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66

Methods

A search of all English language PubMed articles from 1990 to 2015 was performed
using the following terms: Crohn’s disease, Crohn’s colitis, ileal pouch anal anasto-
mosis, restorative proctectomy, restorative proctocolectomy, completion proctec-
tomy, proctocolectomy and ileostomy. These terms were in keeping with the PICO
table below on which this chapter is based. All relevant articles were reviewed and
appropriate references interrogated.
Patient
Population Intervention Comparator
Outcomes
studied
Crohn’s
colitis
Ileal pouch anal
anastomosis
(IPAA)
Proctocolectomy/completion
proctectomy with end ileostomy
Pouch morbidity;
pouch excision;
quality of life

Results

There is a clear division in the literature regarding the outcomes of IPAA in CD in
terms of the time of diagnosis of the primary disease. Studies divide the timing of
the CD diagnosis as preoperative (resulting in an ‘intentional’ IPAA formation),
perioperative (IPAA formation with ‘incidental’ or ‘accidental’ CD diagnosis on
analysis of the surgical specimen) or at a later date following IPAA creation (so
called ‘delayed’ diagnosis). A comparison of the data for these three distinct groups
has been reported in prior studies. However, the ensuing recommendations and
debate are based solely on those studies pertaining to patients with a documented
diagnosis or high clinical suspicion of CD prior to undergoing IPAA, the aforemen-
tioned ‘intentional’ IPAA cohort.
The first published paper of ‘intentional’ IPAA formation in CD (patients in
whom there was a high clinical suspicion based on the findings described below)
was from Hyman and colleagues from the Cleveland Clinic in 1991 [1]. They
reported on 25 patients with a postoperative pathologic diagnosis of CD out of 362
consecutive patients undergoing IPAA for a preoperative diagnosis of ulcerative
colitis (UC). Of these 25 patients, 9 had preoperative features suggestive of CD: 5
with perianal disease (fistula, fissure or stricture), 2 with abnormal distribution of
colonic disease, 1 with a cecal stricture and possible terminal ileal disease and 1
with a rectovaginal fistula. Although none of these 9 patients had a definitive preop-
erative diagnosis of CD, the above pathology would frequently be cited as a reason
not to perform IPAA in cases with indeterminate pathology. At a mean follow-up of
34.8 months, only 1 of the 9 patients had a functioning pouch. Of the remainder, 1
died, 1 remained diverted and 6 had their pouch excised at a mean of 17.6 months
postoperatively. The authors concluded that patients who manifest clinically as CD
and have confirmatory pathology do very poorly following IPAA with short disease-
free intervals and a high pouch failure rate.
C. Peirce and F.H. Remzi
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67
Following this, Panis and colleagues published their initial results [2]. From
1985 onwards, they considered IPAA in selected CD patients in whom a proctec-
tomy was required for either proctitis or rectal stenosis. Strict inclusion criteria were
employed to ensure the disease was confined solely to the colorectum: all patients
underwent an examination under anesthesia prior to IPAA to exclude anoperineal
disease and also had a small bowel contrast study to exclude concurrent enteric
disease. Eighteen patients were recruited over an initial 7-year period. These 18
patients were combined with a further 13 patients with a pre-IPAA diagnosis of
indeterminate colitis (IC) which was subsequently shown to be CD in the postopera-
tive specimen. This group then totaled 31 patients and reported outcomes were for
the group as a whole (i.e., n = 31) and were not subdivided into the specific diagnos-
tic timeframes of pre-operative (n = 18) or post-operative (n = 13) CD diagnosis. The
results were encouraging: 6 patients had a CD-related complication with 2 of these
ultimately requiring pouch excision and the remaining 4 patients reporting accept-
able pouch function. Overall, 90 % of the cohort had a functional pouch at 5-year
follow up. When compared with a corresponding ulcerative colitis (UC) cohort
(n = 71) over the same time period, there was no demonstrable difference in terms of
stool frequency, continence, gas/stool discrimination, leak or need for protective
pads and sexual activity.
The same group subsequently reported on their experience with 41 patients, 26
of whom had a preoperative CD diagnosis [3]. Once again, the results in terms of
CD-related complications are reported for the whole group and not reported in sub-
