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400 O. Aziz, P. Tekkis
Fig. 6. Meta-analysis of functional outcomes
Ileo-Rectal Anastomosis vs. Ileo-Anal Pouch as the Surgical Treatment for Familial Adenomatous Polyposis 401
with mucosectomy. The decision to undertake IRA as
the primary operation for FAP is therefore justified
on the basis of post-operative morbidity and func-
tional outcome (except for faecal urgency, which is
greater than in IPAA), but at the expense of cancer
recurrence. Whether regression of rectal polyps with
drugs such as sundilac, a non-steroidal anti-inflam-
matory agent used to reduce rectal polyp size and
number in FAP patients following IRA [49], remains
to be seen. Whilst a selective COX-2 inhibitor has
also recently been shown to reduce the number of
colorectal polyps in patients with FAP [50], concerns
over the long-term risks of this class of drug have
made its role uncertain.
Molecular genetic testing may be useful in guiding
the surgical management of patients with FAP, with
authors suggesting that patients with APC mutations
following codon 1250 are at higher risk of developing
rectal cancer and should therefore undergo IPAA
rather than IRA [51]. Other mutations that have been
implicated include codons 1309 and 1328, again sug-
gesting the need for IPAA in these patients [9]. It is
important to consider, however, that these patients
are also at higher risk of developing pouch adenomas,
and will therefore require close follow-up of their
pouch following IPAA. In the FAP patient where
molecular genetic testing has not detected mutations
predisposing to a higher risk of cancer, severity of
polyposis must also be taken into account. For exam-
ple IPAA may be the best primary surgical interven-
tion for patients with over 20 rectal polyps and greater
than 2 000 colonic polyps, whereas those with less
than five rectal polyps and fewer than 1 000 colonic
polyps would qualify for IRA and surveillance of the
rectal stump as they may represent an attenuated FAP
group. Careful patient selection is required in the
intermediate group (those with more than five but
less than twenty rectal polyps) before deciding on
their primary surgical interventional strategy.
This chapter highlights the merits and weaknesses
of both IPAA and IRA as the primary treatment of
FAP, both of which require close follow-up after sur-
gery. Although it highlights several factors that
should be considered, it also highlights the need for
further investigation of which patients benefit most
from which operation depending on their risk fac-
tors. The age of the patient, their sex, lifestyle and
preferences, occupation, and compliance to follow-
up are all very important in deciding the operative
outcome most appropriate for them.
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Introduction
Familial polyposis syndromes create a group of
hereditary syndromes of gastrointestinal (GI)
tumours. This chapter focuses on those primarily
affecting the large bowel and which require radical
surgery.
A GI polyp is defined as a mass of the mucosal sur-
face protruding into the bowel lumen. Polyps can be
neoplastic, non-neoplastic or submucosal. GI poly-
posis is characterised by multiple polyps within the
GI tract (GIT). A variety of polyposis syndromes can
affect the GIT. Familial polyposis syndromes can be
classified as familial, inherited (autosomal domi-
nant) or nonfamilial. Inherited polyposis syndromes
can be further subdivided into two groups depending
on whether the polyps are adenomas or hamartomas.
Adenomatous polyposis syndromes include the clas-
sic familial adenomatous polyposis (FAP), Gardner's
syndrome and Turcot syndrome. Hamartomatous
familial polyposis syndromes include Peutz-Jeghers
syndrome (PJS), juvenile polyposis syndrome (JPS),
Cowden disease and Ruvalcaba-Myhre-Smith syn-
drome. The non-inherited polyposis syndromes
include Cronkhite-Canada syndrome and a variety of
miscellaneous non-familial polyposis.
From a prognostic viewpoint, these syndromes
must be recognised, because adenomatous polyps are
premalignant. These syndromes should be consid-
ered when an intestinal polyp is recognized in the
young, when two or more polyps are seen in any
patient, when colic carcinoma is discovered in
patients younger than 40 years and when extrain-
testinal manifestations associated with these syn-
dromes are discovered. GI polyps may be asympto-
matic but may also occur with rectal bleeding and
diarrhoea. The urgency of case tracing and genetic
counseling is related not so much to symptoms of the
disease but to the potential for development of a colic
carcinoma. It is probable that patients with FAP, if
untreated, will develop a colic carcinoma.
