crohn`s disease

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ELIGIENDO LA MEJOR OPERACIÓN PARA
COLITIS ULCEROSA Y CROHN
Dr. John Pemberton
INTRODUCTION
Surgery for inflammatory bowel disease is evolving. The overarching strategy
in the surgery for Crohn’s disease is to conserve the bowel, especially as new
drug therapy such as Remicade®, an anti-TNF antibody, is coming on-line.
Moreover, the management of anal Crohn’s is evolving toward an aggressive
combined multimodality treatment approach with the surgeon draining fistulas
and abscesses and the gastroenterologists administering Imuran in an attempt
to “dry up” the perineal disease. In patients with chronic ulcerative colitis, an
ileal pouch-anal anastomosis has become the treatment of choice in those
patients requiring a proctectomy for the disease.
This syllabus will describe some of the surgical indications for different types of
presentations of Crohn’s disease and in turn Mayo’s experience with ileal
pouch-anal anastomosis since 1981.
CROHN’S DISEASE
The proper prospective for any discussion of surgery for Crohn’s disease is
maintained by remembering that the disease cannot be cured by surgery.
When to be aggressive (be removing disease, the surgeon restores health and
decreases doses of medications) and when to be conservative (the surgeon
removes bowel, causes nutritional debility as well as sometimes creating the
need for a stoma) are central issues. The aim of surgery should be to deal with
the patient’s presenting problem as simply as possible by conserving bowel,
while striving to improve the quality of life.
Indications
Indications for operation in patients with Crohn’s disease are rarely absolute
ones, except when free air, an abscess or uncontrolled bleeding is present.
Obstruction
and
fistulas
(entero-entero,
enetercolic,
enterovesicle,
enterocutaneous) are the most common indications for surgery. In addition,
severe perianal disease often prompts proctectomy.
Surgery for Gastroduodenal Crohn’s Disease
Gastroenterostomy and truncal vagotomy are the treatment of choice for
patients with intractable gastroduodenal Crohn’s disease. Gastroenterostomy
alone results in ulcerations at the anastomosis (or is it perhaps recurrent
Crohn’s disease)?1 Alternatively, a short but severe stricture in “burned out”
Crohn’s of the duodenum could be treated by strictureplasty.2
Small Bowel Crohn’s Disease
There are two interesting debates in surgery for small bowel Crohn’s; the
importance of microscopically free margins and resection versus
strictureplasty. Pennington and colleagues3 examined the margins of resection
in patients with small bowel Crohn’s disease; they found no correlation between
the presence of inflammation and recurrence of disease. Wolff and
colleauges4, however, found a significantly higher rate of recurrence of Crohn’s
disease if the margins of resections were involved by inflammation compared to
historic controls (control 45% versus involved margin 90%).
In the past, it was recommended that at least 10 cm of macroscopically and
microscopically free margins be achieved on either side of Crohn’s disease.5
However, other authors found no beneficial affect on recurrence by resecting
increased amounts of normal bowel proximal to the uninvolved segment.6
Indeed a recent study found that it seemed entirely unnecessary to resect to
microscopically free margins.2 There were some problems in this study, not the
least of which was the criteria for involvement of the margin. In Pennington’s
study,6 margin involvement was defined as the presence of fibrosis, edema, or
increased lymphocyte or plasma cell infiltration.
In our study,7 tissue destruction actually had to be present. We found that
patients with microscopic evidence of residual disease had a 75% chance of
developing recurrent Crohn’s disease.7 In general, the risk for recurrence of
Crohn’s disease at Mayo is 15% at two years, 40% at five years, and 55% at
ten years. There were also some problems with this study, the largest of which
was the lack of a control group.
A good rule to follow is this: in a previously unoperated patient with active small
bowel Crohn’s disease, a margin of 3-5 cm should be sought proximally and
distally. If the margins are involved by Crohn’s--specific inflammation (mucosal
infiltration by inflammatory cells or ulceration) then an additional 3-5 cm margin
should be sought. If the margin is still positive, then the anastomosis should be
performed without further resection. In a patient previously operated upon, a
margin of 2 cm should be sought. If positive, a further 2 cm should be resected
and no more.