group analysis for the intentional IPAA patients and incidentally diagnosed CD
patients following IPAA. Twenty patients were followed for 10 years or more with
a CD-related complication rate of 35 % and an impressive pouch excision rate of
only 10 %.
The Cleveland Clinic adopted the intentional IPAA in CD patients in the late
1990’s and subsequently reported its initial experience [4]. The analysis included 20
patients who underwent an intentional IPAA out of the study cohort of 204 patients
(additional 97 patients with incidental diagnosis and 87 patients with delayed diag-
nosis). These 20 patients had a median time of 6.6 years from CD diagnosis to IPAA
with a median follow up of 5 years and were more likely to be female. The 10- year
pouch retention rate in the 20 patient strong intentional group was 85 % and thus
closely mirrored the long-term follow up reported by Regimbeau and colleagues of
90 % pouch retention at 10 years as described above. For those patients with retained
IPAA, 72 % reported near-perfect or perfect continence, 68 % reported rare or no
fecal urgency and the median number of daily bowel movements was 7 (range 2 –
20). Interestingly, these patients also reported their quality of life and quality of
health as 9/10 and 9/10 respectively and happiness with the IPAA procedure as
10/10.
The Mount Sinai Medical Center, New York, reported their experience with 13
patients who received an IPAA, 4 of whom were definitively diagnosed preopera-
tively with CD [5]. None of these patients had perianal disease and all had disease
solely limited to the colon. Two of these 4 patients (50 %) subsequently developed
perianal disease, 2 (50 %) developed postoperative complications and 1 patient
8 Crohn’s Colitis and Ileal Pouch Anal Anastomosis
68
(25 %) required a pouch excision. Of note, the outcomes for all 13 CD patients were
compared with a matched cohort of patients undergoing IPAA for chronic UC; the
CD patients had fewer bowel movements per 24 h, a lower incidence of inconti-
nence and a lower incidence of pouchitis.
The most recent series on the intentional use of IPAA in CD patients reported on
17 patients [6]. Seven of 17 patients (41 %) developed recurrent CD following IPAA
and this compared with a corresponding postoperative incidence of 11 % in a UC
cohort undergoing IPAA during the same time period. The pouch excision rate over
an average follow up of 60 months in the 17 preoperatively diagnosed CD patients
was an impressive 6 %. This study is also notable in that 9 of the 17 patients had a
preoperative diagnosis of CD outside of the colorectum: 5 patients had previously
undergone small bowel resections with no evidence of active small bowel disease
and 4 patients had perianal disease (3 perianal fistulae, 1 anal stenosis), where the
fistulae were managed by insertion of draining setons with subsequent evaluation
demonstrating no evidence of active perianal sepsis.
The most current study on this topic is a United States multi-institutional study
examining the cost-effectiveness of two surgical options in patients with Crohn’s
colitis [7]. They compared what is referred to as ‘colectomy with permanent ileos-
tomy’ with IPAA. It should be noted that some of the evidence for the former group
involves patients described in a prior study who underwent either total abdominal
colectomy with end ileostomy or panproctocolectomy with end ileostomy [8] and
the reader cannot determine whether it was only the panproctocolectomy patients
who were included in the cost analysis by Taleban and colleagues. Additionally,
Taleban and colleagues assumed that patients undergoing J-pouch formation would
have ‘complete mucosectomy’, yet this is clearly not the operative approach
employed by all. Nonetheless, colectomy with permanent end ileostomy was shown
to be more cost-effective unless the associated surgical cost exceeded $20,167 at
which point IPAA was the more effective option. They also reported that IPAA was
the more effective strategy with an incremental cost-effectiveness ratio of $70,715
per QALY gained.
Author Year Number of patients
Postoperative
morbidity
Pouch
excision
Quality of
evidence
Hyman 1991 9 8/9 (89 %) 6/9 (67 %) Low
Panis 1996 31 (18 intentional; 13
incidental)
11/31 early (35 %)
6/31 CD related
(19 %)
2/31 (6 %) Low
Regimbeau 2001 41 (26 intentional; 15
incidental)
10/41 early (24 %)
11/41 CD related
(27 %)
3/41 (7 %) Low
Melton 2008 20 Not reported 2/20
(10 %)
Low
Grucela 2011 4 2/4 (50 %) 1/4 (25 %) Low
Le 2013 17 4/17 early (24 %)
7/17 CD related
(41 %)
1/17 (6 %) Low
C. Peirce and F.H. Remzi
www.ebook3000.com
69

