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Deciding onanIRA vs. IPAA forFAP
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37
SamuelH.Lai andJonD.Vogel
Introduction
Familial adenomatous polyposis (FAP) is an inherited colorectal cancer syndrome
that results from a germline mutation of the adenomatous polyposis coli (APC)
gene. Transmission of this mutation occurs in an autosomal dominant manner.
However, in up to 25% of cases [1, 2], vertical transmission of the APC mutation
cannot be clearly determined and is considered to be sporadic or de novo in origin.
The precise APC mutation is quite variable and results in signicant differences in
the phenotype, or clinical manifestations, of the mutation. For example, certain
APC mutations will result in hundreds of colorectal adenomatous polyps and others
in thousands. Other APC mutations are associated with desmoid tumor formation or
advanced periampullary adenoma [3]. In some cases, the APC mutation may result
in an “attenuated” phenotype with scores, rather than hundreds or thousands, of
colorectal polyps.
A primary concern in patients with FAP is that one of or more of the colorectal
adenomas will progress to adenocarcinoma [4]. Endoscopic surveillance is used to
assess polyp (i.e. adenoma) burden and perform biopsies, as needed, to exclude
high-grade dysplasia or cancer. Colectomy is generally indicated when either highgrade dysplasia or cancer is detected, when endoscopic management is hindered by
polyp number or density, or when technical or logistical challenges make endoscopic surveillance impractical. The surgical options for FAP include proctocolectomy with ileostomy or ileal pouch anal anastomosis (IPAA) or total abdominal
colectomy with ileorectal anastomosis (IRA). When IRA is performed, endoscopic
surveillance of the remnant rectum is required. Determination of the best surgical
S. H. Lai · J. D. Vogel (*)
University of Colorado Surgery, Aurora, CO, USA
e-mail: samuel.lai@cuanschutz.edu; jon.vogel@cuanschutz.edu
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2023
K. Umanskiy, N. Hyman (eds.), Difcult Decisions in Colorectal Surgery,
Difcult Decisions in Surgery: An Evidence-Based Approach,
https://doi.org/10.1007/978-3-031-42303-1_37
409
410
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S. H. Lai and J. D. Vogel
option for FAP requires consideration of multiple variables including oncological
and functional outcomes, operative, and postoperative complications. Other considerations include postoperative desmoid formation and fertility impairment. This
chapter aims to provide a summary of the current literature with evidence-based
recommendations in selecting the appropriate procedure for patients with FAP.
Search Strategy
Patients
Patients with
familial
adenomatous
Polyposis
Intervention Comparator
Ileorectal
anastomosis
Ileal pouch-anal
anastomosis
Outcomes
Health-related quality of life;
operative complications; functional
outcomes; surgical outcomes
We performed a systematic literature search through June 2022 with the aims of
answering the following PICO (Patients, Intervention, Comparator, Outcome) question: “In patients with familial adenomatous polyposis (FAP), who should undergo
ileorectal anastomosis (IRA) versus ileal pouch-anal anastomosis (IPAA)?”. A targeted search of published literature in English up to 2022 was performed in PubMed,
MEDLINE, Google Scholar, and Cochrane Database of Collected Review. Search
terms utilized included: “familial adenomatous polyposis”, “ileorectal anastomosis”, “ileal-pouch anal anastomosis”, and “restorative proctocolectomy”. Studies
were excluded if there were no direct comparisons between IRA and IPAA, unable
to differentiate the outcomes between IPAA, IRA, total colectomy with end ileostomy or other procedures, or were not specic to patients with FAP (e.g., included
patients with ulcerative colitis). Reference lists of included studies, systematic
reviews, and meta-analyses were then reviewed to identify and incorporate additional relevant studies.
