If the colon and rectum is left in place in Familial Adenomatous Polyposis
(FAP) it will inevitably give rise to cancer. The aim of treatment is to remove all
colonic and rectal tissue in order to prevent this event. The anal canal consists of
a different kind of epithelium (squamous) which is not prone to carcinogenesis by
the FAP genes so it can be left in place. However once the colon and rectum
have been removed we are faced with a dilemma:
-how are we going to restore the continuity of the bowel?
If we join the end of the small bowel on the anal canal, this is described as an
ileoanal anastomosis, then excessive diarrhoea ensues. The small bowel fluid
is very loose and it also contains large quantities of bile which is an irritant for the
skin. Life with an ileoanal anastomosis is of poor quality because of constant
diarrhoea (20 – 40 times per day and night) and anal irritation. Severe
dehydration can happen very easily. Not surprisingly ieoanal anasotmosis is not
an option practiced today.
There is an option of actually not restoring the continuity. We can completely
remove the colon and rectum which is called a panproctocolectomy and
exteriorise the end of the small bowel as an end ileostomy. Ileostomy output can
usually be managed with application of appropriate bags in a way which retains
good quality of life for the patient. Panproctolectomy and ileostomy is a
reasonable surgical solution in that it removes the risk of cancer completely and
preserves good quality of life. However the patient has to live with a permanent
ileostomy. It has been shown in studies that ileostomy does not affect in the long
term quality of life but it does have an impact on the patient’s body image.
Although some patients accept easily the idea of ileostomy there are others who
cannot. The patient’s views and feelings have to be examined thoroughly before
a decision on this operation.
Restorative proctocolectomy and ileoanal pouch (for brevity reasons we will
call this procedure simply a “pouch” for the rest of this text. This operation
consists of removing completely the colon and rectum and then creating a pouch
or reservoir of small bowel by using two or more loops of small bowel. Depending
on the shape of the loops which are put together it is called a “J” pouch, a “W”
pouch or an “S” pouch. The more commonly used today is the “J” pouch.
The pouch decreases the diarrhoea to acceptable levels so that the patient’s
quality of life can be preserved. However patients with a pouch still open their
bowels 4 – 8 times a day. This may be acceptable by some patients but not by
others. The professional occupation of the patient and their lifestyle is a major
factor. Having to go the toilet 4 to 8 times a day may be tolerable to a housewife
or someone who works in an office with good toilet facilities but may be a major
problem for a bus driver. The diarrhoea of “pouch” is sometimes caused by a
condition called “pouchitis”. This is an inflammation of the pouch probably caused
by bacterial proliferation caused by stagnation of the bowel contents. Medication
such as loperamide or the antibiotic metronidazole may improve the frequency of
bowel movements.
In most cases the creation of a “pouch” has to be accompanied by a temporary
prophylactic ileostomy. The ileostomy can be closed 2-3 months later.
The choice between a permanent ileostomy and a “pouch” is not an easy one
and it finally lies with the patient. Both operations have the same efficiency in
curing the risk of colorectal cancer. The advantages of the ileostomy are that it
involves a single operation and does not cause any diarrhoea or pelvic
inflammation problems. In that sense it is an operation suitable for people who
are anxious to put an end to their treatments as soon as possible and get on with
their lives. As long as the patient can accept the idea of living with an ileostomy,
panproctocolectomy is the fastest and safest option.
For patients who feel strongly against ileostomy the “pouch” is the preferred
option. They must accept the necessity for a temporary ileostomy and there is a
risk of pelvic sepsis complications for some time (due to leaks from the pouch and
creation of pelvic collections). This may be a problem for young women because
of pelvic sepsis may increase the risk of pelvic adhesions and infertility. Also the
patient may accept that they will have some form of , even mild, diarrhoea and
they should examine if this is compatible with their lifestyle. Last but not least,
some pouches fail and ultimately have to be taken down and substituted with a
permanent ileostomy.
