Acs0511 Crohn Disease 2004

© 2004 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice
5 GASTROINTESTINAL TRACT AND ABDOMEN 11 CROHN DISEASE — 1

11 CROHN DISEASE
Susan Galandiuk, M.D., F.A.C.S., F.A.S.C.R.S.

The role of surgery in the management of Crohn disease has the known risk of disease recurrence after surgical treatment of
undergone a dramatic evolution over the past 50 years. Crohn disease and the significant associated operative morbidity. In
Currently, surgical treatment of Crohn disease is seldom per- one single-center study, the reoperation rate for Crohn disease was
formed in the emergency setting; it is nearly always performed 34% at 10 years.3 The agents used to treat Crohn disease can be di-
after failed medical therapy.The decision to proceed with opera- vided into several broad groups: probiotics, antibiotics, anti-inflam-
tive management is based on careful patient evaluation, with full matory drugs, immunosuppressive drugs, and biologic agents.These
awareness of the potential complications and ramifications of can be used alone or in combination to treat disease, as well as to
treatment. In particular, attention must be paid to the risk of maintain remission [see Table 1].
recurrent disease, the possible surgical sequelae, and the side Few good studies have been done on the cost-effectiveness of
effects of medical therapy. medical or surgical therapy4,5 versus that of timely surgery fol-
lowed by maintenance medical therapy.There is clearly a need for
such studies.The use of potent and expensive immunomodulator
Classification therapy (e.g., maintenance infliximab) for simple ileocolic disease
There are many systems for classifying Crohn disease. One of is questionable, especially in the light of studies indicating that
the simplest is the classification developed by Farmer and associ- such treatment is not at all innocuous.6,7
ates,1 which categorizes the disease on the basis of disease location
CHANGING CONCEPTS IN SURGERY FOR CROHN DISEASE
alone (ileocolic, purely colonic, small bowel, and perianal). A more
elaborate system is the Vienna classification, which categorizes the Although first described in the beginning of the 19th century,
disease on the basis not only of location but also of age of onset Crohn disease was not recognized as a discrete clinical entity until
and disease behavior.2 In this system, there are four categories for the first part of the 20th century.8 At one point, it was treated sur-
disease location: terminal ileum (L1), colon (L2), terminal ileum gically in much the same way as cancer, with frozen-section mar-
and colon (L3), and any location proximal to the terminal ileum gins obtained at the time of resection.This approach did not yield
(L4). There are two categories for age of onset: less than 40 years any substantial reduction in the recurrence rate.9 In fact, overzeal-
of age (A1) and 40 years of age or older (A2). Finally, there are ous resections often resulted in Crohn patients’ requiring lifelong
three categories for disease behavior: nonstricturing and nonpen- parenteral nutritional support.10 Accordingly, conservative surgery
etrating (B1), stricturing (B2), and penetrating (B3). is now the rule: only gross macroscopic disease is resected into pal-
Given that there are as many types and combinations of Crohn pably normal margins (in particular, a palpably normal mesenteric
disease as there are patients with this condition, the most sensible border of the bowel).
approach is probably to use some combination of these two clas-
sification schemes. Careful evaluation of the specifics of each case
will yield the best treatment results; however, general classification General Indications for Surgical Treatment
of the disease can help guide therapy. Broadly speaking, Crohn
SIDE EFFECTS OF MEDICAL THERAPY
disease of the small bowel has the highest recurrence rate. Because
of the important function of the small bowel in digestion, surgeons Significant side effects of medical therapy include those associ-
tend to emphasize conserving small bowel length during operative ated with failure to wean from prednisone (e.g., cataract forma-
treatment of Crohn disease. Currently, however, there is an in-
creasing focus on colon conservation with the aims of maintaining
water absorption in patients and delaying (or perhaps eliminating) Table 1 Medical Treatment of Crohn Disease
the need for a stoma.
Category Example Application Form Expense
Roles of Medical Therapy and Surgical Therapy
Probiotics Food, capsules,
Lactobacillus $–$$
In planning treatment of Crohn disease, it is important not to pills, powders
make the use of medical therapy or surgical therapy an either-or Metronidazole,
Antibiotics p.o., I.V. $–$$
issue. Just as one tool cannot be expected to fill every household ciprofloxacin
need, operative management cannot be expected to solve every
p.o. $
problem related to Crohn disease. Overall, careful use of medical Sulfasalazine
Anti-inflammatories p.o., suppositories, $$
therapy, appropriately combined with surgical therapy, provides 5-ASA products
enemas
the best treatment of Crohn disease. Single-minded reliance on
Conventional steroids p.o., I.V. $
either therapy to the exclusion of the other often leads to inade-
Budesonide p.o. $$
quate patient care. Immunosuppressives Antimetabolites p.o. $$
Generally speaking, except in the case of a free perforation, can- Methotrexate p.o. or I.M. $
cer, or dysplasia, one should not operate on a patient with Crohn Cyclosporine p.o. or I.V. $$
disease without first attempting medical therapy.With the dramati-
Infliximab $$$$
cally improved medical treatment options currently available, surgery Biologics —
Investigational agents NA
can be avoided in many cases.This is often a desirable result, given