Recommendations Based on the Data

Since the introduction of IPAA as part of our surgical armamentarium, there have
only been 67 patients reported with a preoperative diagnosis of CD and thus an
intentional IPAA. This number can be increased to 76 when the 9 patients with a
high preoperative suspicion of CD reported in the initial study from the Cleveland
Clinic are included. Based on this, the evidence for intentional IPAA in Crohn’s
colitis is low and the recommendation for IPAA formation in patients with Crohn’s
colitis is weak.

A Personal View of the Data

The top priority is providing a personalized and tailored plan of care for each
patient. We believe that some of the most critical and complex parts of working
with a patient with Crohn’s disease occur outside of the operating room. Not only
is it imperative that detailed medical and surgical histories are obtained, but it is
also essential to develop a relationship with the patient at the first encounter and
to gain an understanding of the patient’s goals in terms of the potential for surgery
and possible outcomes. The patient and their family/caregivers should be
approached on a personal level, understanding their own goals and work for open,
honest dialogue whilst forming a specific individual surgical strategy. Having
done this, together the patient and colorectal surgeon embark on a lifelong rela-
tionship. In our experience, this specific group of patients are very well informed
on the potential surgical options and present to us with the intention of undergoing
IPAA.
The formation of an IPAA for Crohn’s colitis is considered provided there are no
gross manifestations of small bowel disease (unless it is backwash ileitis) or peri-
anal CD; a single, limited perianal fistula can be acceptable but a rectovaginal fistula
is not. CT enterography is the preoperative imaging modality of choice to examine
the small bowel and a thorough bedside perianal examination is performed and if
there are questionable findings, patients proceed to a formal examination under
anesthesia. Risk factors, especially a personal history of smoking and a family his-
tory of CD, are always sought as these patients are at increased risk for subsequent
development of CD of the ileal pouch. Patients referred from other institutions may
undergo repeat colonoscopy with biopsies and all previous outside pathology slides
are reviewed again by a dedicated inflammatory bowel disease histopathology team.
All patients have their nutritional status optimized preoperatively. Preoperative
counseling regarding the potential for complications is extensive, with particular
emphasis on the risk for significant small bowel loss if there is a requirement for
pouch excision and that a re-do pouch may not be an option. Similarly, patients are
advised that even if preoperative imaging is reassuring, there is always the potential
that small bowel CD may be discovered perioperatively.
8 Crohn’s Colitis and Ileal Pouch Anal Anastomosis
70
The technical approach to IPAA relies on careful and meticulous handling of the
bowel and dissection in natural, anatomic tissue planes. When presented with a new
patient with isolated Crohn’s colitis, a 3-stage procedure is recommended and
patients should ideally be steroid and biologic free prior to the second stage (i.e.,
pouch formation). We do not recommend a one-stage procedure and will perform a
2-stage procedure in select cases. Regardless of the operative approach (open or
laparoscopic), the small bowel must be examined in its entirety from the ligament
of Treitz to the ileocecal valve and if there is a suspicious area, this should be inter-
rogated and may require an enterotomy to ensure there is no luminal evidence of
disease. We strongly favor the total mesorectal excision technique when performing
proctectomy. Residual distal tissue may lead to pouch emptying issues, which may
significantly affect pouch function and quality of life. The J-configuration is the
pouch subtype of choice and the double-stapled pouch-anal anastomosis technique
is favored. In highly motivated patients who wish to avoid a permanent ostomy in
whom there is limited perianal disease as previously referred to, a mucosectomy
and hand-sewn anastomosis can be utilized when necessary. We have previously
reported on the learning curve for IPAA formation which is estimated to be 23 cases
when performing the stapling technique [9]. All new IPAAs are defunctioned after
their creation for a minimum of 3 months and interrogated with a radiological con-
trast enema prior to ileostomy closure.
In the unfortunate case when a Crohn’s patient with an IPAA develops anoperi-
neal sepsis or anastomotic issues, the algorithm is to begin by checking one’s own
‘footsteps’: it is critical to distinguish symptoms due to sequelae of Crohn’s disease
from a technical complication (which are more likely to develop within 3 months of
surgery). These have very different solutions and management approaches to say
the least.
IPAA surgery in patients with Crohn’s disease is technically and emotionally
challenging, but is also rewarding in that it offers a life-changing avenue for the
patient and surgeon alike.