Familial Adenomatous Polyposis
FAP is also known as familial polyposis coli. The
polyps are adenomatous and occur primarily in the
colon. Carcinoma occurs mostly in the left colon and
are often multiple. It is also possible, however, for
polyps, adenomas and carcinomas to grow in the
stomach, duodenum and small intestine. Connected
with extraintestinal manifestation, it is known as
Gardner's syndrome. Manifestations include osteo-
mas, jaw cysts, dental anomalies, brain tumours,
other skin and soft tissues tumours and congenital
hypertrophy of the retinal pigmentary epithelium [1].
FAP shows autosomal dominant transmission. Fre-
quency is one in 8,300-13,500 births. In Denmark, new
mutations account for 25% of cases [2]. Average age
at the time of diagnosis is 35 years, and average age at
cancer diagnosis is 39 years [3]. About 50% of symp-
tomatic and 10% of asymptomatic patients have car-
cinoma of the colon at the time of diagnosis. Carcino-
ma may be present in all cases by the age of 50 years.
Penetrance is nearly completed by age 40 years [2].
Gardner’s Syndrome
Gardner's syndrome was originally described in indi-
viduals with FAP-like polyps who had additional
findings outside the GIT, including epidermal cysts
and subcutaneous fibromas of the skin, desmoids of
the skin and abdomen, osteomas, dental abnormali-
ties, pigmented patches in the retina and tumours of
other organs [4]. Gardner's syndrome includes a
variety of manifestations:
Soft-tissue tumours:
• Epidermoid inclusion cysts of the skin
• Dermoid tumours
• Fibromas
• Lipomas
• Lipofibromas
• Neurofibromas
• Leiomyomas
Surgery and Surveillance in Colon Polyposis Syndrome
To m á š Skřička
406 T. Skrˇička
• Mammary fibromatosis
• Intra-abdominal desmoid tumours and peritoneal
fibromas
GI tumours:
• Colic polyposis (nearly 100% precancerosis)
• Gastric adenomatous polyps
• Gastric hamartomatous polyps
• Duodenal adenomatous polyps
• Periampullary carcinoma
• Hepatoblastoma
• Pancreatic carcinoma
• Lymphoid hyperplasia of the terminal ileum
Osseous abnormalities:
• Usually involving the membranous bones,
mandible, calvaria, maxilla, ribs and long bones
• Self-limited benign exostosis
• Bone islands and periosteal thickening
Endocrine tumours:
• Thyroid carcinoma (papillary)
• Carcinoid tumours of the small bowel
• Parathyroid adenoma
• Adrenal adenoma/carcinoma
• Pituitary chromophobe adenoma
Central nervous system medulloblastoma;
Abnormal dentition:
• Supernumerary teeth
• Impacted teeth
• Odontoma
• Hypercementosis
• Teeth more prone to carries
The distinction between Gardner's syndrome and
FAP was initially unclear, however, because some
individuals diagnosed with FAP also had extraintesti-
nal signs. Clinically, Gardner's syndrome and FAP
can occur in the same kindred. It was subsequently
found that both conditions can result from the same
mutation in the APC gene. Thus, Gardner's syn-
drome and FAP are allelic. [Many genes show alter-
ations in GIT neoplasia that do not, as yet, have an
immunohistochemically detectable protein product.
These genes include the APC (adenomatous polypo-
sis coli) and the MCC (mutated in colon cancer)
genes].
Other Hereditary Syndromes of GI Tumours
Turcot Syndrome
Turcot first described an association between colic
polyps and tumours [5]. Turcot syndrome is charac-
terized by the presence of a malignant brain tumour
in patients with colic adenomatous polyps [5].