Abscess Formation
Abscesses are particularly common with ileocolic Crohn dis-
ease. If they cannot be controlled by means of computed tomog-
raphy–guided drainage, surgical therapy may be indicated.
Cancer or Dysplasia
The risk of colorectal cancer is approximately three times higher
in patients with Crohn disease than in the general population.11-13
Failure to Grow
In children, failure to grow and develop normally is one of the
main indications that medical therapy for Crohn disease has been
unsuccessful.Timely surgical therapy will permit normal develop-
ment. On occasion, when bone age lags significantly behind
chronological age, treatment with recombinant human growth hor-
mone is required.

Figure 1 Shown is an example of stenotic ileocolic Crohn dis- Special Considerations
ease resulting in obstructive symptoms that were not relieved by
medical therapy. PREGNANCY

Persons who have Crohn disease may be less fertile than healthy
tion, aseptic necrosis of the femoral head, and weight gain). Side age-matched persons. One possible explanation for this difference is
effects of antimetabolite therapy include pancreatitis, neutrope- that feeling ill may result in reduced sexual desire or decreased sexu-
nia, and opportunistic infections. al activity. Another is that pelvic inflammation caused by Crohn dis-
ease or by scarring and adhesion formation resulting from surgery
COMPLICATIONS OF DISEASE may impair fertility.To reduce the chances of the latter, hyaluronic
acid sheets may be placed around the tubes and ovaries; alternative-
Lack of Response to Medical Therapy ly, the ovaries may be tacked to the undersurface of the anterior ab-
Many patients with so-called toxic colitis do not respond satis- dominal wall with absorbable sutures and thereby prevented from
factorily to medical treatment. In severe cases of refractory dis- entering the pelvis.
ease, if surgery is not performed, colonic perforation, peritonitis,
and multiple organ failure may ensue. Such cases are much less
frequent now than they once were.
Obstruction
In many patients with Crohn disease, the behavior of the dis-
ease changes over time, from a more inflammatory and edema-
tous process to one characterized more by fibrosis and scarring.
Whereas anti-inflammatory drugs are ideal for treating the for-
mer, surgery is frequently necessary for the latter. Failure to refer
for surgical treatment of obstruction is, unfortunately, a common
error among gastroenterologists. Severe abdominal pain is always
a warning sign of obstruction and should be taken seriously [see
5:1 Acute Abdominal Pain and 5:4 Intestinal Obstruction]. The im-
portance of this point is illustrated by a case from my experience,
involving a patient who had obstructing ileocolic Crohn disease
with gross proximal distention of the terminal ileum [see Figure 1].
This patient lost 20 lb, was experiencing severe abdominal pain,
and was treated for more than a year with 6-mercaptopurine before
being referred for operative management. Ileocolic resection led
to rapid resolution of the symptoms.
Symptomatic Fistulas
Enteroenteric fistulas, by themselves, are no longer considered
an absolute indication for operation in the absence of other com-
plicating factors. Symptomatic fistulas, such as those associated
with obstruction or those associated with disabling symptoms
(e.g., rectovaginal fistulas or enterocutaneous fistulas [see Figure
2]), may have to be treated surgically. Ileosigmoid fistulas, which Figure 2 Shown is an enterocutaneous fistula that persisted for
effectively bypass the entire colon, may be associated with pro- more than 1 year after an ileocolic resection (arrow). The choice
found and refractory diarrhea (i.e., ≥ 20 bowel movements/day) of parallel incisions by the previous surgeon made selection of a
and may also have to be treated operatively. temporary stoma site much more difficult.