References

1. Hyman NH, Fazio VW, Tuckson WB, Lavery IC. Consequences of ileal pouch-anal anastomo-
sis for Crohn’s colitis. Dis Colon Rectum. 1991;34:653–7.
2. Panis Y, Poupard B, Nemeth J, Lavergne A, Hautefeuille VP. Ileal pouch/anal anastomosis for
Crohn’s disease. Lancet. 1996;347:854–7.
3. Regimbeau MD, Panis Y, Pocard M, Bouhnik Y, Lavergne-Slove A, Rufat P, Matuchansky C,
Valleur P. Long-term results of ileal pouch-anal anastomosis for colorectal Crohn’s disease. Dis
Colon Rectum. 2001;44:769–78.
Patients with Crohn’s colitis and no evidence of small bowel or perianal
disease are candidates for IPAA following thorough preoperative counseling
and discussion regarding potential postoperative outcomes (evidence low;
weak recommendation).
C. Peirce and F.H. Remzi
www.ebook3000.com
71
4. Melton GB, Fazio VW, Kiran RP, He J, Lavery IC, Shen B, Achkar J-P, Church JM, Remzi
FH. Long-term outcomes with ileal pouch-anal anastomosis and Crohn’s disease. Ann Surg.
2008;248:608–16.
5. Grucela AL, Bauer JJ, Gorfine SR, Chessin DB. Outcome and long-term function of restorative
proctocolectomy for Crohn’s disease: comparison to patients with ulcerative colitis. Colorectal
Dis. 2011;13:426–30.
6. Le Q, Melmed G, Dubinsky M, McGovern D, Vasiliauskas EA, Murrell Z, Ippoliti A, Shih D,
Kaur M, Targan S, Fleshner P. Surgical outcome of ileal pouch-anal anastomosis when used
intentionally for well-defined Crohn’s disease. Inflamm Bowel Dis. 2013;19:30–6.
7. Taleban S, Van Oijen MG, Vasiliauskas EA, Fleshner PR, Shen B, Ippoliti AF, Targan SR,
Melmed GY. Colectomy with permanent end ileostomy is more cost-effective than ileal pouch-
anal anastomosis for Crohn’s colitis. Dig Dis Sci. 2016;61(2):550–9.
8. Fichera A, McCormack R, Rubin MA, Hurst RD, Michelassi F. Long-term outcome of surgi-
cally treated Crohn’s colitis: a prospective study. Dis Colon Rectum. 2005;48:963–9.
9. Tekkis PP, Fazio VW, Lavery IC, Remzi FH, Senagore AJ, Wu JS, Strong SA, Poloneicki JD,
Hull TL, Church JM. Evaluation of the learning curve in ileal pouch-anal anastomosis surgery.
Ann Surg. 2005;241:262–8.
8 Crohn’s Colitis and Ileal Pouch Anal Anastomosis
73© Springer International Publishing Switzerland 2017
N. Hyman, K. Umanskiy (eds.), Difficult Decisions in Colorectal Surgery,
Difficult Decisions in Surgery: An Evidence-Based Approach,
DOI 10.1007/978-3-319-40223-9_9
Chapter 9
Steroid Management in Patients Undergoing
Surgery for IBD
Karen Zaghiyan and Phillip Fleshner