Recent advances [6] have clarified the relationship
between Turcot syndrome and Gardner's syndrome,
which may also be associated with brain tumours.
Cronkhite-Canada Syndrome
This syndrome includes generalized GI polyposis in
association with neuroectodermal changes consist-
ing of alopecia, hyperpigmentation and nail atrophy.
Electron microscopic studies of the skin reveal
increased numbers of melanin granules in kerato-
cytes, increased number of melanosomes in
melanocytes, increased melanocytes, compact hyper-
keratosis, perivascular inflammation and exocytosis.
A number of haematologic, neurologic and metabol-
ic abnormalities are associated with Cronkhite-Cana-
da syndrome.
Attenuated FAP
This is a less severe form of polyposis, with a low
number of polyps (adenomas), usually less than 100,
yet patients sustain a high risk for colorectal cancer.
These cancers usually develop 15 years later than the
classic FAP patient, but ten years earlier than spo-
radic cancer [7].
Hamartomatous Polyposis
Polyps in both PJS and familial JPS are hamartoma-
tous, but the major tissue components are different.
The Peutz-Jeghers polyp is composed of intestinal
crypts and villi and smooth-muscle bundles in a dis-
organised fashion. Inflammatory cells are absent or
scanty, and glandular structures are not excessively
dilated. The juvenile polyp is composed of dilated
intestinal glands and abundant connective tissue of
the lamina propria. Smooth-muscle elements are
usually absent; lymphoid cells are common. These
features closely resemble those of inflammatory
retention polyps in children, also known as juvenile
polyps, as well as inflammatory polyps of Cronkhite-
Canada syndrome, which occur among the elderly.
PJS is characterized by the presence of Peutz-
Jeghers polyps throughout the entire GIT and
melanin spots on the lip (96%), buccal mucosa
(83%), face (36%) and extremities (32%) [8].The
small bowel is the favoured site of polyposis and the
number of polyps is small. Other associated abnor-
malities include polyps in the urinary bladder and
nasal cavity, bone deformities, congenital heart dis-
ease and retarded development. The symptoms of
PJS develop before the age of 20 in two-thirds of
cases, with an average age at time of diagnosis of 22
years [3]. Symptoms are noted at a younger age than
those associated with FAP. Peutz-Jeghers polyps are
generally not prone to malignant change. However,
Surgery and Surveillance in Colon Polyposis Syndrome 407
carcinoma of the GIT has been reported in about 3%
of cases, most commonly in the proximal small intes-
tine [9]. In many reports, the relationship between
polyp and carcinoma is unclear. When the origin of
malignancy was carefully studied, the carcinoma was
usually found to arise in the adenomatous or dys-
plastic epithelium in the polyp [10]. Malignancy can
also occur outside of the GIT. The condition is linked
to 19p chromosomes in at least some families.
Discrete Adenomas and Carcinomas of the Colon
Whereas polyposis syndromes are rare, discrete ade-
nomas in small numbers occur in from 10% to 50%
of the general population. Overall, colorectal cancer
may affect 3% of the population. The inheritance pat-
tern of these cases is largely unknown. Park et al. [11]
studied a large groups with clusters of colorectal can-
cer. Extensive screening with flexible proctosigmoi-
doscopy revealed adenomas in 21% of the 191 pedi-
gree members in contrast to 9% of controls. The
excess of adenomas showed autosomal dominant
inheritance. A subsequent expanded study of 670
persons in 34 kindreds revealed the estimated preva-
lence of adenoma at age 60 years in related family
members was 24% whereas that in unrelated spouses
was 12%. Such studies emphasise the importance of
screening for colorectal tumours in first-degree rela-
tives of patients with these lesions.