There is no evidence that pregnancy exacerbates Crohn disease; not hesitate to convert to an open procedure.Typically, most areas
however, there are some specific concerns that apply to pregnant that feel fibrotic or contain fibrotic adhesions are actually areas of
patients with this condition. Because patients with Crohn disease fistulizing disease and should be treated as such until proved oth-
often have more-liquid bowel movements, they have a particular erwise. In one study, patients with recurrent disease, those older
need for a well-functioning anal sphincter. If there is any chance of than 40 years, and those with an abdominal mass were more like-
an obstetrics-related injury (e.g., from a large baby in a prima- ly to require conversion to an open procedure.16
gravida or from a breech presentation), a cesarean section is advis-
able to minimize the risk of sphincter trauma.The same is true in
the presence of severe perianal Crohn disease. During pregnancy, Surgical Management of Crohn Disease at Specific Sites
prednisone and 5-aminosalicylic acid (5-ASA) medications are
ESOPHAGEAL, GASTRIC, AND DUODENAL DISEASE
safe, whereas drugs such as metronidazole are not. If imaging
studies are needed, magnetic resonance imaging and ultrasonog- Crohn disease of the upper alimentary tract can be difficult to
raphy are the modalities of choice. diagnose, largely because it is relatively uncommon. Obstructing
strictures due to Crohn disease in this area are unusual; the unsus-
MARKING OF STOMA SITES AND CHOICE OF INCISION
pected finding of noncaseating granulomas in biopsies of erythem-
When a patient with Crohn disease is expected to need an atous areas in a patient with Crohn disease in other locations is
ileostomy [see 5:30 Intestinal Stomas], it is extremely important to diagnostic.
mark the site preoperatively.What looks flat when the patient is on Occasionally, a patient with Crohn disease of the distal esopha-
the operating table may not be flat when he or she is upright.The gus requires dilatations, but this is uncommon. Surgical treatment
patient must be asked to sit and lean over to confirm that the for Crohn disease of the upper alimentary tract is almost exclu-
marked stoma site is in an area without folds, creases, or previous sively reserved for disease affecting the duodenum. Diagnosis of
incisions. Stoma appliances do not adhere well to areas of previ- duodenal Crohn disease can be difficult and requires a certain
ous scarring, and these should be avoided whenever possible. amount of suspicion. Frequently, the diagnosis is not made until
Patients with Crohn disease do not react to intra-abdominal relatively late, because diagnostic imaging tends to focus on
infection in a typical fashion. It is not unusual to find unsuspect- endoscopy and because the degree of duodenal obstruction is
ed abscesses that were not revealed by preoperative CT scans and often not evident except on barium studies.The rigidity and lumi-
other imaging studies. If there is even a remote chance of an nal narrowing of the second portion of the duodenum is typically
unsuspected abscess (particularly in cases of obstructing ileocolic much more readily apparent on contrast studies than on endos-
Crohn disease), the possibility of a temporary stoma should be copy. Duodenal Crohn disease can lead to gastric outlet obstruc-
raised with the patient and the proposed stoma site marked pre- tion. In children, it can be mistaken for annular pancreas.
operatively. When duodenal Crohn disease does not respond to medical
A key point is the necessity of planning for the future. Many therapy, gastrojejunostomy with vagotomy is the preferred surgical
patients with Crohn disease will eventually require a stoma. Oper- treatment.17,18 Failure to perform a vagotomy may result in mar-
ating through a midline abdominal incision preserves all four quad- ginal ulcer formation and obstruction. Some surgeons have per-
rants for possible future stoma sites (if needed). formed duodenal strictureplasty to treat duodenal Crohn disease.
The results have been conflicting19,20; the feasibility of this opera-
LAPAROSCOPY
tive approach is limited by the pliability of the duodenum. Many
Laparoscopic surgical techniques have gained acceptance in the patients experience prompt and full recovery of normal gastric
treatment of Crohn disease. In performing a laparoscopic opera- emptying after operation, but some patients with long-standing
tion for Crohn disease, it is essential to adhere to the same techni- gastric outlet obstruction continue to experience impaired empty-
cal standards that apply to corresponding open procedures. ing. The latter may benefit from administration of a prokinetic
Careful intraoperative exploration of the abdomen is important, in agent (e.g., metoclopramide or erythromycin).
that many patients have multifocal disease. Without such explo-
JEJUNOILEAL DISEASE
ration, patients may experience persistent postoperative symptoms
as a consequence of persistent proximal pathologic states that were
not addressed. As with other treatment modalities, there are some Short Bowel Syndrome
circumstances in which laparoscopy is particularly useful and oth- Although Crohn disease of the small bowel is not common and
ers in which it should not be used. For example, a laparoscopic accounts for a relatively small proportion of all cases, disease in
approach is ideal for fecal diversion in patients with perianal this area is associated with one of the highest overall recurrence
Crohn disease. rates. Resection of large portions of the small bowel can result in
Ileocolic resection for Crohn disease also lends itself well to a short bowel syndrome. For this reason, before proceeding with any
laparoscopic-assisted approach; compared with open resection, type of small bowel or ileocolic resection, one should measure the
laparoscopic resection has been reported to result in shorter hos- length of the existing small bowel to determine the patient’s
pital stays and reduced costs.14,15 The ileocolic vessels originate “bowel resource.” One naturally would more readily perform a
centrally, and they only lie over the retroperitoneum. Once the lat- resection in a patient who has 400 cm of normal small bowel than
eral peritoneal attachments are divided, the colon and the small in one who has only 200 cm.
bowel mesentery can be exteriorized, and the mesentery can be
divided and the anastomosis performed extracorporeally. Resection versus Strictureplasty
Many studies have shown that even fistulizing Crohn disease The major advance in the surgical treatment of Crohn disease
can be safely addressed laparoscopically, depending on the skill of over the past quarter-century has been the technique of small
the surgeon. A hand-assisted approach is often useful with cases of bowel strictureplasty, first proposed by Lee and subsequently pop-
dense fixation, in which fistulas are common and finger dissection ularized by Williams, Fazio, and others.17,18 Currently, the two
may facilitate definition of the anatomy. If in doubt, one should most prevalent strictureplasty techniques are Heineke-Mikulicz