Introduction

Often faced with the challenge of operating on steroid-treated patients with inflam-
matory bowel disease (IBD), colorectal surgeons must be well versed in the periop-
erative steroid management of this patient cohort. Historically, standard practice has
entailed stress-dose or high-dose perioperative steroids in these patients undergoing
surgery to prevent perioperative adrenal insufficiency (AI), cardiovascular collapse
and death. Stress-dose steroids typically consist of hydrocortisone 100 mg intrave-
nous (IV) given preoperatively and continued every 8 h postoperatively with a taper
down to the preoperative dose over 2–3 days [1]. However, this practice is anecdotal
and largely based on case reports from the 1950 s [2, 3] demonstrating cardiovascu-
lar collapse and death in 2 patients whose steroids were abruptly discontinued
before surgery.
Furthermore, perioperative high-dose steroids are not without consequence and
have been associated with impaired wound healing, hyperglycemia, hypertension,
fluid and electrolyte imbalance, immunosuppression and psychological effects [4]. It
has been suggested that the typical recommendation for supplementation with 200–
300 mg of hydrocortisone per day is supraphysiologic and a much smaller (maximum
of 150 mg/day) dose is necessary to overcome surgical stress [5]. While suppression
of the hypothalamic-pituitary-adrenal (HPA) axis is known to occur with chronic
corticosteroid supplementation [4], the amount and duration of steroid exposure nec-
essary to suppress an appropriate response to surgical stress is unknown, nor is the
K. Zaghiyan, MD • P. Fleshner, MD (*)
Section of Colon and Rectal Surgery, Cedars-Sinai Medical Center,
8737 Beverly Blvd. Suite 101, Los Angeles, CA, USA
e-mail: kzaghiyan@yahoo.com; pfleshner@aol.com
www.ebook3000.com
74
duration of time necessary to overcome this HPA axis dysfunction [6]; some reports
suggest this may take up to a year [7]. Thus, stress-dose steroids have even been
advocated for patients previously treated with corticosteroids within the past year.
Over the past 6 decades, several studies in IBD and non-IBD patients have
challenged the treatment algorithms for the use of perioperative stress-dose ste-
roids. Yet, there remains great variability in perioperative steroid dosing for IBD
patients undergoing colorectal surgery [8]. In this chapter, the literature pertaining
to perioperative steroid dosing is reviewed and followed by our recommendations
for steroid management in patients with IBD undergoing colorectal surgery.

Search Strategy

Relevant PICO (Population, Intervention, Comparator, Outcome) questions were
generated (Table 9.1). A Medline and PubMed search was conducted for publica-
tions in the English language between January 1952 and November 2015 using the
following search terms: (‘inflammatory bowel disease’ or ‘IBD’ or ‘ulcerative coli-
tis’ or ‘Crohn’s’ or ‘organ transplant’ or ‘transplant’ or ‘rheumatoid arthritis’ or
‘steroid-treated’) and (‘corticosteroid’ or ‘steroid’) and (‘colorectal’ or ‘colorectal
surgery’ or ‘surgery’ or ‘surgical’ or ‘operation’ or ‘operative’ or ‘perioperative’)
and (‘stress-dose’ or ‘high-dose’ or ‘low-dose’ or ‘dosing’ or ‘coverage’ or ‘previous
steroid’) and (‘adrenal insufficiency’ or ‘hemodynamic’ or ‘outcome’ or ‘complica-
tion’ or ‘morbidity’ or ‘mortality’). We also searched the reference section of each
relevant article to identify additional articles pertaining to this topic. Retrospective
and prospective, observational and randomized studies were included. Given the
paucity of studies investigating IBD patients undergoing colorectal surgery, the
search was expanded to include organ transplant recipients and other non- IBD ste-
roid treated patients undergoing non-colorectal surgery.
Table 9.1 PICO questions
P (patients) I (intervention) C (comparator) O (outcomes)
Steroid-treated
patients with or
without IBD
undergoing colorectal
or non-colorectal
surgery
Low-dose
perioperative
steroids
High-dose or
stress-dose
perioperative
steroids
Perioperative
hemodynamic instability,
adrenal insufficiency,
morbidity, mortality,
infectious complications
Patients with or
without IBD,
previously treated
with steroids within 1
year undergoing
colorectal or
non-colorectal
surgery
No corticosteroids High-dose or
stress-dose
perioperative
steroids
Perioperative
hemodynamic instability,
adrenal insufficiency,
morbidity, mortality,
infectious complications
K. Zaghiyan and P. Fleshner
75

