists and muscular relaxation is impaired. The “level” of
aganglionosis is not circumferentially even; the leading edge
of ganglion cell loss occurs around the bowel circumference
in an undulating fashion over a length of several centimeters,
resulting in adjacent zones that differ with respect to innervation. Therefore, the colostomy should be created at a level at
least several centimeters proximal to the site of the most distal ganglionated biopsy specimen. The most dilated and
hypertrophied segment of ganglionic bowel may be discarded as long as the remaining bowel will be of suffi cient
length to reach the anus. The transition zone occurs in the
rectosigmoid region in roughly 75% of cases, in the more
proximal colon in about 15% (“Long-segment HD”), at the
ileocecal junction in about 10% (“Total-colon HD”), and
rarely in the more proximal intestinal tract.
When the diagnosis is known from prior rectal biopsy
and the typical recto-sigmoid transition zone is suspected
Fig. 16.22 Serial seromuscular biopsies to establish level of aganglionosis. Biopsies are performed through the antimesenteric Tinea coli ,
preserving mucosal layer intact. Limited resection of grossly abnormal
colon segment
radiologically, the procedure is usually done through an
oblique left-lower quadrant incision. Some advocate performing a loop colostomy in the ganglionic proximal sigmoid colon, reasoning that a loop stoma does not interrupt
the mesenteric blood supply, leaving more options when the
defi nitive pull-through procedure is performed. We fi nd that
the propensity for loop stomas to prolapse make them less
desirable, and that chronic prolapse renders the proximal
limb chronically congested and thickened, and less suitable
for a pull-through anastomosis. The authors prefer a simple
end colostomy at a level proximal to the most dilated and
hypertrophied bowel. We resect any bowel above the transition zone that is massively dilated and hypertrophied as
we feel bowel chronically compromised in this fashion is
an unreliable pull-through segment even after decompression. Preservation of bowel length in this area is irrelevant,
and a pull-through procedure is easily performed using any
point in the sigmoid or proximal left colon. In creating the
end colostomy, the authors divide only the terminal vascular arcade to allow the colostomy to be straightened as it is
externalized, and usually position the stoma in the lateral
(superior) pole of the incision (Fig. 16.23 ). Some surgeons
prefer a mucous fi stula for decompression of the distal segment, but a closed distal pouch is well tolerated as long
as no dilated bowel is left above the transition zone. If a
mucous fi stula is performed, it is usually placed in the lower
20316 Gastrointestinal Stomas in Infants and Children
Fig. 16.23 End colostomy in upper pole of incision. Vented mucous
fi stula in lower pole
pole of the incision, and may be performed at skin level,
excising a corner of the staple line to provide a simple vent
(Fig. 16.24 ).
If the transition zone is suspected of being more proximal
than the rectosigmoid, then a vertical midline incision may
be used and the stoma created where appropriate. If intraoperative frozen section biopsies are unobtainable, either
because the operation is performed on an emergency basis or
appropriate pathology expertise is unavailable, then a loop
stoma may be created in the proximal transverse colon
(Fig. 16.25 ). This option decompresses in both directions
and will be above the transition zone in approximately 85%
of cases, and close enough to it in the remainder to provide
effective emergency decompression if required. Seromuscular
biopsies are taken and processed for later examination. If the
gross appearance of the bowel suggests total colon involvement, an end ileostomy should be performed.
Colostomy for Anorectal Malformations
Anorectal malformations are a heterogeneous group of congenital defects occurring in about 1 in 4,000 births. They are
broadly classifi ed according to the patient’s sex, the distance
Fig. 16.24 Completed “leveling” colostomy and mucous fi stula
from the end of the atretic rectum to perineum, and the
presence or absence of fi stulas in the perineum and genitourinary systems. Anorectal malformations are commonly
associated with genitourinary, sacral, cardiac, tracheoesophageal, and other anomalies, which must be investigated.
Their complexity and heterogeneity precludes an in-depth
discussion of defi nitive management of anorectal malformations and the associated anomalies here. Detailed descriptions are found in many excellent chapters by Pena and other
experts in standard pediatric surgery texts [ 10, 11 ] . Broadly
speaking, colostomies are usually constructed for “high”
defects prior to more complex reconstructions. “Low” defects
are usually reconstructed primarily in the neonatal period.
Decisions about “high” or “low” defects are made most of
the time based on the perineal examination, cross-table lateral fi lms, and urinalysis. These clinical decisions are generally delayed for 18–24 h after birth to allow time for
meconium to appear on the perineum, as in the case of imperforate anus with perineal fi stula, allowing identifi cation of
these “low” defects by visual inspection.