Strictureplasty
Introduced by Lee and Papaionnou8 and popularized by Alexander-Williams2,
and others9 strictureplasty in selected patients with multiple areas of
involvement by Crohn’s disease throughout the small bowel (skip lesions) is a
valuable addition to the Crohn’s surgeon’s armamentarium. The secret of this
procedure, one that flies in the face of conventional surgical wisdom, is that it is
performed in segments of bowel involved by “burned out” disease. It is an
elegantly simple but bold solution to the problem of conserving bowel; a simple
longitudinal incision through the stricture with transverse, single layer closure
(similar to the Heineke-Mikulicz pyloroplasty) is all that is done. Experienced
authors have reported good to excellent results; there is surprisingly little
morbidity from the procedure.2, 8, 9 The perfect patient for this approach is one
who has symptoms of chronic obstruction and who has multiple skip areas of
fibrotic disease.
Whether to bypass diseased segments of small bowel is an argument that is
passé. This operation is reserved for especially difficult situations such as
ileocecal disease with a densely adherent retroperitoneal abscess. Otherwise,
bypass is not useful in the management of Crohn’s disease.
COLONIC CROHN’S DISEASE
Segmental Resection of the Colon
Segmental resection is performed in patients with ileocecal Crohn’s
(ileoascending or transverse colostomy) and is the treatment of choice. In
general, however, segmental resection of colonic Crohn’s disease is associated
with high rates of recurrence. The best rule to follow is to resect all colon
proximal to the site of disease. The scenario most appropriate for segmental
resection, however, is the one where there is ileocolonic disease and isolated
sigmoid involvement; in this situation, ileocolostomy and segmental resection of
the sigmoid colon should be performed.
Abdominal Colectomy and Ileorectostomy
There is no question that for patients with colonic Crohn’s disease, but in whom
the rectum is spared, colectomy and ileorectostomy is the procedure of
choice.10, 11
Proctocolectomy and Ileostomy
If the rectum is not spared and/or if the patient has severe anal involvement by
Crohn’s disease, then proctocolectomy and ileostomy is the procedure of
choice. The mortality rate is less than 2%.12 This operation has major impact,
of course, on life-style; the patients are incontinent and must wear a stomal
appliance at all times. However, patients are relieved of symptoms and of
having to take heavy doses of steroids; in general, they recover quickly and do
very well. The biggest problem after proctocolectomy for Crohn’s disease is a
non-healing perineal wound which occurs in up to 1/3 of the patients.13
Anorectal Crohn’s
Involvement of the anorectum by Crohn’s will be the initial manifestations of
disease in about 7% of patients.14 The spectrum of involvement is vast, from a
single healed secondary site to a destroyed anal canal. Fistula (simple or
complex) and fissures (which are often painless but are characteristically huge
and have overhanging skin edges) often smolder, controlled by metronidazole
and sometimes other anti-inflammatory medications. Although proximal
diversion by a sigmoid colostomy will mitigate anal disease, as soon as bowel
continuity is restored, the disease recurs.15 Occasionally, a simple rectalvaginal fistula may be repaired successfully provided the rectum and anal canal
are free of granularity, edema, and ulceration. Patients undergoing this
procedure should be diverted.
Regarding the influence of proximal disease on anorectal Crohn’s disease, we
looked at a group of 86 patients who had anorectal Crohn’s disease to
determine the proctectomy rate and the effect of proximal disease on anorectal
function.16 The follow-up was about ten years. We found that the probability of
avoiding proctectomy in patients who did not have proximal disease at the time
of initial treatment of their anorectal Crohn’s disease was significantly higher
than those for patients who had proximal disease (97% versus 69% of twenty
years, p<0.002).
Our recommendations therefore for patients with active anorectal Crohn’s
disease are as follows. Certainly a conservative approach to anorectal Crohn’s
disease is warranted. This means unroofing of abscesses and adequate
drainage, as well as dilatation of anal stenosis. Painful fistulas and fissures
should be converted to painless or asymptomatic ones either by conservative
lateral internal sphincterotomy or low fistulotomy. About 85% of all patients with
complicated anorectal Crohn’s disease can be relieved of their symptoms.
Moreover, metronidazole treats perianal Crohn’s disease quite effectively.