Results

Over the years, several studies have been performed to assess the clinical utility and
optimal dose of perioperative corticosteroids in steroid-treated patients undergoing
surgery (Table 9.2). Initial studies challenging the concept of stress-dose steroids
were performed in an era where there were serious concerns about operative wound
healing. In these studies, patients underwent surgery without any perioperative ste-
roids, and clinical parameters and HPA function were evaluated. In 1962, Solem
and Lund reported 30 patients whose steroids had been stopped more than 4 weeks
before surgery undergoing various surgical procedures (IBD undergoing major
colorectal surgery, n = 4) without perioperative steroid cover and showed no impend-
ing hemodynamic collapse with this management [9]. Two studies investigated
patients on steroids at the time of surgery who were operated on without periopera-
tive steroids, measured HPA axis testing and clinical parameters. Hypotension
attributed to AI occurred in 4 out of 125 patients combined [10, 11].
In a follow up study, Kehlet and Binder showed that preoperative ACTH stimula-
tion testing correlated with perioperative HPA function in 48 steroid treated patients
undergoing surgery (colorectal, n = 7) without perioperative steroids, but no patients
had perioperative hemodynamic instability or required stress-dose steroids [12]. In
1981, Knudsen performed a retrospective study evaluating 250 steroid-treated IBD
patients undergoing major colorectal surgery [13]. In 50 patients, perioperative ste-
roid cover was provided whereas the remaining 200 patients underwent surgery
without perioperative steroids. The study included 3 groups of patients: (1) patients
on steroids at the time of surgery (n = 48); (2) patients whose steroids were stopped
1 week to 2 months before surgery (n = 76); and (3) patients off steroids greater than
2 months before surgery (n = 126). Intraoperative hypotension occurred in 29
patients (11.6 %) but was less common in the cohort off steroids more than 2 months
before surgery (5.6 %). In 9 patients, intraoperative rescue hydrocortisone was
given, although none of these patients had proven biochemical evidence of adreno-
cortical insufficiency. Of 8 patients developing postoperative hypotension, 2 patients
on steroids at the time of surgery who underwent surgery without steroid cover were
thought to have AI (1 biochemically proven). These early studies suggested the
need for perioperative steroids in patients on steroids at the time of surgery. However
the optimal perioperative steroid dose necessary to prevent AI and the utility of
stress-dose steroids remained unclear at that time.
Subsequent studies evaluated various perioperative steroid dosing regimens con-
sisting of low-dose steroids or maintaining patients on their preoperative steroid
dose without a stress-dose. In 1981, the utility of a single preoperative stress-dose
(hydrocortisone 100 mg) versus no stress-dose followed by reinstitution of the
patient’s preoperative steroid dose after surgery was prospectively studied in 61
steroid-treated patients with rheumatoid arthritis undergoing 107 major or minor
orthopedic operations [14]. The authors found no significant difference in the need
for perioperative rescue steroids in patients treated with stress-dose steroids (24 %)
or not (17 %). In a small study of 14 steroid-treated patients (IBD, n = 7) compared
9 Steroid Management in Patients Undergoing Surgery for IBD
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76
Table 9.2 Studies evaluating perioperative steroid dosing
First author (year) Patients studied Intervention
Study
design N Outcome
Quality of
evidence
Solem (1962) [9] Patients previously treated with
steroids/various surgeries (n = 4
IBD/CRS).
No periop
steroids
R 30 No unexplained death
attributed to AI
Very low
Jasani (1968) [10] RA/anterior synovectomy No periop
steroids
PO 21 steroid
treated vs. 20
controls
1 patient with abnormal
preop ACTH had
hypotension responsive to
steroids.
Very low
Kehlet (1973)
[11]
Steroid-treated patients
undergoing various major/minor
operations
No periop
steroids
PO 104 3 patients with
hypotension and low
cortisol thought to be AI
Low
Knudsen (1981)
[13]
IBD/CRS 200 with no
periop steroids,
50 received
steroids
R 250 11 cases of hypotension
treated with steroids/
possible AI
Very low
Lloyd (1981) [14] RA/Orthopedic surgery Stress-dose vs.
usual daily dose.
PO 61 No difference in periop
steroid supplementation
between the 2 groups
Very low
Symreng (1981)
[15]
Various patients (n = 7 IBD,
n = 16, CRS)
If impaired
ACTH stim
test > HC 25 mg
IV preop then
100 mg IV/24 h.
If normal ACTH
stim test: no
periop steroids.
Return to usual
daily dose
postop.
PO 14 steroid-
treated
patients and 8
steroid-naïve
controls
No hemodynamic
instability
Very low
K. Zaghiyan and P. Fleshner