strictureplasty [see Figure 3] and Finney strictureplasty [see Figure a
4].The former is best suited for strictures up to 5 to 7 cm long [see
Figure 5], the latter for strictures up to 10 to 15 cm long.The side-
to-side strictureplasty described by Michelassi21 is suitable for
longer areas of stricture; however, this technique involves longer
suture lines and is mainly considered for patients who already
have, or are at high risk for, short bowel syndrome.
The short, isolated strictures characteristic of diffuse jejunoileal
Crohn disease are more frequently described in patients with long-
b
standing Crohn disease. It has been postulated that over time,
Crohn disease progresses from an edematous condition to a more
fibrotic, stricturing condition.22 It is the fibrotic strictures charac-
teristic of the later stage of the disease that are amenable to treat-
ment with strictureplasty. Patients with these short fibrotic stric-
tures typically have obstructive symptoms and often are unable to
tolerate solid food, experiencing dramatic weight loss as a result.
Although strictureplasty leaves active disease in situ, it usually
leads to prompt resolution of obstructive symptoms, regaining of
lost body weight, and restoration of normal nutritional status.

c
a

b
Figure 4 Finney strictureplasty. (a) This procedure is suitable
for longer areas of stricture (up to 10 to 15 cm). (b) The strictured
bowel is bent into the shape of an inverted U. Stay sutures are
placed at the apex of the U, which is at the midpoint of the stric-
ture, and at the far ends, which lie 1 to 2 cm proximal and distal
to the stricture. A longitudinal enterotomy is made on the
antimesenteric border of the bowel with the electrocautery. A
side-to-side anastomosis is then performed, with the posterior
wall done first. (c) Shown is the completed anastomosis.