Hellers et al. reported that patients treated surgically for anal fistula had a much
better healing rate if the proximal bowel was not involved with active
disease.17 Moreover, Hellers17 suggested that anorectal Crohn’s disease
should only be treated if the proximal disease has been controlled by medical or
surgical management. Buchmann et al.18 and Marks et al.19 have found that
the resection of the proximal bowel did not improve healing. McIlrath20 showed
that proximal diversion, either by ileostomy or colostomy, was not beneficial to
anorectal Crohn’s disease. Interestingly, we found that there was an early
benefit in the healing of anorectal Crohn’s disease in those patients in whom all
active disease was removed. However, there was no long-term benefit to the
involved anorectum from resection of proximal Crohn’s disease; if active
disease remained proximally or recurred, anorectal Crohn’s also persisted or
recurred.
Finally, we have been aggressively managing anorectal Crohn’s disease with a
multi-modality approved: FK506, Imuran and prednisone, surgical draining of
abscess and control of fistula seems promising and is based on several recent
reports.21, 22
Recurrence
Recurrence rates in patients with Crohn’s are extraordinarily high--so high, in
fact, that recurrence may not be the right word--rather, recrudescence of
disease may be a better term. Major factors which influence recurrence rates
include: i) age, ii) site of initial disease, iii) previous disease and iv) positive
margins.
A randomized prospective trial was performed to determine if 5 ASA (3.0
gm/day) is effective in decreasing the risk of recurrence of Crohn’s disease. In
brief, the recurrence rate among patients in the 5 ASA group was 31% while in
controls, it was 41% (p=0.03).23
Comment
The whole gastrointestinal tract from mouth to anus is at risk for developing the
sequela of Crohn’s disease. Moreover, because the disease is not cured by
surgery, recurs predictably and operations sometimes result in an ileostomy and
loss of the anus, surgery should be conservative. On the other hand, symptoms
are relieved and patients return to active lives. Importantly, patients with
Crohn’s do not relate their quality of life whether or not an operation was
required in the past, but rather to the activity of the disease.24
Summary and Future
The FDA has approved the drug Remicade® for the treatment of severe
Crohn’s disease. Remicade® is an anti-TNF antibody and will be available in
October 1998. The efficacy of anti-TNF antibody treatment in Crohn’s disease
in large populations is unknown and we all await its widespread application with
anticipation.
SURGERY FOR CHRONIC ULCERATIVE COLITIS
EXPERIENCE WITH ILEAL POUCH ANAL ANASTOMOSIS
THE
MAYO
From January 1, 1981, to September 14, 1998, 1,990 IPAA operations have
been performed at Mayo. One thousand two hundred and eighteen patients
were operated upon between January 1, 1981, and September 15, 1998 from
the data base for this syllabus. The standard operation consisted of abdominal
colectomy, proximal proctectomy, distal endorectal mucosal resection to the
dentate line, construction of a J-ileal pouch and a pouch anal anastomosis. In
addition, since 1994, double-stapled IPAA has also been performed. Most
patients underwent a diverting ileostomy which was closed two to three months
later. The ratio of men to women was 1:1 and the mean age of the group was
32 years.
Very Early Clinical Results
Ninety-six percent of patients had a protecting ileostomy established at the first
operation; of these, 97% were loop ileostomies.
On dismissal after ileostomy closure, 79% of patients were "completely
continent," 19% were "mostly continent" (occasional daytime and nighttime
soilage), and 2% were incontinent. Ninety-four percent of the patients had 10 or
less stools per day and 96% had two or fewer stools at night within 10 days of
ileostomy closure. Stool bulkers were given to 47% of the patients on
dismissal, and agents to slow transit were prescribed for 49%.
Mortality
Two patients have died postoperatively.
Morbidity
Overall, 30% of patients had a postoperative complication. Most complications
occurred in the early postoperative period and did not result in loss of the pouch
or long-term disability.
Pelvic infection was a serious complication occurring in the early postoperative
period in <5% of patients with ulcerative colitis. CT scan was useful for
demonstrating a pelvic fluid collection or phlegmon. Patients with a pelvic
phlegmon usually responded to conservative treatment with broad-spectrum
antibiotics and bowel rest. Patients with a pelvic abscess underwent CT-guided
drainage, if technically feasible, or laparotomy and drainage. The incidence of
pelvic sepsis is declining due to increased experience with the procedure, and
the construction of a shorter rectal muscular cuff.