Results
Currently, there are no randomized control trials directly comparing IRA to IPAA
in patients with FAP.The majority of studies are retrospective and include FAP
registries or single center series Tables 37.1 and 37.2 summarizes the major ndings in postoperative [5–14] and functional outcomes [7, 9–12, 14–17] from the
available studies. Few studies published in the last 10 years include only FAP
patients. More often, both FAP and ulcerative colitis patients are analyzed together.
However, as FAP and ulcerative colitis (UC) are distinct diseases, studies incorporating both UC and FAP without separated subgroup analyses have been excluded.
As a result, the grade of evidence in current literature remains low to moderate
regarding this topic.
37 Deciding onanIRA vs. IPAA forFAP
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411
Overall
Complications
Desmoid
Tumor
5 (1) 2 (1) – –
Secondary
proctectomy or
pouch excision
IRA greater
risk
HR 14.9, CI
1
(0.3)
Metachronous Cancer in
pouch or rectum, n(%)
52
(9)
0.75
(survival
rsk)
– – – – –
1.8–121
0 (0) 18%
risk at
10years
(8)
26 (30)
(21)
0 (0) 39 (28) – – – 28
(13)
13 (26)
(23)
13 (48)
(19)
1 (4) 3 (7) – – – 8
(17)
24 (26)
(continued)
(17)
0 (0) – – – – 4
(11)
IRA IPAA IRA IPAA IRA IPAA IRA IPAA IRA IPAA
0.82
(survival
risk)
Cohort Size,
n(%) Overall Survival
IRA=585
(63)
IPAA=340
Grade of
Evidence
Low
quality
Retrospective
review
Ardoino
etal.
Study
Range Authors Study Design
1947–
Table 37.1 Surgical outcomes after IRA vs IPAA
2015
– – 60
(37)
IRA=776
Low
Retrospective
Bulow
1950–
(62)
IPAA=471
quality
review
etal.
2006
77% 96% 18
(38)
IRA=140
Low
Retrospective
Koskenvuo
1963–
(61)
IPAA=88
quality
review
etal.
2012
– – – – – – 10 (17) 3 (6) 13
(39)
IRA=60
Low
Retrospective
Soravia
1980–
(55)
IPAA=50
quality
review
etal.
1997
– – 6
IRA=42
(45)
Low
Retrospective
Campos
1977–
(49)
IPAA=27
quality
review
etal.
2006
– – 2
(31)
IRA=21
Low
Retrospective
Ambroze
1978–
(12)
IPAA=94
quality
review
etal.
1988
(82)
412
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S. H. Lai and J. D. Vogel
6 (25)
Overall
Complications
Desmoid
Tumor
Secondary
proctectomy or
pouch excision
Metachronous Cancer in
pouch or rectum, n(%)
(21)
4 (29) 5 (15) 3
(0)
39 (51)
10 (6) 6 (5) – –
(3)
– 32 (20) 4
(3)
0 (0) 2 (3) 11
– 12 (28) 0
(26)
(0)
(28)
3 (16)
(66)
– –
Open
= (22)
(13)
– – – – Open =
Open=
98%
Lap =
(19)
Lap=(4.8)
Lap=
98%
IRA IPAA IRA IPAA IRA IPAA IRA IPAA IRA IPAA
– – – 0 (0) 0 (0) 0
Cohort Size,
n(%) Overall Survival
IRA=14
(37)
IPAA=24
Grade of
Evidence
Low
quality
Prospective
cohort
Tonelli
etal.
Study
Range Authors Study Design
1984–
Table 37.1 (continued)
1995
– – 4
(63)
IRA=183
Low
Retrospective
Duijvendijk
1961–
(57)
IPAA=140
quality
review
etal.
1996
86% 100% 12
(43)
IRA=43 (42)
Low
Bjork etal. Retrospective
1984–
– – – – – – – – 4
IPAA=59
(64)
IRA=6
quality
Low
review
Retrospective
Mozafar
1996
2008–
(24)
IPAA=19
quality
review
etal.
2012
(76)
Open=
100%
Open
IRA=33
Low
quality
Retrospective
review
Konishi
etal.