Hereditary Flat Adenoma Syndrome
Hereditary flat adenoma syndrome (HFAS) is
presently thought to be a variant of FAP, with the
genetic defect linked to 5q21-22. Signs of HFAS are:
• Multiple colorectal adenomas, but usually fewer
than 100
• Polyps tend to occur at a later age than in classic
FAP
• Adenomas tend to show a proximal location
• Onset of colorectal cancer is later than in heredi-
tary non-polyposis colorectal cancer (HNPCC)
and FAP
• These individuals have adenomas and cancers of
the stomach and duodenum
• Fundic gland polyps of the stomach are also noted
In some patients fundic may be present in the
absence of colorectal adenomas [12]. Lynch stressed
that only about 1% of such adenomas have high-
grade dysplasia, which is much lower than reported
in patients with sporadic flat adenoma [12].
Muir Torre syndrome
Muir Torre syndrome was originally subclassified as
a form of FAP. Muir Torre syndrome is a rare auto-
somal dominant disorder with fewer than 100 adeno-
mas, typically present in the proximal colon. This
syndrome is associated with skin lesions such as
basal cell carcinoma, sebaceous carcinoma and squa-
mous carcinoma. The genetics of this syndrome is
not yet known.
Cowden Disease
Cowden disease is an uncommon autosomal domi-
nant disorder and is the family name of the original
report patient, Rachel Cowden. This syndrome is a
rare disorder that is inherited in autosomal dominant
manner with intrafamilial and interfamilial differ-
ences in symptom expression. In Cowden disease, one
sees facial trichilemmomas, acral keratosis and oral
mucosal papillomas. This disorder is also associated
with breast and thyroid cancer. There are numerous
colic and small-intestinal polyps. They have been
described as hamartomatous lesions [13] consisting
of mildly fibrotic, mildly disordered mucosa overly-
ing a submucosa that display disorganisation and
splaying of smooth-muscle fibres. These lesions show
some similarities to the pathology seen in solitary rec-
tal ulcer syndrome. Other authors reported polyps
that they described as inflammatory lesions, lipomas
and ganglioneuromas. There is no increased risk for
GI cancers in this disorder [14].
Ruvalcaba-Myhre-Smith Syndrome
Ruvalcaba-Myhre-Smith syndrome is inherited in an
autosomal dominant manner. There are also some
sporadic cases. The syndrome is characterised by
macrocephaly, pigmented genital lesions, subcuta-
neous and visceral lipomas, haemangiomas and GI
hamartomatous polyps. Mesodermal hamartomas
can affect the subcutaneous, intracranial, visceral,
intestinal, thoracic and osseous tissues. Hydro-
cephalus and diffuse thickening of the corpus callo-
sum have also been reported [15].
Intestinal Ganglioneuromatosis
Intestinal ganglioneuromatosis is a familial disorder
that has been associated with multiple endocrine
neoplasia syndrome type 2b and with the Reckling-
hausen's disease. There may be a diffuse proliferation
408 T. Skrˇička
of ganglioneuromatous elements, which at times may
be polypoid. In some instances, the ganglioneuro-
matosis has been found in association with JP and
adenomas [16].
Lymphoid Polyposis
Multiple benign lymphoid polyposis of the large
bowel has been reported. Most cases occur in chil-
dren. Histologically similar to solitary lymphoid
polyps of the rectum, lymphoid polyposis consists of
prominent active lymphoid nodules in the mucosa
and submucosa [17]. Lesions are entirely benign and
in some cases have been reported to disappear spon-
taneously. In patients with family histories of polyps,
it is essential to determine the exact histologic nature
of the lesions so that unnecessary surgery is not per-
formed. Benign lymphoid polyposis of the terminal
ileum has been reported in patients with Gardner's
syndrome and FAP [18].
Hereditary Mixed Polyposis Syndrome
This is an autosomal dominant disorder that has
been mapped to chromosome 6q. Five types of
polyps have been described in individuals with this
disorder:
• tubular adenomas
• Villous adenomas
• Flat adenomas
• Hyperplastic polyps
• Typical juvenile polyps.
Colorectal cancer is also seen in this disorder, which
might be a variant of JP. But in JP, adenomas are
uncommon (2%) while in hereditary mixed polypo-
sis, the number of polyps are fewer than seen in JP
[19]. JP usually presents one decade earlier than
hereditary mixed polyposis.