The incidence of abdominal sepsis was 6%25 and was an ominous
development. Ultimately, 41% of patients who underwent laparotomy for control
of this sepsis required pouch excision. Moreover, normal function was achieved
in only 29% of patients requiring operation. However, among septic patients
who did not require reoperation, but rather aggressive non-surgical
management, 92% had satisfactory pouch function over the long term.
Small bowel obstruction occurred in 17% of the patients, 8% of whom required
surgical intervention.26
Closure of temporary ileostomies was also associated with complications.
Peritonitis occurred in 4% and postoperative obstruction in 12% of patients.
Proximal and distal serosal tears during stoma mobilization, in addition to
anastomotic leaks, are important causes of peritonitis. If all extraperitoneal
bowel (afferent and efferent limbs and the stoma itself) are resected, however,
the chance of leaving an unrecognized perforation is nearly eliminated.
Nearly all patients had a web-like stricture of the ileoanal anastomosis on
returning for ileostomy closure. This stricture was dilated digitally without
difficulty. If the pouch retracts under anastomotic tension, heavy scarring and a
long, fibrotic stricture results. This type of stricture is manifested by increased
difficulty with straining at stool, a sensation of incomplete pouch evacuation, or
a high stool frequency (>10-12 stools/day). Repeated anal dilatation may
prevent progression of the stricture.27
Late Clinical Results
The average stool frequency during the day was six stools with one stool at
night. Importantly, daytime and nocturnal stool frequency and the ability to
discriminate flatus from stool has remained stable over time while the need for
stool bulkers and hypomotility agents has declined. This has been confirmed by
others.28 Several large series have reported similar stool frequencies.29, 30,
31, 32 The lower stool frequencies six months after the operation compared to
early postoperatively are likely due to increased pouch capacity with time.33, 34
Major fecal incontinence (more than twice per week) occurred in 5% of patients
during the day and 12% during sleep.35 In contrast, minor episodes of
nocturnal incontinence were found in 20 to 30% of patients at least one year
after the operation. A pad was worn by 28% of patients for protection against
seepage. Interestingly, up to 60% of fully continent women wear a pad for fear
of accidental fecal soilage. Minor perianal skin irritation was reported by 63% of
patients.
Not surprisingly, patients over 50 years had a higher daytime stool frequency
than patients under 50 years (<50 years: 6+3 stools/day vs. >50 years: 8+4
stools/day). Men and women had similar stool frequencies postoperatively but
women had more episodes of fecal soilage during the day and night.
Late Complications
Pouchitis: The principle late complication after IPAA is non-specific reservoir
inflammation or "pouchitis". The incidence of pouchitis has increased as followup has lengthened.36 At Mayo, the incidence of pouchitis among patients with
chronic ulcerative colitis was 31%.36 The mean interval from operation to first
occurrence was 17 months. A second episode was identified in 61% of
patients. The incidence was not affected by the type of pouch constructed,
presence or absence of perioperative pelvic sepsis or backwash ileitis, or the
age or sex of the patient. Higher recurrence rates occurred in patients with
extraintestinal manifestations of ulcerative colitis (39%) than in those without
manifestations (26%).36 Furthermore, exacerbations of extraintestinal
manifestations were often temporarily related to flares of pouchitis.
Pouchitis is characterized by the spontaneous onset of watery, often bloody
diarrhea, increased stool frequency, fecal urgency and soilage, abdominal
discomfort, malaise and fever. The etiology of pouchitis is unknown but causes
include disorders of pouch motility, bacterial overgrowth and by-product
accumulation from intra-luminal bacterial colonization, pouch ischemia and
reperfusion injury, or a de novo type of inflammatory bowel disease. To date,
no distinguishing absorptive, histological, or emptying abnormality has been
identified in patients with recurrent pouchitis.37 Pouchoscopy often
demonstrates mucosal friability, edema or ulceration, but endoscopic findings
fail to consistently correlate with clinical presentation.38 We have developed
recently a pouchitis disease activity index (PDAI) which, because it uses
symptoms together with endoscopic and histologic findings to arrive at an
objective score, may be a better diagnostic tool than any single parameter
alone.39
Traditional treatment of pouchitis has been oral antibiotics directed against
anaerobic bacteria with prompt response usually seen within 24 to 48 hours of
starting therapy. Those patients refractory to antibiotics may respond to
sulphasalazine and/or steroid enemas. In addition, there is recent evidence that
Bismuth Subsalicylate (Pepto Bismol) is efficacious in the treatment of antibiotic
resistant pouchitis.40 In approximately 10% of pouchitis patients, chronic
pouchitis develops requiring low-dose prophylactic oral antibiotics or enema
administration. Ultimately, a small number of pouchitis patients require either
pouch revision or excision.