2000–
2012
Lap=
(39)
96%
IRA=52
Lap
(61)
Open
IPAA=97
(57)
Lap
IPAA=74
(43)
IRA ileorectal anastomosis, IPAA ileal pouch-anal anastomosis, Lap laparoscopic surgery, Open open surgery
37 Deciding onanIRA vs. IPAA forFAP
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413
Antidiarrheal
Use, n(%) Sexual dysfunction, (%)
16(40) 12(28) 8(20) 7(16) – –
n(%)
n(%)
Daytime=
Daytime=
<6/day=
Urgency,
Incontinence
episodes,
10(23)
Night time=
4(6)
Night time=
30 (70)
>6/day=
Male = (2)
– – 0 (0) 16 (17) Male
21(49)
Daytime=
5(13)
Daytime=
13 (30)
Daytime
Female = (8)
= (10)
Female
= (13)
12 (13)
Night time=
28 (30)
4 (17)
Night time=
3 (11)
=5
Night time
=1
Male=2 (7)
Female=3
(11)
0 (0)
Female=
2 (18)
15 (50) 17 (32) 1 (3) 13 (24) Male=
Daytime=
6 (11)
Night time=
20 (37)
Daytime=
0 (0)
Night time=
0 (0)
>5/day=
14 (24)
– – (15) (28) – –
Daytime
Daytime
Daytime
= (6)
Night time
= (14)
Night time
=6
Night time
= (26)
= (3)
=2
6(29) 6(6) 3(14) 13(14) – –
Daytime=
Daytime
Daytime
23(24)
Night time=
=7(33)
Night time
=6
Night time
(continued)
36(38)
=5(24)
=2
Number of stools/
day
IRA IPAA IRA IPAA IRA IPAA IRA IPAA IRA IPAA
<6/day=
30 (75)
>6/day=
Cohort
Size,
n(%)
IRA=
60 (55)
IPAA=
Grade of
Evidence
Low
quality
Study
Design
Retrospective
review
Soravia
etal.
Study
Range Authors
1980–
Table 37.2 Functional outcomes after IRA vs IPAA
1997
10 (25)
Daytime
50 (45)
IRA=
Low
Retrospective
Ambroze
1978–
=4
Night time
21 (12)
IPAA=
quality
review
etal.
1988
=1
94 (82)
>5/day
=5 (17)
IRA=
43 (42)
quality
Low
Retrospective
review
Bjork
etal.
1984–
1996
IPAA=
Daytime
59 (64)
IRA=
Low
Retrospective
Duijvendijk
1961–
=4.7
Night time
=1.5
183 (57)
IPAA=
140 (43)
quality
review
etal.
1996
Daytime
=5
IRA=
21 (18)
Low
quality
Retrospective
review
Hassan
etal.
1981–
1998
Night time
IPAA=
5 8 0 (0) 13 (43) – – – – – –
=1
IRA=
94 (82)
Low
Ko etal. Retrospective
1980–
14 (32)
IPAA=
quality
review
1998
30 (68)
414
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7 (35) – –
Antidiarrheal
Use, n(%) Sexual dysfunction, (%)
Urgency,
n(%)
(17)
3 (21) 2 (8) 3 (19) 4 (17) – –
Daytime
=2 (8)
Night time
S. H. Lai and J. D. Vogel
=6 (25)
Incontinence
episodes,
Number of stools/
Cohort
(continued)
Table 37.2
n(%)
day
IRA IPAA IRA IPAA IRA IPAA IRA IPAA IRA IPAA
Size,
n(%)
Grade of
Evidence
Study
Design
Study
Range Authors
3 5 17 (28) 8 (40) 31 (50) 3 (15) 10
IRA=
62 (75)
IPAA=
Low
quality
Retrospective
review
Madden
etal.
1977–
1989
– – (50) (21) – – (50) (26) – –
20 (25)
IRA=
Low
Retrospective
Mozafar
2008–
6 (24)
IPAA=
quality
review
etal.