Non-Surgical Treatment of FAP
There are reports [20] of attempts to treat FAP with-
out operation, and even though the authors reported
a decrease in the number of polyps to 44% a their size
to 35% by means of sulindac (sulfoxide non-steroidal
anti-inflammatory drug) in 18 patients, this method
could not replace colectomy.
Surgical Treatment of FAP
As FAP is 100% precancerous, colectomy is recom-
mended. Treatment of FAP is influenced by the nat-
ural history of the disease, which is variable. If
patients are left long enough without colectomy, they
will all develop carcinoma. The sooner is patient
diagnosed, the better prognosis could be if colectomy
is performed. There are three possibilities of surgical
treatment. Each has its pros and cons:
• Total colectomy with permanent ileostomy (in
cases of malignancy in the lower rectum)
• Total colectomy with ileoanal anastomosis (IAA)
(poor functional results)
• Subtotal colectomy with ileorectal anastomosis
(IRA) (good functional results but risk of recur-
rence in the rectal stump)
The procedure of choice is the subject of much
debate, particularly since restorative proctocolecto-
my became feasible [21]. The other options are colec-
tomy and IRA, proctocolectomy or colectomy and
rectal mucosectomy without restoration of GI conti-
nuity. Conventional panproctocolectomy has the
advantage of eliminating all colorectal polyps and
virtually eliminating the risk of carcinoma of the
large bowel. This operation does not protect against
ampullary or small-bowel malignancy. In addition,
there is the burden of an ileostomy in a condition
where 50% of family members are at risk of disease,
and the patient is exposed to the small risk of pelvic
nerve damage with resulting bladder and sexual
problems. Also, there is a perineal wound, which
although less likely to break down than after procto-
colectomy for inflammatory bowel disease, may leave
the patient with a persistent sinus.
Intersphincteric excision of the rectum reduces
risk of pelvic nerve injury, and rectal mucosectomy
eliminates problems with the perineal wound. Risk
associated with a long rectal mucosectomy is that all
the diseased mucosa may not be removed, and there
is then a slight risk of carcinoma developing in any
remaining remnant. The overwhelming disadvan-
tage, however, of any procedure that leaves a perma-
nent ileostomy, is that it is a poor advertisement of
treatment for other members of the family. Although
a patient with an ileostomy can lead a full and active
life, it is difficult to convince a young and active fam-
ily member, who may well be asymptomatic, to
undergo such a procedure. It is for this reason that
sphincter-saving procedures such as colectomy with
IRA or restorative proctocolectomy have become
popular [22].
The procedure of colectomy and IRA has the
advantage that GI continuity is restored and bowel
function is reasonable. Its disadvantage is that polyps
are left in the rectum, with the potential of malignant
change. To prevent this unfortunate outcome,
patient need to have repeated fulguration of residual
or newly formed polyps [23]. Not only can this pro-
cedure be uncomfortable, it also requires the patient
Surgery and Surveillance in Colon Polyposis Syndrome 409
to return repeatedly for rectoscopic examination. In
addition, although it would seem logical, if the ade-
noma-carcinoma sequence is accepted - that removal
of polyps removes the risk of carcinoma - this does
not necessarily follow. There is evidence to suggest
that carcinoma can develop de novo in rectal mucosa
not occupied by polyps.
Mucosal proctectomy and pelvic ileal reservoir,
now generally termed restorative proctocolectomy,
has the theoretical advantage that the disease is erad-
icated, GI continuity is restored and continence is
maintained. It appears, unfortunately, from some
results reported, that bowel function is not always as
satisfactory as after IRA. However, these results have
in the main been described in patients who have suf-
fered from ulcerative colitis. There is now substantial
evidence either from our own experience or from
others that the clinical results of restorative procto-
colectomy for polyposis are far superior to those
reported in patients with colitis. Nevertheless,
restorative proctocolectomy is a complex procedure
and although mortality is very low, morbidity can be
high. Although results are steadily improving, the
procedure is still developing, and the long-term
results in FAP are unknown, perhaps due to the small
number of patients. Further modifications may be
desirable before it can be categorically accepted as
the operation of first choice for all patients with FAP.