Sexual Dysfunction
Impotency and retrograde ejaculation developed in 1.5% and 4% of men,
respectively (11). Dyspareunia developed in 7% of women postoperatively.
Interestingly, fully 49% of women noted sexual dysfunction preoperatively yet
sexual activity increased dramatically after IPAA due to an improvement in
general health.41
Failure
At Mayo, 7% of patients ultimately required pouch excision or construction of a
permanent ileostomy. Other large series have reported failure rates between 2
and 12%.29, 30, 31, 32 The most frequent causes of failure, either alone or in
combination, included pelvic sepsis, high stool volumes, Crohn's disease, and
uncontrollable fecal incontinence.35 Pouchitis was the sole cause in 2% of all
patients who failed. Importantly, of the patients who failed, 75% failed within
one year, 12% by two years, and 12% by three years. Thus, failure after IPAA
is manifested within several years after the operation and is the result of a
combination of early or late complications of the procedure.
Quality of Life
Issues of quality of life are often the deciding factor for patients choosing a
particular operation for ulcerative colitis. Several studies which analyzed the
outcome of surgery for ulcerative colitis have demonstrated that most patients
are satisfied with the operation and lead a normal lifestyle regardless of the
procedure.43, 44
The quality of life following Brooke ileostomy and IPAA for ulcerative colitis and
familial adenomatous polyposis studied by us.45 Patients were highly satisfied
following either operation (93% of Brooke ileostomy, 95% IPAA). However,
daily activities (sexual life, sports, social, work, recreation, family relationships,
travel) were more likely to be adversely affected with a Brooke ileostomy than
IPAA.
CONTROVERSIES
Which pouch design is best?
Of all the pouch designs championed in the literature ("S", "H", "W", "K" & "J"),
the "J"-shaped pouch is easiest to construct and has identical functional
outcomes as the more complex designs. It is thus the pouch design of choice
at Mayo.
TWO STAGES OR ONE?
The Role of a Defunctioning Ileostomy
The most feared complication of IPAA is pelvic sepsis, and therefore, a
defunctioning ileostomy after pouch construction is usually performed to
minimize its occurrence. Whereas pelvic sepsis complicates 6% of patients
undergoing IPAA at the Mayo Clinic1, the rates reported in the literature vary
between 0% and fully 25%. Moreover, disturbingly high rates of pelvic sepsis
have been reported in patients undergoing a one-stage procedure (no
ileostomy).46
Although the incidence of pelvic sepsis is low, when it occurs it is responsible
for a significant number of the failed pouches in the Mayo series.
Protagonists of defunctioning ileostomies also argue that diverting stomas allow
the anal sphincter and ileal mucosa to recover before restoration of intestinal
continuity, and that patients have a short-lived experience of a stoma to fully
appreciate the ultimate benefit of IPAA.47
Use of loop ileostomy, however, does not appear to fully protect the patient from
pelvic sepsis.48 It is nonetheless much easier to manage a patient with sepsis
if an ileostomy is in place.48 Of Mayo patients who require laparotomy to
control sepsis, 41% lost the pouch ultimately and only 29% ever recovered
ileoanal function. However, if no reoperation was required, 92% of patients with
sepsis eventually had a functioning pouch.48 This is an important observation.
