Seminars in Pediatric Surgery 26 (2017) 379–383

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Seminars in Pediatric Surgery

journal homepage: www.elsevier.com/locate/sempedsurg

Surgical options in the treatment of ulcerative colitis

Daniel P. Ryan, MD, FACSn, Daniel P. Doody, MD, FACS
Department of Pediatric Surgery, MassGeneral Hospital for Children, Harvard Medical School, 55 Fruit St, Boston, Massachusetts 02114

a r t i c l e in fo a b s t r a c t

Children and young adults with ulcerative colitis tend to present with more extensive colonic disease
Keywords: than an adult population. The need for surgical intervention in the pediatric population with ulcerative
Pediatric ulcerative colitis colitis occurs earlier after diagnosis and has a greater incidence than a comparably matched adult
Ileostomy population with an estimated need for colectomy at 5 years following diagnosis of 14–20%. Perhaps, even
Restorative proctocolectomy more than the adult population, there is a desire to restore intestinal continuity for the pediatric patient
Ileoanal pouch to achieve as healthy and normal quality of life as possible. With surgery playing such a prominent role in
Pouchitis the treatment of ulcerative colitis in this age group, an understanding of the surgical treatment options
Crohn disease
that are available is important. The surgeon’s awareness of the complexities of the different operations
associated with proctocolectomy and reestablishing intestinal continuity may help to avoid early
complications and minimize the risk of less than ideal long-term outcomes.
& 2017 Elsevier Inc. All rights reserved.

The surgical treatment of ulcerative colitis has been evolving
over the last 50 years. Ulcerative colitis, a mucosal disease
confined to the colon and rectum, is amenable to a “surgical cure”
(no colon, no colitis), unlike Crohn disease which affects the entire
alimentary tract. In the past, it was identified that removing the
colon and diverting the fecal stream would allow the inflammatory
process of the rectum to resolve or become treatable locally. Once
the fecal stream was reestablished, the inflammatory process often
returned in the rectum over time. The initial surgical option, until
the late 1960s was total proctocolectomy and an ileostomy. This
operation may still be the best option for some patients, although
surgery to maintain intestinal continuity is the primary goal for
most children and young adults.
Indications
Indications for removing the colon in patients with ulcerative
colitis have been fairly well established. The reasons may be
emergent, urgent, or elective. Over the course of the disease
anywhere from 20% to 45% of all patients with ulcerative colitis
will need surgery.1–4 Many patients presenting with newly dis-
covered colitis or acute flare of their disease can be treated with
medical therapy and go into remission. The medical therapy of
n the new biologic agents, like the tumor necrosis factor inhibitors,
Correspondence to: Department of Pediatric Surgery, MassGeneral Hospital for
Children, Harvard Medical School, 55 Fruit St, Boston, MA 02114.
E-mail address: DRYAN@PARTNERS.ORG (D.P. Ryan).
ulcerative colitis is discussed further in other sections of this
journal.
The most common indication for surgery is “failure of medical
treatment”, either as significant ongoing symptoms despite med-
ical therapy or unacceptable side effects to medical treatment.
Emergency surgery for ulcerative colitis can become necessary due
to colonic perforation, ongoing severe bleeding, or toxic mega-
colon. A certain percentage of patients will fail the initial medical
treatment or present with an uncontrolled acute flare of the
disease and, despite optimal medical therapy, will require urgent
surgery to treat the poorly controlled colitis.5 The safest surgical
option in the emergency situation is an abdominal colectomy with
ileostomy and Hartmann pouch, preserving the rectum for later
reconstruction. In the population with a need for urgent surgical
treatment, the options for immediate reconstruction or diversion
will be determined by patient factors, including nutritional status,
patient’s desires, and the degree and length of corticosteroid
therapy among other things.