A significant concern with strictureplasty is the possibility that
small bowel adenocarcinoma may develop; several cases have been
c reported.23,24 I have treated a patient in whom a poorly differenti-
ated jejunal adenocarcinoma developed at the site of a stricture-
plasty that had been performed 10 years earlier. Accordingly, many
surgeons advocate routine biopsy of the active ulcer on the mesen-
teric side of the bowel at the time of strictureplasty [see Figure 6].
Another concern has to do with the number of strictureplasties
that can safely be performed in a single patient in the course of a
single operation. As many as 19 strictureplasties have been per-
formed during one procedure without increased morbidity.25
Figure 3 Heineke-Mikulicz strictureplasty. Stay sutures are Strictureplasty can be performed with either a single-layer or a
placed parallel to each other on the antimesenteric border of the
double-layer anastomosis. It should not be performed in the pres-
bowel over the area of the stricture. (a) The antimesenteric bor-
der of the bowel is then opened with the electrocautery over the
ence of an abscess, a phlegmon, or a fistula; and like any other
area of the stricture, and the opening is extended for approxi- anastomosis, it should not be performed proximal to an existing
mately 1 to 2 cm on either side of the stricture. (b, c) Traction is obstruction that is not treated at the time of operation.
placed on the stay sutures, and the original longitudinal enteroto- Areas of small bowel Crohn disease that are too long to be treat-
my is closed in a horizontal fashion in one or two layers. ed with strictureplasty can be treated with segmental resection.


erature to suggest that the postoperative recurrence rate may be low-
er with a wider anastomosis.27 The anastomosis can be performed in
either one or two layers. If the bowel is thicker, a handsewn anasto-
mosis is preferred to a stapled one.
The incidence of reoperation for recurrent disease after ileocol-
ic resection is high and increases with the number of resections.28
Postoperative chemoprophylaxis with mesalamine can significant-
ly reduce the recurrence rate.29 Patients who smoke should be
strongly encouraged to stop: the rate and severity of recurrence are
increased in smokers.20
Special Circumstances
Ileocolic Crohn disease is often associated with intra-abdominal
abscesses or fistulas. If an associated abscess is known to be pres-
ent, CT-guided drainage should be done preoperatively so that a
single-stage procedure can then be performed. If an unsuspected
abscess is identified at the time of operation, the safest approach is
to proceed with bowel resection, perform the posterior wall of the
Figure 5 Shown is a short fibrotic stricture that is ideally suited
anastomosis, and exteriorize the anastomosis as a loop ileostomy.
to treatment with Heineke-Mikulicz strictureplasty.
This loop ileostomy can then be safely closed, often without a for-
mal laparotomy, 8 weeks after operation if there are no signs of
The area to be resected should be as short as possible.There is no ongoing sepsis. If the abscess or the terminal ileal loop is adherent
need to obtain frozen-section margins to determine the extent of to the sigmoid colon, an ileosigmoid fistula may be present. The
resection; doing so leads to unnecessary loss of small bowel decision whether to resect the sigmoid colon is dictated by the
length.26 The resection should extend into palpably normal areas appearance and feel of the sigmoid in the involved areas. If only a
of small bowel. The easiest way of determining the area to be portion of the anterior colon wall is involved, that portion can be
resected is to feel the mesenteric margin of the bowel until palpa- excised in a wedgelike fashion and the excision site closed primar-
bly normal tissue is reached. Because Crohn disease is generally ily. If the entire circumference of the sigmoid colon at that point is
more severe on the mesenteric side of the bowel, palpation in this indurated and woody feeling, a short segmental resection with
area gives the most accurate impression of the intraluminal char- anastomosis is the best option.
acter of the bowel. Because it is not uncommon for patients to
have multifocal Crohn disease, the entire small bowel should al-
ways be inspected at the time of operation. Operating on one area
of disease while failing to treat a more proximal lesion is clearly not
in the patient’s interest.
Because of the high rate of recurrence in patients with isolated
small bowel disease, postoperative chemoprophylaxis should be
strongly considered. In these patients, I prefer to use a more potent
agent, such as an antimetabolite, rather than a 5-ASA agent.
ILEOCOLIC DISEASE