There is little doubt that by avoiding an ileostomy altogether, complications of
closure are not an issue, and only one hospital admission is required. Low
complication rates with good functional outcome have previously been reported
with such an approach.49, 50 At Mayo, the clinical and functional outcomes of
37 patients who had a one-stage IPAA without a defunctioning ileostomy have
previously been reported.48 The group was age- and sex-matched with 37
patients who had a defunctioning ileostomy during the same period. The
incidence of complications was higher when a defunctioning ileostomy during
the same period. The incidence of complications was higher when a
defunctioning stoma was not used (22% versus 11%, respectively). One-stage
IPAA was shown to be a reasonable option if patients were in good health, were
not on chronic steroid therapy and if the IPAA could be constructed without
tension or technical difficulty.
A trial of 45 chronic ulcerative colitis patients randomized to defunctioning
stoma (n=23) or no stoma (n=22) showed no differences in rates of
complications between the groups.51 The patient group was highly selected,
however, in that no patients were taking steroids. Although the authors found
no increased risk of pelvic sepsis in patients without an ileostomy, the study
was notable for the very high rate of ileostomy-related complications (52%).
A reasonable approach to this dilemma is to use a defunctioning ileostomy in
those patients receiving steroid treatment at the time of surgery and in patients
who are nutritionally compromised or undergoing an urgent operation.
Additionally, if there are concerns about pouch blood supply or anastomotic
tension, a diverting stoma is almost mandatory. Using these criteria, 56 of
1,800 patients undergoing IPAA have had a one-stage procedure performed at
the Mayo Clinic between 1980 and 1996.
Double-Stapled Versus Handsewn Anastomosis
Much of the debate whether to staple the anastomosis or not has evolved
because functional outcomes should be improved if the anal transition zone
(ATZ) is preserved. Does preserving the ATZ enhance continence after the
IPAA? Stapled anastomosis in nonrandomized trials has been equated with
better outcome, which in turn is attributed to less injury to the anal sphincters,
with preservation of the ATZ and hence anal sensory discrimination, and with
preservation of the rectoanal inhibitory reflex.52, 53, 54 However, one
randomized trial by Seow-Cheon et al.54 comparing endoanal mucosectomy
and handsewn anastomosis with stapled anastomosis did not show any
difference in functional outcome between the two methods.
We recently reported upon a randomized study of our own. Forty-one patients
at the Mayo Clinic have been randomized to double-stapled (17 patients) or
handsewn (15 patients) techniques.55 In the stapled group, 1.5 to 2.0 cm of
ATZ was preserved, whereas a complete mucosectomy was performed in the
hand-sewn group. Overall complications were the same between the groups.
Stool frequency, rates of fecal incontinence during the day and night were
similar between the groups. However, fewer double-stapled patients had
incontinence at night. Moreover, resting and squeeze pressures were better
preserved after double stapling. We concluded that both handsewn and
double-stapled IPAA improved the quality of life dramatically. Double stapled
IPAA had further benefits because it may better preserve the anal canal than
does the handsewn anastomosis, and thus, enable older and perhaps
overweight patients to be candidates for IPAA for the first time.
SUMMARY
The length of ileum used rather than pouch configuration per se is related to
eventual functional outcome: A pouch constructed from 15 to 20 cm limbs is
ideal. One-stage procedures are reasonable in those patients not malnourished
or taking steroids and in whom a tension-free anastomosis is constructed.
Because most of our patients do not satisfy these criteria, single-stage IPAA is
rarely used at Mayo. The decision to excise the ATZ should relate to the risk of
developing subsequent neoplasia. Patients who do not have a mucosectomy
should have life-long surveillance. Indeed, an argument can be made that all
patients should undergo surveillance after IPAA. The decision to staple the
anastomosis impacts little on eventual functional outcome but does preserve
the ATZ with the attendant risk of recurrent disease, polyps, and neoplasia.
Ileal pouch-anal anastomosis cures chronic ulcerative colitis with acceptable
morbidity and mortality. Following the operation, the great majority of patients
achieve satisfactory continence with an excellent quality of life. However,
continence is not perfect and fecal soilage is a troublesome problem for a small
number of patients. Moreover, up to one-third of patients develop pouchitis for
which an effective means of long-term prevention or treatment has yet to be
developed. Finally, controversial issues such as optimal pouch design or
technique of anastomosis will only be resolved when long-term follow-ups of
prospective randomized trials have been completed.
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