Even in patients with long-standing, well controlled
disease, there is a risk of cancer developing.6 This risk seems to
be higher in patients with pancolitis. Approximately 5% of patients
will develop cancer related to the colitis. The risk of developing
neoplasia also increases with duration of the disease.7 Patients
often develop mucosal dysplasia before developing cancer, and
surgery should be considered if these changes are found on
surveillance colonoscopy.8 Recent studies have suggested that
may decrease the risk for both colectomy and cancer develop-
ment.9–12

http://dx.doi.org/10.1053/j.sempedsurg.2017.10.001
1055-8586/& 2017 Elsevier Inc. All rights reserved.
380 D.P. Ryan, D.P. Doody / Seminars in Pediatric Surgery 26 (2017) 379–383
Surgical options
Removing the entire colon and rectum will cure the disease of
ulcerative colitis. For many years the only option for patients was a
permanent ileostomy. In the late 1960s, the concept of a “continent
ileostomy” was developed. The most successful of these continent
ileostomy constructs was the Kock pouch that allowed the patient
to pass a catheter into the stoma and empty the reservoir at a
convenient time, instead of wearing an external appliance.13–15
Many patients had excellent outcomes from this procedure;
however, particularly in the early experience, a significant number
of patients had complications or required multiple operations to
obtain a good result.16–19
In 10–29% of the patients, even though the colitis was gone, a
new inflammatory process developed within the continent pouch
itself and was called “pouchitis.” This may affect any ileal pouch in
patients with ulcerative colitis, but rarely complicates the same
operation for polyposis syndromes.20–22 The pouch inflammation
often responds to a course of oral antibiotics. The exact cause of
this problem remains uncertain and will be discussed in another
chapter in this issue.
In the late 1970s, some surgical centers proved the feasibility of
an ileoanal anastomosis after total colectomy and rectal mucosec-
tomy to treat ulcerative colitis.23–27 The concept allowed patients
to evacuate normally and avoid having a lifelong stoma. One key to
a successful ‘restorative proctocolectomy’ was removing the
inflamed mucosa from the rectum while preserving the muscles
of continence and the normal innervation in the pelvis that would
allow the patients to sense and control their bowel movements.
Using part of terminal ileum either directly or as a reservoir to
replace the rectum was proposed and proved. By constructing an
internal pouch, it was felt the overall number of bowel movements
would be decreased as compared to direct ileoanal anastomosis
without reservoir.28–30
While Martin’s early report first used a straight ileoanal
anastomosis,25 Parks and Nicholls26 described a folded S-pouch,
acknowledging the previous work of Kock proving that ileal pouch
formation could be associated with near-normal intestinal func-
tion. The “J” pouch was described shortly thereafter,31 and other
modifications were developed over time which included a “W”
configuration (Figure).32,33 It was hoped that these more compli-
cated constructions would have advantages, acting as a larger
reservoir and decreasing the number of daily bowel movements.
However, over time, many surgical groups have gone back to the
Fig. Common procedures to restore intestinal continuity following proctocolectomy. (A) Direct or straight ileoanal anastomosis without reservoir. (B) Utsunomiya’s J-pouch
with side-to-end ileoanal anastomosis. (C) Parks’ S-pouch ileoanal anastomosis with the efferent limb in the sphincter complex with an end-to-end ileoanal anastomosis.
(D) Quadruple limb W-pouch ileoanal anastomosis with side-to-end anastomosis as described by Nicholls and Harms. (E) Distal rectal-ileal pouch stapled anastomosis with
the stapled anastomosis above the sphincter complex.
original J-pouch as an easier intestinal reconstruction with equally
good outcomes. The S-pouch reservoir may develop problems with
the efferent limb lengthening and kinking over the sphincter
complex, contributing to outlet obstruction. In one series, it
seemed that the larger volume of the S-pouch led to more
problems with pouchitis,34 although another group noted that
chronic pouchitis was seen more frequently with a J-pouch
construction.35 A few groups do not use a pouch,36,37 but other
authors found that patients without an ileoanal reservoir typically
have a greater number of daily bowel movements.29,38
The development of improved stapling devices has facilitated
the construction of the J-pouch and allowed surgeons to use the
circular stapling devices for a distal ileorectal anastomosis as well.