Approximately half of those diagnosed with Crohn disease have
ileocolic disease. Ileocolic resection is, in fact, the operation most
frequently performed to treat Crohn disease. Currently, there is a
trend toward more aggressive medical management of Crohn dis-
ease; at the same time, surgeons are seeing more complicated dis-
ease at the time of operation. These developments have implica-
tions for management. An easy ileocolic resection is an experience
that a patient generally tolerates well and recovers from very quick-
ly; however, delaying operative management with years of aggres-
sive medical therapy can lead to more complicated disease associ-
ated with enteroenteric fistulas, which can be difficult to treat.
Ileosigmoid fistulas are among the most common fistulas associ-
ated with ileocolic Crohn disease, along with fistulas between the
terminal ileum and the ascending colon and fistulas between the
terminal ileum and adjacent loops of small bowel.
Disease recurrence is common after ileocolic resection. Colonos-
copy is the most accurate modality for postoperative surveillance and
the easiest to use; it is more sensitive than either small bowel follow-
through or air-contrast barium enema. For this reason, I favor an
end-to-end anastomosis after ileocolic resection. In the event of re-
current disease, an end-to-side, side-to-end, or side-to-side anasto- Figure 6 A large ulcer is nearly always present on the
mosis may be difficult to intubate.There is some evidence in the lit- mesenteric luminal border of small bowel strictures.


COLONIC DISEASE colonic mucosa; such sloughing permits endotoxins to enter the
Colonic involvement is present in 29% to 44% of patients with circulatory system and evoke a septic response. The signs and
Crohn disease.30 One of the challenges in treating colonic Crohn symptoms of toxic megacolon include those characteristic of sep-
disease is obtaining the correct diagnosis. Whereas Crohn disease sis—leukocytosis, fever, tachycardia, and hypoalbuminemia.These
of the small bowel is fairly easy to diagnose, colonic disease often patients are very ill and often manifest ileus, which is an ominous
is not. Because granulomas are not present in most cases of development that frequently signals impending perforation. Emer-
colonic Crohn disease and because this condition can look very gency surgical intervention is required. At operation, the colon is
similar to ulcerative colitis both endoscopically and macroscopi- often distended, and when the specimen is opened, the colon may
cally, differentiation between Crohn colitis and ulcerative colitis appear almost autolytic [see Figure 9b]. In this state, the bowel fre-
can be difficult in the absence of small bowel or anal disease. quently does not hold staples well; accordingly, it is often helpful
Colonic Crohn disease appears to be more frequently associated to sew the distal Hartmann stump between the left and right
with cutaneous manifestations (e.g., pyoderma gangrenosum) [see halves of the anterior inferior rectus fascia at the lower abdominal
Figure 7]. incision and then to close the skin over it.31 Thus, if the staple line
is disrupted, the result is essentially a surgical site infection that
Indications for Surgical Treatment can be opened and drained, rather than the pelvic abscess [see
The main indications for operative management of colonic Figure 9c] that could develop if the rectal stump were located deep
Crohn disease are stricture [see Figure 8], malignancy, side effects within the pelvis.
of medical therapy, and failure of medical therapy. In children, fail-
Types of Disease
ure to recognize and treat this condition promptly may result in
growth retardation. It is important to monitor both bone age and Segmental disease In a 2003 review of 92 consecutive cases
insulinlike growth factor–1 levels. If these are abnormal, timely of patients with Crohn colitis, the number of patients with seg-
institution of human growth hormone therapy, operative manage- mental colonic Crohn disease and the number of those with pan-
ment of inflammatory bowel disease, or both may still permit nor- colonic disease were nearly equal.30 Approximately 63% of those
mal growth and development. with segmental colitis had other disease involvement as well (e.g.,
Side effects of medical therapy can be substantial. They may in- jejunoileal, ileocolic, or perianal), compared with only 12% of
clude such varied complications as aseptic necrosis of the femoral those with pancolitis. The recurrence rate, however, was higher in
head and cataract formation (both related to steroid use), as well as patients with segmental colitis than in those with pancolitis. In
an increased incidence of opportunistic infections (from immuno- addition, the risk of recurrence was higher in patients who had
suppression secondary to antimetabolite therapy). granulomatous disease than in those who did not.
Failure of medical therapy can refer to continuing severe disease
activity or, at worst, to so-called toxic megacolon. The term toxic Pancolonic disease In cases of pancolonic Crohn disease
megacolon is actually a misnomer, in that not all patients with this with associated perianal, jejunoileal, or ileocolic involvement, diag-
condition actually have a true megacolon [see Figure 9a]. In com- nosis is not difficult. However, most patients with Crohn pancoli-
mon usage, the term toxic megacolon refers to any condition asso- tis do not have other sites of disease involvement, nor do they have
ciated with colitis that is severe enough to result in sloughing of the granulomas.30 Consequently, differentiation of Crohn pancolitis