A colostomy is indicated in neonates with complex or
“high” defects (females with a cloaca, a rectovestibular fi stula, or no obvious perineal cutaneous fi stula; males with a
fl at underdeveloped perineum and buttocks, genitourinary
204 D.K. Magnuson and O.S. Soldes
Fig. 16.25 Proximal transverse loop colostomy
fi stula or without a perineal fi stula). In environments of limited resources and expertise with anorectal malformations,
when failure to relieve the distal obstruction will result in
signifi cant harm, a colostomy may be the wisest initial course
of action. Girls with a rectoperineal cutaneous fi stula may
undergo dilations of the fi stula, until an anoplasty can be
performed.
A descending divided colostomy with a distal mucous
fi stula done via a left lower quadrant oblique incision is rec-
small mucous fi stula are brought out at the upper and lower
ends of the wound and the fascia and skin are closed in
between. The distal meconium is irrigated out. This confi guration allows the proximal colostomy to be bagged separately from the mucous fi stula to prevent urinary tract
infection, in the case of genitourinary fi stulas. The mucous
fi stula allows access to the distal colon for colostography to
defi ne rectourethral and bladder neck fi stulas, passage of
urine to reduce metabolic acidosis from absorption, and
decompression of the distal segment to avoid megarectosigmoid. The divided descending colostomy reduces the risk of
stomal prolapse, versus loop colostomies. The critical point
Fig. 16.26 Descending divided colostomy for anorectal malformations
in the construction of the descending colostomy is to open it
just distal to the fi xed portion of the descending colon and
proximal to the more mobile sigmoid colon. This prevents
prolapse of the proximal stoma and allows adequate distal
length for the rectosigmoid colon to reach the perineum at
the time of defi nitive reconstruction via a laparoscopic pullthrough or posterior sagittal anorectoplasty.
References
1. Gauderer MW, Ponsky JL, Izant Jr RJ. Gastrostomy without laparo-
tomy: a percutaneous endoscopic technique. J Pediatr Surg.
1980;15:872–5.
2. Gauderer MWL. Feeding gastrostomy button: experience and rec-
ommendations. J Pediatr Surg. 1988;23:24–8.
3. Gauderer MWL, Picha GJ, Izant RJ. The gastrostomy “button” – a
simple, skin-level, nonrefl uxing device for long-term enteral feedings. J Pediatr Surg. 1984;19:803–5.
4. Nixdorff N, Diluciano J, Ponsky T, et al. The endoscopic U-stitch
technique for primary button placement: an institution’s experience.
Surg Endosc. 2010;24:1200–3.
20516 Gastrointestinal Stomas in Infants and Children
5. Williams AR, Borsellino A, Sugarman ID, et al. Roux-en-Y feeding
jejunostomy in infants and children. Eur J Pediatr Surg. 2007;17:29–33.
6. Bishop HS, Koop CE. Management of meconium ileus: resection,
Roux-en-Y anastomosis and ileostomy irrigation with pancreatic
enzymes. Ann Surg. 1957;145:410–4.
7. O’Neill JA, Grosfeld JR, Boles ET, et al. Surgical management of
meconium ileus. Am J Surg. 1970;119:99–105.
8. Nanigian DK, Kurzrock EA. Intermediate-term outcome of the simplifi ed laparoscopic antegrade continence enema procedure: less is
better. J Urol. 2008;179:299–303.
9. Swenson O, Rheinlander H, Diamond I. Hirschsprung’s disease: a
new concept of the etiology – operative results in thirty-four patients.
N Engl J Med. 1949;241:551–6.
10. Pena A. Atlas of surgical management of anorectal malformations.
New York: Springer; 1990.
11. Shaul DB, Harrison EA. Classifi cation of anorectal malformations –
initial approach, diagnostic tests and colostomy. Semin Pediatr Surg.
1997;6:187–95.
Antegrade Colonic Enema (ACE)
Brooke Gurland, Crina V. Floruta, and Ian C. Lavery
1 7
Introduction
Retrograde rectal irrigation through various enema techniques is a well-accepted practice to empty the lower bowel
and to relieve constipation and fecal soiling in patients with
functional bowel disorders [
can be technically challenging for elderly and disabled
patients and may require caregiver assistance. For the patient
with severe bowel dysfunction who is contemplating a permanent colostomy, the antegrade colonic enema (ACE) procedure may be a viable option. This procedure allows easy
access to the colon through the abdominal wall with intermittent catheterization, irrigation of the colon, and rapid,
controlled bowel purging (Fig. 17.1 ). The goal is to avoid
wearing a stoma pouch while allowing the patient to independently manage his or her own bowel activities.