Bleeding from the staple lines is less of a problem with modern
stapling devices. There are no prospective studies to identify
advantages or disadvantages between the stapling devices and
hand sewn ileoanal anastomosis. One retrospective analysis
of a large database did not show any difference, except for a
higher incidence of anal strictures with the hand sewn
anastomosis.39
The procedure for the total colectomy and ileorectal or ileoanal
anastomosis has evolved along with the development of minimally
invasive laparoscopic surgery techniques.40–42 It is currently pos-
sible to do the operation with good outcomes laparoscopically or
with a traditional open approach. Many people do a “laparoscopic-
assisted” operation to remove the colon and mobilize the small
bowel. A small incision is then made to remove the colon speci-
men, construct the pouch, and complete the anastomosis. This
allows for a more cosmetic lower abdominal Pfannenstiel-type
incision, which many patients prefer. A “hand assisted” laparo-
scopic technique is also used in some centers.43 In a few select
centers, “single-port” laparoscopic surgery has been described in
children and adults.44,45
Technical aspects
One of the essential elements of this operation is adequate
mobilization of the small intestine to be able to bring down the
pouch to the anus or rectum without any tension and with
adequate blood supply. Care has to be taken to identify the
appropriate blood vessels that may need to be divided for
mobilization while preserving adequate blood supply to the pouch.
Intraoperative Doppler probes can be useful in identifying the
 D.P. Ryan, D.P. Doody / Seminars in Pediatric Surgery 26 (2017) 379–383
vascular anatomy and confirming good pulsatile flow in the vessels
to the pouch when various vessels are temporarily occluded with
vascular clamps. Freeing up the mesentery up to the ligament of
Treitz area and the distal duodenum are other aspects of the small
bowel mobilization. An inadequate mobilization or compromise of
the blood supply can lead to problems with leak or pelvic sepsis,
may contribute to pouch retraction and stricture formation, and all
of these problems potentially may contribute to chronic pouch
dysfunction. One must avoid twisting the mesentery or the bowel
as the pouch is delivered into the pelvis.
The method of the ileoanal anastomosis has been controversial
—a hand-sewn ileoanal anastomosis versus a stapled distal rectal-
ileal pouch anastomosis. There are no randomized studies to
determine if one is better than the other. There are potential
problems with both approaches. The exact length of the internal
sphincter that needs to be preserved for good outcome is not
totally clear. Certainly leaving 3–4 cm is enough in adults. This
internal distance can be easily reached from a dissection transa-
nally. If a circular stapled anastomosis is planned, one can perform
a perimuscular laparoscopic or open dissection from above in
order to place a stapling device through the anus for the anasto-
mosis to the distal rectum. Care should be taken to avoid having
the circular staple line cross another staple line in the pouch or the
rectum, as this may lead to an increased risk of pouch leak.
Preserving the anal mucosa and the “transition zone”, which
extends approximately 1 cm above the top of the anorectal
columns, is important for maintaining fine continence, namely
the sensation to determine if one is evacuating solid, liquid or
flatus to prevent accidental soiling. If the ileal pouch is brought too
low, while gross continence may be preserved, involuntary acci-
dents and soiling can lead to patient dissatisfaction. Leaving too
much of a rectal cuff with inflamed rectal mucosa (although this
preserves continence) can lead to problems with “cuffitis,” an
ongoing colitis in the retained rectal mucosa. With an ileal pouch-
distal rectal anastomosis, there may be a need for ongoing medical
treatment as well as the need for more frequent surveillance for
dysplasia or malignant change in the rectal remnant. Stricture
formation appears to be greater with a hand sewn ileoanal
anastomosis, perhaps because the anastomosis is lower than when
the stapler is used.39
Placing a drain into the pelvis near the pouch is recommended.