a b

Figure 7 (a) Shown is pyoderma gangrenosum affecting the peristomal and incisional area 6 months after
creation of a loop ileostomy in a 16-year-old girl who had undergone colectomy with IPAA for a presumed
initial diagnosis of ulcerative colitis. (b) Shown is peristomal gangrenosum of the breast in an otherwise
asymptomatic patient with Crohn colitis and perianal Crohn disease who had a diverting loop ileostomy.


a

b
Figure 8 Shown is a sigmoid colon stricture secondary to Crohn
disease that caused obstructing symptoms refractory to medical
therapy.

from ulcerative colitis can be very difﬁcult. Many patients with
Crohn disease have been inappropriately subjected to colectomy
with ileal pouch–anal anastomosis (IPAA) because they were ini-
tially presumed to have ulcerative colitis.
Operative Procedures
Total proctocolectomy with end ileostomy The tradition-
al procedure for colonic Crohn disease is total proctocolectomy
with end ileostomy, which is associated with an 8% to 15% rate of
recurrence in the bowel proximal to the stoma.32-34 This operation
remains the best choice in patients with severe rectal and anal c
Crohn disease (e.g., those with so-called watering-can perineum
[see Figure 10]) and carries the lowest risk of disease recurrence. In
contrast to the approach taken in patients with rectal cancer,
which involves excising the external anal sphincter and a large por-
tion of the levator muscles, the approach taken in those with
colonic Crohn disease is intersphincteric, with dissection per-
formed in the plane between the internal and external anal
sphincters to reduce the size of the perineal wound and facilitate
healing. Even with the intersphincteric approach, delayed healing
of the perineal wound is common, occurring in as many as 30%
of patients.

Subtotal colectomy with ileorectal or ileosigmoid anas-
tomosis Because many patients with Crohn disease are young,
surgeons have long been interested in operations that do not
involve an ileostomy. In the absence of signiﬁcant rectal and anal
disease, subtotal colectomy with ileorectal or ileosigmoid anasto-
mosis is an option. Unfortunately, this operation is associated with
high recurrence rates (up to 70%)35; however, with the advent of
more effective immunosuppressive and biologic therapy, it is hoped
that these rates can be reduced. As much palpably normal distal
rectum and colon as possible should be spared. The anastomosis Figure 9 (a) Shown is toxic megacolon in a 17-year-old girl with
Crohn colitis. The colon is massively distended and near perforation
can be stapled, though if the bowel wall is thickened, many sur-
at the time of operation. (b) When the specimen is opened, it is
geons would feel more secure with a handsewn anastomosis in apparent that large segments of the mucosa have sloughed off, leav-
either one or two layers. ing denuded muscle wall. (c) The distal rectosigmoid is incorporated
between the left and right halves of the anterior inferior rectus fas-
Segmental resection Currently, more surgeons are advo- cia at the lower abdominal incision and placed underneath the skin,
cating colon-sparing procedures [see 5:34 Segmental Colon Resection] which is then closed over it. Thus, in the event of disruption of the
for Crohn disease. Although this is a relatively new approach, distal stump, the contents drain harmlessly through the wound.


would knowingly perform in a patient with Crohn disease, every
year there are many such patients who undergo this procedure as
treatment of colonic inflammatory bowel disease that initially is
incorrectly presumed to be ulcerative colitis but later is diag-
nosed as Crohn disease (on the basis of either final pathologic
analysis of the resected specimen or the disease’s clinical behav-
ior). Generally speaking, in the absence of fistulizing disease,
most of these patients are able to maintain their pouch, but they
require medical therapy for disease control.30,37-40
ANAL DISEASE