The ACE technique was fi rst described by Malone in 1990
using the appendix as the conduit but since them the cecum,
ileum, and left colon have been utilized as the continence
mechanism [ 2– 5 ] . Malone adapted this concept from the
urology literature where a cutaneous appendicovesicostomy
was introduced to maintain urinary continence [ 6 ] . These
procedures have become increasingly popular for children
with spinal dysraphism and anorectal malformations and are
well reported in the pediatric literature [ 7 ] . The ACE proce-
dure is gaining recognition in the adult population for patients
with colonic neurologic dysfunction, colonic inertia,
obstructed defecation, and fecal incontinence [ 8– 15 ] .
Variations to the traditional ACE procedure have also
been reported with success. The results of laparoscopic ACE
procedures using the technique of in situ appendix without
cecoplication have promising results [ 16, 17 ] . Left-sided
ACE procedures with irrigation of the descending colon for
1 ] . However, retrograde enemas
B. Gurland (*) • C. V. Floruta • I. C. Lavery
Department of Colorectal Surgery , Digestive Disease Institute,
Cleveland Clinic Foundation , Cleveland , OH , USA
e-mail: gurlanb@ccf.org
V.W. Fazio et al. (eds.), Atlas of Intestinal Stomas,
DOI 10.1007/978-0-387-78851-7_17, © Springer Science+Business Media, LLC 2012
Fig. 17.1 The catheter is intermittently inserted into an orifi ce on the
anterior abdominal wall and into the cecum for irrigation of the colon
and rapid and controlled bowel purging. Illustration © CCF
207
208 B. Gurland et al.
Fig. 17.2 A 12–14 Fr silastic catheter is used to intubate the stoma.
A tube feeding bag with long tubing, to allow for adequate access, and
a handheld control fl ow regulator is recommended
patients with constipation have shown decreased irrigation
times and require less fl uid compared to those patients with
right colon access [ 18 ] . Percutaneous endoscopic placement
of tubes into the left or right colon can provide minimally
invasive access to the bowel for irrigation and decompression [ 19 ] .
Indications/Patient Selection
Patients who have severe bowel dysfunction with fecal soiling or constipation who have failed medical or surgical
treatments may be candidates for the ACE procedure. The
ACE procedure does not preclude colectomy or stoma creation. Successful outcomes depend on patient expectations
and motivations. Colonic irrigation is a life-long commitment and a rigid, time-consuming regimen. Bowel irrigation and evacuation takes approximately 45–60 min every
day or every other day to adequately purge the bowel.
Determining the correct volume of fl uid irrigant and additives for optimal colonic wash out is achieved through trial
and error. Nursing support to work with patients to determine the irrigation recipe is as important as the technical
aspects of the procedure.
Fig. 17.3 The abdominal wall is marked preoperatively so that the
conduit is not brought up into a fold in the abdominal wall and that
access is easy in the sitting or lying position. Any of the three positions
that are marked can be used depending on the patient body habitus.
Illustration © CCF
Preoperative Preparation
Equipment : Patients are familiarized with the enema equip-
ment preoperatively. A 12–14 Fr silastic catheter is used to
intubate the stoma. A tube feeding bag with long tubing to
allow for adequate access and a handheld control fl ow regulator is recommended (Fig. 17.2 ).
Stoma marking: The abdominal wall is marked preopera-
tively so that the conduit is not brought up into a fold in the
abdominal wall and access is easy in the sitting or lying position. It is imperative that the site is visible to the patient. The
conduit can be brought out through the umbilicus, right or
left lower quadrant. Although the umbilicus may be cosmetically appealing the authors prefer the right lower quadrant in
adults with a thick abdominal wall. For patients who are
wheelchair bound it may be necessary to site the stoma on
the upper abdomen for ease of access (Fig. 17.3 ).
Perioperative prophylactic antibiotics and bowel cleans-
ing preparations are given to all patients.
20917 Antegrade Colonic Enema (ACE)
Fig. 17.5 A valve mechanism is created to avoid backfl ow of fecal
material through the conduit. Illustration © CCF
Fig. 17.4 The tip of the appendix is removed and a stay suture is
inserted to stretch out the appendix to reveal the mesentery. A 12 Fr
catheter is passed through the appendix to the cecum. Illustration ©
CCF
Operative Steps
1. The ACE can be performed via a laparoscopic or open
technique with a midline, transverse, or right lower quadrant abdominal incision. The cecum is mobilized so that it
easily reaches the abdominal wall. Malone initially
described using the reversed appendix as the conduit with
the amputated tip tunneled in the cecum, but this is no
longer recommended and the appendix is left in situ.