There is often a large volume of fluid that is removed in the first
few days because of the dissection of the lymphatics in the
mesorectum. Removing this fluid may lessen the risk of pelvic
infections. In addition, once bowel function returns, a leak can be
identified early and prompt treatment initiated. If a leak
occurs, the surgical approach can include endoscopic pouch
evaluation with closure of the leak with or without a diverting
ileostomy.
Another area of controversy about the procedure is whether a
proximal diverting ileostomy at the time of the ileoanal pouch
procedure is necessary or beneficial. Many groups feel strongly
that every new pouch should be protected by a proximal diverting
ileostomy for a short period of time, usually 8–12 weeks. Others
have achieved equivalent outcomes without a proximal ileostomy
in selected cases.34,46,47 At least one group has offered a model
based on a large retrospective review that identified variables that
may help the surgeon in the decision to perform the procedure
with or without a proximal diverting ileostomy.48 The in-hospital
recovery from an ileoanal procedure without a diverting ileostomy
is longer than in-hospital stay if a diverting ileostomy is per-
formed. Not performing a diverting ileostomy, however, avoids a
second operation and hospitalization in select patients and is
associated with similar outcomes and complications. Currently,
however, there are no randomized studies to help resolve this
question.
381
Outcomes
The complication rate for this ileoanal pouch procedure is
substantial, as would be expected. The usual issues with wound
infection, pouch leakage, pelvic abscess, pouch retraction, and
stricture of the ileoanal anastomosis are well described. The
overall rate of problems in most studies approaches 20–50%.49–51
Small bowel obstruction appears to be higher with this particular
operation, approaching 15–27% in most series.34,52–57
Because the presence of inflammatory bowel disease is a
significant risk for deep venous thrombosis (DVT)58–62 and rarely
mesenteric venous thrombosis,63–65 consideration of anticoagula-
tion in the perioperative period is appropriate. Patients with
inflammatory bowel disease are at greater risk than other patients
for DVT during hospitalizations for medical therapy, and this
thrombosis risk has been reported to be even greater with surgery,
with DVT occurring in 5–8% of patients undergoing IBD-related
surgery.60,66 Patients who develop mesenteric venous thrombosis
typically present with abdominal pain approximately 3 weeks
after the procedure, along with nausea and vomiting. The physical
examination may be nonspecific. CT scanning with intravenous
contrast is the diagnostic modality of choice. If mesenteric clot is
seen, a course of systemic anticoagulation should be instituted and
is usually successful.63
Another issue that has been recognized recently is the fact that
the ileal pouch is at risk for dysplasia and tumor formation. Cases
of adenocarcinoma of the pouch have been identified.67,68 Dyspla-
sia of the pouch is believed to be a precursor to the development
of cancer. A longer duration of colitis before surgical treatment, a
family history of colorectal cancer, a longer time with the pouch, a
history of chronic pouchitis, the presence of primary sclerosing
cholangitis, an ileal pouch-distal rectal anastomosis, and dysplasia
or carcinoma in situ in the colon specimen are risk factors for the
development of pouch neoplasias.67–70 Patients with an ileal
pouch should have surveillance endoscopy and biopsies 1 year
after the construction of the pouch and every 3 years thereafter.
Patients with risk factors will need more frequent surveillance.
The ileoanal pouch created in the treatment of ulcerative colitis
is at significant risk for pouchitis (40–50%).71,72 One group sug-
gests an initial endoscopy at 6 months following closure of the
diverting ileostomy to predict the risk and characteristics of future
pouchitis.73 The problem of pouchitis and its treatment is
addressed more completely in another manuscript in this issue.