The conventional policy in many units dealing
with these patients is still to perform a colectomy and
ileorectal anastomosis in the first instance, provided
patients are likely to be reliable in attending for fol-
low-up. Follow-up at regular 6-month intervals is
usually necessary so that rectal polyps, if present, can
be destroyed by fulguration. In order to determine if
this is still a reasonable policy, it is necessary to
access the risk of developing a rectal carcinoma after
IRA.
Carcinoma Risk after Colectomy and Ileorectal
Anastomosis
There is considerable controversy concerning the
incidence of carcinoma following colectomy and
IRA. The evidence against ileorectal anastomosis
came mainly from the Mayo Clinic. Moertel et al. [24]
found that in 145 patients treated by subtotal colec-
tomy and IRA, there was a continuing risk of rectal
cancer. This was 25% after 15 years of follow-up and
59% after 23 years. They found that women were at
greater risk than men and that the incidence was
markedly increased if the patient had carcinoma in
the resected colon. Another factor with important
clinical application was that the risk of carcinoma
was considerably reduced if there were fewer than 20
polyps in the rectum compared with patients having
more than 100. Some doubt has since been expressed
about how many of these patients actually had a true
IRA. It would appear that many underwent an
ileosigmoid anastomosis, and it is unknown what
influence the latter procedure might have had on the
outcome, particularly since surveillance following
this operation is more difficult than after a true IRA
[25]. Fuel for the controversy was further supplied by
reports from other centres [26]. They reported a
series of 89 patients treated by a true IRA and 6-
monthly surveillance. Included in this series were 47
patients followed for 10 years, 27 followed for 15
years and 13 followed for 20 years. Only two patients
(2.2%) developed carcinoma, both of which were
Duke's A lesions, and both patients survived. There
are similar reports from other centres [27, 28] show-
ing, that carcinoma develops in up to 6% of cases.
Making recommendations from these contradic-
tory data, particularly at a time when restorative
proctocolectomy is not fully tested, is clearly diffi-
cult. There is no doubt that there is a risk of develop-
ing carcinoma after colectomy and IRA, which
relates to the number of polyps in the rectum and the
presence of coexisting carcinoma. This may also be
related to how much colorectum remains; there may
be a geographic difference. Consequently, there has
been general unease about performing colectomy
and IRA in various countries.
Present Surgical Policy
At present, policy around the world is variable. Most
surgeons still seem content to treat their patients
with colectomy and IRA followed by regular surveil-
lance. An increasing proportion, on the other hand,
is performing restorative proctocolectomy in most of
their patients and certainly on those with severe rec-
tal polyposis. A more rational policy, perhaps, is the
compromise advocated by some surgeons. If the
number of polyps within 15 cm from the anal verge is
less than 20, we perform a colectomy and IRA.
Restorative proctocolectomy is used for patients with
more than 100 rectal polyps. Patients with 20-100
polyps are treated by colectomy and IRA if the mini-
mum time off school is desirable or if the patient can
be relied upon to attend follow-up. All others in this
intermediate group are advised to have a pouch. As
the morbidity of restorative proctocolectomy falls
and more centres gain expertise, we are sure that it
will become the operation of first choice for all
patients with the disease. Until then, operative treat-
ment is likely to be governed by each surgeon's
beliefs and experience coupled with the patient's atti-
tude towards attending 6-monthly rectoscopy as well
410 T. Skrˇička
as the views of the patient's family.
In general, there are three options available to sur-
geon seeking to prevent patients with FAP from
dying of colorectal cancer:
• proctocolectomy with ileostomy
• Colectomy with IRA
• Proctocolectomy with ileoanal reservoir.