2012
Daytime=
Night
Night time
19 (76)
IRA=1
Low
Prospective
Tonelli
1984–
0 (0)
Night time=
0 (0)
time=0.5
=0.9
4 (37)
IPAA=
24 (63)
quality
cohort
etal.
1995
IRA ileorectal anastomosis, IPAA ileal pouch-anal anastomosis
37 Deciding onanIRA vs. IPAA forFAP
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415
Surgical Outcomes
The technical challenges and complexity of IPAA raise the concern for signicantly
greater risks for morbidities compared to IRA.Indeed, Bjork etal. demonstrated a
signicantly higher overall complication rates in patients who underwent IPAA
compared to patients with IRA at 51% vs 26%, respectively (p= 0.04; OR3, CI
1.3–7.1) [12]. Similarly, in a retrospective series of 39 patients, Tonelli etal. showed
more frequent early postoperative complications in patients with IPAA (21%) compared to IRA (0%) [10]. Additional studies likewise suggest higher complication
rates after IPAA than IRA [9, 17]. On the contrary, other studies have not shown a
higher rate of complication with IPAA.In a meta-analysis of 12 comparative studies
between IRA and IPAA, Aziz etal. found no signicant differences in rates of bowel
obstruction, bleeding, anastomotic leak, surgical site infection, or intra- abdominal
sepsis. However, the 30-day re-operation rate was found to be higher in patients
who underwent IPAA (23.4%) than IRA (11.6%) (OR 2.11, CI 1.21–3.70) [18].
Risk ofDesmoid Tumor Development
Desmoid tumor is a signicant contributor to mortality in FAP and can affect up to
31% of FAP patients [19]. Risk factors for desmoid tumor in FAP patients includes
family history of desmoid disease, APC mutation location, and colorectal surgery
[20, 21]. Because surgical trauma is a signicant trigger in desmoid development,
substantial interest exists in establishing the optimal surgical operation in FAP
patients who are more prone to desmoid disease.
Multiple strategies have been advocated, including the recommendation to perform IPAA for all patients or those who may be more prone to desmoid development [22]. This approach is based on the concern of the potential inability to perform
a secondary proctectomy and ileoanal pouch after initial IRA since mesenteric desmoid development after initial IPAA may cause mesenteric shortening or hinder the
ability to perform proctectomy [23]. Alternatively, another approach recommends
the avoidance of a primary IPAA due to concern that restorative proctocolectomy
may actually be associated with an increased risk of desmoid development [24–26].
In addition, with proper screening and selection of patients based on disease severity (e.g. polyp number and amenability to endoscopic surveillance) and the precise
APC mutation, proctectomy may not be necessary [27]. In one of the largest studies
to date, Sommovilla etal. showed that in FAP patients who underwent either IPAA
(n=172) or IRA (n=173), IPAA was the single greatest independent risk factor for
desmoid formation [21]. Additional independent risk factors for desmoid disease
included family medical history (FMH) of desmoids, desmoid risk factor score
(based on patient gender, APC mutation location, extra-intestinal manifestations of
FAP, and FMH desmoids), and increasing patient height. However, the increased
association of IPAA with desmoid development is not universally accepted. In a
retrospective cohort analysis of 387 patients from 5 European polyposis registries,
no signicant differences were found in risk of desmoid development between IRA
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ww(11.1%) and IPAA (10.1%) (p = 0.53) [20]. Similarly, a meta-analysis of 12
retrospective studies on incidence of desmoid tumor showed no signicant difference in desmoid development between the two procedures (11.8% vs. 9.5%, OR
0.95, p=0.85) [28].