2. The tip of the appendix is removed and a stay suture is
inserted to stretch out the appendix to reveal the mesentery. A 12 Fr catheter is passed through the appendix to
the cecum (Fig. 17.4 ).
3. A valve mechanism is created to avoid backfl ow of fecal
material through the conduit. The appendix is folded and
the cecum is loosely wrapped around the appendix
(Figs. 17.5 and 17.6 ). The suture picks up the seromuscu-
lar layer on the cecum on each side of the appendix to
anchor the tunnel. The cecum is anchored to the back of
the anterior abdominal wall where the appendix emerges
to prevent twisting and kinking of the conduit.
Fig. 17.6 The appendix is folded and the cecum is loosely wrapped
around the appendix. Illustration © CCF
4. For patients without an appendix: A 5–10 cm segment of
terminal ileum is isolated on its vascular pedicle
(Fig. 17.7 ). A longer conduit is needed for a patient with
a thicker abdominal wall. Bowel continuity is restored
using a standard end-to-end anastomosis (Fig. 17.8 ). The
isolated ileum is tubularized over a 12 Fr catheter by using
a stapling device on the antimesenteric surface to narrow
the lumen (Fig. 17.9 ). One end is then implanted into a
submucosal tenial tunnel in the cecum and the other is
brought to the skin as a stoma.
5. The colonic submucosal tunnel is created by incising the
seromuscular layer of the tenia with a scalpel down to the
210 B. Gurland et al.
Fig. 17.7 For patients without an appendix, a 5–10 cm segment of terminal ileum is isolated on its vascular pedicle. Illustration © CCF
Fig. 17.8 Small bowel continuity is restored using a standard
end-to-end anastomosis. Illustration © CCF
Fig. 17.9 The bowel is
tubularized over a 12 Fr catheter
by using a stapling device on the
antimesenteric surface to narrow
the lumen. Illustration © CCF
21117 Antegrade Colonic Enema (ACE)
Fig. 17.10 An enterotomy is made at the distal end of the tunnel and
the mucosa is sutured to the full thickness of the ileal conduit using
absorbable sutures. Illustration © CCF
submucosa over a 7 cm length. An enterotomy is made at
the distal end of the tunnel and the mucosa is sutured to
the full thickness of the ileal conduit using 3.0 Vicryl
sutures (Fig. 17.10 ). The seromuscular wall of the colon
is closed over the conduit using 3.0 chromic sutures picking up partial thickness of the tunnel to prevent slippage
(Fig. 17.11 ).
6. Stoma creation: A V-shape skin incision is made at the
previously marked stoma location. An aperture is created through the abdominal wall that is suffi ciently
wide to allow the conduit to pass freely. The cecum is
sutured to the anterior abdominal wall to prevent tension
on the stoma or volvulus of the bowel on the conduit. If
the conduit has a very small caliber, such as an appendix,
it is spatulated and the apex of the V-fl ap is sutured into
the defect using 4.0 chromic sutures with the knots outside the catheterizing channel (Figs. 17.12 and 17.13 ) .
Postoperative Care
A 12 Fr silicone catheter remains in the conduit for a minimum of 21 days postoperatively. Irrigations start on postoperative day number 4, using 500–1000 cm 3 of normal saline
as tolerated by patient.
The patient is started on a liquid diet on postoperative day
number one and advanced to a GI soft diet as tolerated.
The patient is discharged with the indwelling catheter on
day 5 and is expected to irrigate the indwelling daily with
Fig. 17.11 The seromuscular wall of the colon is closed over the conduit, picking up partial thickness of the tunnel to prevent slippage.
Illustration © CCF
500–1000 cm 3 of tap water. The patient returns 3 weeks later
to learn intermittent catheterization.
The stoma should be catheterized daily whether or not an
enema is being given to avoid stenosis.
Complications
Stoma stenosis and local wound infections are the most
common complications reported.
Leakage of stool is an uncommon complication. If this
occurs the valve mechanism can be revised.
Conclusion
The ACE procedure is an alternative for patients with refractory fecal incontinence or constipation who would like to avoid
wearing a permanent stoma appliance. Colonic irrigation is a
lifelong commitment and it requires a motivated patient and
educated nursing staff for ongoing support. However, for the
appropriate individual the ACE can be very gratifying.