The choice of operation should be based on clini-
cal knowledge of the disease process and the fact that
prophylactic colectomy does not necessarily cure the
condition. Other considerations involve the patient's
age and anatomy, the presence or absence of extra-
colic manifestations and the surgeon's expertise.
Over the years, debate has centred on the value of
proctocolectomy and ileostomy with colectomy and
IRA. This debate was further compounded by the
development of the ileal reservoir procedure. Today
it seems obvious that proctocolectomy and ileostomy
should rarely be necessary for patients with FAP and
other polyposis syndromes. Therefore, the debate
now centres on the value of IRA versus proctocolec-
tomy with ileoanal reservoir or restorative procto-
colectomy. It is up to surgeon and the institution as
to whether the approach will be “traditional” (open
surgery), laparoscopically assisted, hand assisted or
completely laparoscopic. Recently [29], many
authors refer to good experiences with the laparo-
scopic approach. It could be safe and effective treat-
ment for selected patients with FAP. As techniques
and instrumentation for laparoscopic colon surgery
are perfected, this procedure will likely become an
appealing option in the management of patients with
FAP
Technique of Colectomy and Colorectal
Anastomosis
This method seem recently mostly accepted
The major concern with colectomy and IRA as a pro-
phylactic surgical option in patients with FAP is the
subsequent risk of rectal cancer. It was easier to rec-
ommend IRA to patients, and many surgeons prefer
this option after the advent of the ileoanal reservoir.
The actual documented risk of dying of cancer of the
rectum subsequent to colectomy and IRA is some-
what small [30]. The risk of developing polyps and
cancer are, of course, higher. It would appear that
proctoscopic surveillance and ablation of polyps that
arise in the rectal segment are possible, but this does
not eliminate fully the risk of rectal cancer. Long-
term follow-up studies demonstrate that develop-
ment of cancer does not always equate with death
from that cancer. IRA is compatible with good func-
tional results. It is a simpler procedure than the
ileoanal reservoir and is usually a one-stage proce-
dure as opposed to the two-stage procedure com-
monly used to construct an ileoanal reservoir. More
recent improvements in surgical technique and skill
suggest that a one-stage ileoanal reservoir without
temporary ileostomy would be an appropriate
option. Refinements in technique achieved in recent
years have improved functional results and
decreased surgical complications of proctocolectomy
and ileoanal reservoir. The ability to perform the
operation as a one-stage procedure makes it an
appropriated option as a contemporary alternative to
IRA. To support use of IRA, there are quality-of-life
(QOL) studies [31]. The authors argue that there
were no differences with respect to health status
between patients in groups of IRA and IAA and that
preference for either procedure cannot be based on
QOL.
Rectal Stump Control after Subtotal Colectomy
and Ileorectal Anastomosis
It is advised that a rectoscopy be conducted every 6
months and eventually to remove polyp remnants by
means of laser, fulguration, photocoagulation or
transanal microsurgery. Lifetime surveillance of the
rectal stump is crucial, as showed in authors from
Ankara [32]. They report a local recurrence 19 years
after IRA. There are some optimistic reports [33] that
colectomy with IRA in FAP is associated with a sig-
nificant reduction in rectal mucosal cell prolifera-
tion. These findings claim reduced risk of rectal can-
cer following this procedure in FAP and are of rele-
vance to the study of environmental versus genetic
control of cell proliferation.
Conclusion
FAP and other polyposis syndromes are generalised
disorders with ramifications throughout the body.
Early diagnosis by recognition of at-risk individuals
and proctoscopic surveillance is important in pre-
venting cancer. Surgical options now available can
eliminate the risk of colorectal cancer and provide a
good functional outcome without a permanent
ileostomy. Ongoing surveillance programmes are
important because there is still a risk of dying of
extra-colic manifestations of the disorder. Prophy-
lactic colectomy of whatever type does not predict
total cure of the disease. The two most frequent pro-
cedures (IRA and IAA) have been evaluated [34]. The
long-term survival following either approach is simi-
lar: 87% for IRA and 83% for IAA.
Management of these families is best accom-
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