Types of Disease
With stenosis For patients with anal strictures that are not reg-
ularly dilated, the outlook is poor. Such strictures pose functional ob-
structions and typically lead to continuing problems with fistulas and
suppurative disease.They frequently become more and more fibrot-
ic over time and often extend proximally. Most of these patients
eventually require fecal diversion. Management generally involves
Figure 10 Shown is so-called watering-can perineum secondary self-dilation, which can often be done with Hegar dilators. If the
to severe perianal Crohn disease. stenosis is not dealt with, all other treatment of the Crohn disease is
doomed to failure; obstruction at the level of the anal canal inevitably
results in the persistence of anorectal disease.
there have already been some reports documenting the safety of
segmental resection in cases of limited disease.36 In patients with Without stenosis Anal Crohn disease without stenosis is
colonic strictures resulting in obstruction, segmental resection into much easier to treat medically. Long-term oral metronidazole ther-
palpably normal areas of the bowel yields prompt resolution of apy is often helpful; other medications (e.g., anti–tumor necrosis
symptoms. Because the colon performs an important water-absorb- factor antibody) may be useful as well. Broad fissures are usually
ing function, many patients with a limited amount of small bowel asymptomatic. Surgical treatment should be avoided unless the
can still live without intravenous supplementation if a significant lesions are causing symptoms. Because they tend to have more liq-
segment of the colon is left in situ. However, patients with seg- uid bowel movements, patients with Crohn disease need an opti-
mental Crohn disease appear to have a higher recurrence rate than mally functioning anal sphincter; hence, fistulotomies, which
those with pancolitis, as do patients with granulomas.30 Surgical divide portions of the sphincter, should be avoided if at all possi-
treatment of Crohn disease continues to undergo reevaluation and ble. Placement of setons through fistula tracts can often prevent
reassessment of results on the basis of the availability of newer abscess formation, provide drainage, and thereby prevent perianal
medical therapies. pain while minimizing sphincter trauma. Silk sutures, vessel loops,
or Penrose drains also can be used as setons [see Figure 11]. Recto-
Colectomy with IPAA Although colectomy with IPAA [see
vaginal fistulas pose a particular challenge. In the presence of
5:33 Procedures for Ulcerative Colitis] is not an operation that one
active Crohn disease, advancement flap repair of such fistulas has
a low success rate.41 Laparoscopic-assisted loop ileostomy improves
the success rate, but unfortunately, the fistulas may recur when
intestinal continuity is reestablished.

Postoperative Management

CHEMOPROPHYLAXIS

In 1995, a prospective, randomized study showed that patients
who underwent ileocolic resection and were given mesalamine
postoperatively had a significant reduction in both the symptom-
atic and the endoscopic rate of recurrence.29 Not all of the work
done since then has confirmed these results, but several studies
and a meta-analysis have indicated that mesalamine does reduce
the postoperative recurrence rate of Crohn disease.42 Many pa-
tients undergoing surgical treatment of Crohn disease are ad-
vised to take some type of postoperative preventive medical ther-
apy—either a 5-ASA derivative (e.g., mesalamine) or a stronger
immunosuppressive agent (e.g., 6-mercaptopurine or azathio-
prine). Better studies are required to document the efficacy of
Figure 11 Vessel loops can be used as setons for drainage of the latter agents in preventing recurrence. It is hoped that
abscesses caused by perianal Crohn disease. They can be left in as chemoprophylaxis will reduce the anticipated recurrence rates
long as necessary and help prevent recurrent abscess formation. by 30% to 40%.


SURVEILLANCE BEHAVIORAL MODIFICATION

At present, there are no clear guidelines for surveillance after Exposure to cigarette smoke is known to exacerbate the symp-
operative treatment of Crohn disease. In my opinion, however, toms of Crohn disease. Smoking has been reported to affect the
given the increased risk of colorectal cancer in this setting, overall severity of the disease, with smokers having a 34% higher
patients with Crohn disease who retain some colon should recurrence rate and a higher rate of reoperation than nonsmok-
undergo colonoscopy every 2 years, not only to detect any ers.43-45 A 1999 study of 141 Crohn disease patients who had
development of colonic neoplasia but also to identify any recur- undergone ileocolic resection, of whom 79 were nonsmokers and
rence of disease in a timely manner. If recurrent Crohn disease the remainder were smokers, found that the respective 5- and 10-
is detected, appropriate medical therapy should be promptly year recurrence-free rates were 65% and 45% in smokers and 81%
instituted, with the aim of avoiding subsequent operation if and 64% in nonsmokers. The recurrence rates were higher in
possible. heavy smokers (≥ 15 cigarettes/day) than in moderate smokers.46

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