Notwithstanding the conicting data, Burgess etal. showed that in patients who
develop intraabdominal desmoid disease after initial prophylactic surgery, there is
no differences in complication rates after IRA (69.4%) or IPAA (70.3%) [22]. This
data suggests that in patients who may be more prone to desmoid tumor development, the notion of increased post-surgical morbidity related to desmoid tumors,
including bowel obstructions, stulas, reoperations, and need for permanent stoma,
may not be true. Thus, selecting the optimal treatment selection may be based more
heavily on considerations of functional and oncological outcomes rather than concerns about desmoid formation and its complications.
S. H. Lai and J. D. Vogel
Risk ofMetachronous Rectal Cancer
Perhaps the most important factor in deciding which operation to perform is the
oncological outcome. IPAA will reduce the risk of developing rectal cancer as it
involves removal of all (mucosectomy with sutured IPAA) or most (stapled IPAA)
of the rectal mucosa. In contrast, with IRA, the rectum remains and with it the risk
of rectal cancer. In the pre-pouch era, rates of metachronous rectal cancer in FAP
patients who underwent IRA ranged from 10–30% [29, 30]. However, since the
introduction of IPAA, in late 1970s, along with the accumulation of knowledge
about this disease, the risk of rectal cancer after IRA has decreased to less than 10%
[29] along with a 30% decrease in rate of secondary proctectomy [30]. In a study
that compared FAP patients with IRA, pre- and post-1983, Moreira etal. showed a
signicantly lower rate of rectal cancer in the post-1983 cohort [27]. Nevertheless,
it is important to note that in some patients, the need for secondary proctectomy
after IRA is not due to development of rectal cancer. Other reasons include progressive and unmanageable rectal polyposis [31, 32], functional Iroblems such as incon-
tinence [5], patient preference, or other reasons [33].
The improvement in rectal cancer risk after IRA can be attributed to a more
appropriate patient selection for IRA or IPAA.Factors associated with worse oncological outcomes should be carefully assessed, including number and size of colonic
and rectal adenomas. Patients with severe polyposis (>1000 polyps) have a 2.3
times greater predicted risk for development of colorectal cancer than patients with
<1000 polyps [34]. Thus, in patients with >1000 colorectal adenomas, IPAA or
proctocolectomy with end ileostomy is generally preferred [35]. Similarly, patients
with >20 rectal polyps should also be considered for IPAA or proctocolectomy with
end ileostomy given the signicantly higher risk of rectal failure and secondary
proctectomy compared to mild (<5) rectal polyposis (HR 25.5, 95% CI 11.1–58.8,
p<0.001) [31, 36].
The use of genotype and mutational analyses to select the appropriate surgery in
patients with FAP has been suggested. Although there is considerable variability
37 Deciding onanIRA vs. IPAA forFAP
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between phenotypic presentations among patients with identical APC mutations,
some mutations at certain APC codons, including 1250, 1309, and 1382, have been
shown to be associated with a more severe disease presentation [37]. Nieuwenhuis
etal. retrospectively categorized 475 patients with FAP into attenuated, intermediate, and severe genotype groups and determined the risk of rectal cancer and secondary proctectomy at 20years after IRA to be 6% and 10% in attenuated polyposis
genotype, 3% and 43% in intermediate genotype, and 8% and 74% in patients with
severe genotype [38]. This data suggest that IRA is an appropriate choice in patients
with attenuated genotype while removal of the rectum, with IPAA or ileostomy
creation, should be more strongly considered in patients with an intermediate or
severe polyposis genotype. However, the utility of genotype in surgical selection
has been overshadowed by the more generally accepted use of phenotype (i.e. polyp
number) as a severity indicator [39].
Finally, it is worth noting that while the risk of cancer is signicantly lower with
IPAA than IRA, retained mucosa in the anal transition zone (ATZ), even after rectal
mucosectomy and sutured IPAA, is still at risk for neoplasia [39]. Debate continues
to exist regarding whether a sutured or stapled ileal anal anastomosis is optimal for
IPAA in patients with FAP.While this topic is outside the scope of this chapter, a
separate discussion of the benets, risks, and other nuisances of each technique is
warranted. In general, stapled IPAA is considered to be a technically easier operation with better functional outcomes, and fewer postoperative complications, while
mucosectomy and sutured anastomosis may have a more favorable oncological outcome [4, 29, 40].