Some patients can present with symptoms similar to acute or
chronic pouchitis and are found to have Crohn disease on endos-
copy and biopsies. These patients may also present with fistulizing
disease and perianal infections or have signs of small bowel Crohn
disease with thickening of the bowel wall and stricture formation
well above the pouch.74–76 In one report, up to 14% of patients with
presumed ulcerative colitis subsequently had biopsies confirming
granulomas and were assigned the diagnosis of Crohn disease
following removal of the colon and construction of the pouch. The
distinction between the surgical complications that may occur
with creation of the pouch and Crohn disease involving the pouch
can be difficult. The presence of granuloma on endoscopic biopsies
of the pouch confirms the diagnosis of Crohn disease. Imaging
studies, including contrast enemas and CT or MR enterography
may identify proximal inflammation in the small bowel that would
argue for Crohn disease. Many times, granulomas are not found
and there is no clear evidence of proximal small bowel disease. In
those cases of chronic pouchitis that do not respond to antibiotic
therapy or persistent and refractory perianal disease, one should
consider a trial of immunomodulators or biologics to treat a
presumed Crohn disease of the pouch. These therapies may lead
to rescue of what appears to be a failing pouch.77 Unfortunately,
even with aggressive therapy to treat proven Crohn disease
382 D.P. Ryan, D.P. Doody / Seminars in Pediatric Surgery 26 (2017) 379–383
involving an ileal pouch, chronic, unrelenting symptoms lead to
proximal diversion or pouch excision in approximately 30% of
patients.78
An unusual late problem of restorative proctocolectomy is
prolapse of the pouch. It is believed that this problem is created
by excessive straining to evacuate the pouch, and a stricture
should be ruled out. This problem may require pouch revision or
proctopexy.28,34
While minor ileoanal strictures may respond to simple dilation,
more chronic and refractory strictures may need to be addressed
surgically. Most problems with retraction and/or stricture may be
approached transanally, advancing the pouch and reestablishing
the anastomosis at the appropriate level. The length of the
stricture may help to predict which patients will need intra-
abdominal mobilization to gain adequate vascular length to safely
bring the pouch to the anorectal line.
For those patients with chronic and refractory pouchitis,
intestinal continuity can be salvaged in select patients by remov-
ing the failed pouch and creating a new one if adequate mesenteric
length is available. In some instances, a straight ileoanal anasto-
mosis may be necessary to avoid a permanent stoma and maintain
continence.
Sexual activity and enjoyment in women who have had
colectomy and ileoanal pouch procedure is often normal, but can
be impaired in those with poor pouch function. There is a risk of
infertility in women following this procedure. However two-thirds
of women are able to conceive without in vitro fertilization79 and,
if necessary, they can become pregnant with in vitro fertilization at
a comparable rate as the general population.80 Most pregnancies
are carried to term and vaginal delivery would be expected,
although the need for caesarian section is greater in women with
an ileal pouch as compared to the general population.81
In long-term follow-up, failure of the pouch requiring an
ileostomy occurs in 10–14% of patients.82,83 In one meta-analysis
of adult patients, the authors concluded that proctocolectomy with
ileostomy was equivalent to ileoanal pouch procedure in terms of
overall health-related quality of life.84 Despite the immediate
complications associated with this complex surgery, the chronic
problems with pouchitis, and the long-term risk of pouch failure,
many studies document an acceptable quality of life following
colectomy and ileoanal pouch procedure.85,86 Median stool fre-
quency ranges from 4 to 8 per 24-hour period. Most patients are
fully continent during the day and usually also at night. Urgency
and the occasional accident are common complaints but do not
interfere with activities of daily living. In these surveys, 90–95% of
patients undergoing a restorative proctocolectomy are satisfied or
very satisfied with their choice and quality of life. Despite the
difficult recovery and increased complication risk, but with the
promise of a good to very good quality of life, we believe that ileal
pouch anal anastomosis to re-establish intestinal continuity is the
preferred operation for pediatric and adolescent patients with
complications of their disease or adverse effects from their medical
therapy.
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