417
Functional Outcomes andQuality ofLife
Functional outcomes and quality of life are important considerations in selecting the
optimal surgical approach for FAP patients. In general, IRA results in better functional outcomes due to the preserved rectum, with reduced stool frequency, less
watery stool, less stool seepage, or need for antidiarrheal medication when compared to patients with IPAA [41]. Results from a meta-analysis of 12 studies of
functional outcomes after IRA or IPAA, suggest that majority of functional outcomes were better in patients who underwent IRA than IPAA [18].
In addition, IRA may pose a lower risk to female fecundity than IPAA.In a survey of 162 female patients on reproductive history and length of time to pregnancies, there was a signicant 54% decrease in fecundity after IPAA compared to IRA
[42]. Similarly, in a meta-analysis of the impact of IPAA on female fecundity in
ulcerative colitis and FAP patients, IPAA still posed a signicantly higher relative
risk for infertility (HR 3.91; 95% CI 2.06—7.44).43 In the appropriate young
female patients, these ndings have led to some surgeons advocating for IRA or a
staged approach to IPAA with initial IRA followed by secondary proctectomy and
IPAA after childbearing.42
However, differences in functional outcomes after surgery does not necessarily
translate to worse quality of life. Ko et al. showed that despite having worse
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functional outcomes in patients who underwent IPAA, quality of life was not different between IPAA and IRA patients.16 Other studies have similarly found no signicant differences in patients’ self-perceived quality of life in various areas of
daily activities including social, sexual, travel, or recreation.7, 11, 15 Conversely, a
decision analysis Markov modeling of possible outcomes after IRA or IPAA suggested that when taking quality of life into account, patients with IRA performed
better compared to those with IPAA.44 Inherent to modeling studies, however, is
the study limitation of exclusion of variables including desmoid tumor development
and risk of infertility. Interestingly, in a study of colorectal surgeons’ preferences
and assessment of patients’ quality of life after postoperatively, IRA was found to
have the highest “postoperative health state utilities” through the time trade-off
method.45 This method represents preferences for a given state of health compared
to normal life and death and has previously been used to determine value of prophylactic surgery in cancer prevention. Thus, previous quality of life surveys may not
be sensitive enough to fully capture the differences in preferences and values placed
on quality of life postoperatively by FAP patients.
While this chapter is focused mainly on the creation of an IPAA or IRA in
patients with FAP, it is important to note that patients, who choose or require an end
ileostomy after proctocolectomy, may have similar overall quality of life as patients
with an ileal pouch anal anastomosis. Even though no current studies have focused
specically on FAP patients in this context, several authors have reported similar
quality of life outcomes in ulcerative colitis patients after IPAA versus end ileostomy 46, 47. In particular, Seidel etal., showed similar outcomes in work, social and
family life, and relationships in ulcerative colitis and FAP patients who underwent
IPAA or end ileostomy 48.
S. H. Lai and J. D. Vogel
Data Based Recommendations
1. In general, patients with mild or attenuated polyposis are best suited for total
abdominal colectomy with IRA, while those with severe polyposis (>1000
colonic polyps) are more appropriate for proctocolectomy with IPAA or end
ileostomy (evidence quality low to moderate; weak recommendation).
2. Patients with less than 20 rectal polyps are generally good candidates for IRA,
while patients with more than 20 rectal polyps are better suited for an operation
that includes proctectomy (evidence quality low; weak recommendation).
3. In general, patients with a diagnosis of colon or rectal cancer should undergo
proctocolectomy (evidence quality low; weak recommendation).
4. In female patients of childbearing age, the association of a diminished ability to
get pregnant and IPAA surgery should be discussed when surgery is being
planned (evidence quality low; moderate recommendation).
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