Total colectomy and ileorectal anastomosis is a compromise between the two
previously examined options. The whole colon is removed but the rectum is left in
place. The small bowel is joined with the rectum. This results in loose stools but
generally the frequency of diarrhoea is much milder than the ileo-anal
anastomosis – most patients manage to have an acceptable quality of life. A
temporary ileostomy is not routinely required (only in a case of complications)
and therefore only one operation is required.
The disadvantage of ileorectal anastomosis is that, by leaving the rectum in
place, it does not remove the risk of rectal cancer. If this option is chosen then
sigmoidoscopy should be performed every 6 months and any polyps should be
destroyed. There are some available data showing that this policy can be efficient
for some patients and that the risk of rectal cancer on those patients is actually
lower than theoretically anticipated. There are no large series evaluating the long
term safety of this option however it is worth discussing with patients who are not
happy with either of the previous two.
If the option of ileorectal anastomosis is chosen then the patient should, apart
from six-monthly sigmoidosocpy, also have medical treatment with sulindac (an
anti-inflammatory), calcium supplements and vitamin E. This medication
decreases also the risk of duodenal and upper gastrointestinal polyps.
As is obvious above, surgery for Familial Adenomatous Polyposis is complex
major and it involves some important decisions which have to be made jointly by
surgeon and patient. It is important that the patient fully understands the
advantages and disadvantages of each surgical procedure because there is no
ideal option.
(FAP) it will inevitably give rise to cancer. The aim of treatment is to remove all
colonic and rectal tissue in order to prevent this event. The anal canal consists of
a different kind of epithelium (squamous) which is not prone to carcinogenesis by
the FAP genes so it can be left in place. However once the colon and rectum
have been removed we are faced with a dilemma:
-how are we going to restore the continuity of the bowel?
If we join the end of the small bowel on the anal canal, this is described as an
ileoanal anastomosis, then excessive diarrhoea ensues. The small bowel fluid
is very loose and it also contains large quantities of bile which is an irritant for the
skin. Life with an ileoanal anastomosis is of poor quality because of constant
diarrhoea (20 – 40 times per day and night) and anal irritation. Severe
dehydration can happen very easily. Not surprisingly ieoanal anasotmosis is not
an option practiced today.
There is an option of actually not restoring the continuity. We can completely
remove the colon and rectum which is called a panproctocolectomy and
exteriorise the end of the small bowel as an end ileostomy. Ileostomy output can
usually be managed with application of appropriate bags in a way which retains
good quality of life for the patient. Panproctolectomy and ileostomy is a
reasonable surgical solution in that it removes the risk of cancer completely and
preserves good quality of life. However the patient has to live with a permanent
ileostomy. It has been shown in studies that ileostomy does not affect in the long
term quality of life but it does have an impact on the patient’s body image.
Although some patients accept easily the idea of ileostomy there are others who
cannot. The patient’s views and feelings have to be examined thoroughly before
a decision on this operation.
Restorative proctocolectomy and ileoanal pouch (for brevity reasons we will
call this procedure simply a “pouch” for the rest of this text. This operation
consists of removing completely the colon and rectum and then creating a pouch
or reservoir of small bowel by using two or more loops of small bowel. Depending
on the shape of the loops which are put together it is called a “J” pouch, a “W”
pouch or an “S” pouch. The more commonly used today is the “J” pouch.
The pouch decreases the diarrhoea to acceptable levels so that the patient’s
quality of life can be preserved. However patients with a pouch still open their
bowels 4 – 8 times a day. This may be acceptable by some patients but not by
others. The professional occupation of the patient and their lifestyle is a major
factor. Having to go the toilet 4 to 8 times a day may be tolerable to a housewife
or someone who works in an office with good toilet facilities but may be a major
problem for a bus driver. The diarrhoea of “pouch” is sometimes caused by a
condition called “pouchitis”. This is an inflammation of the pouch probably caused
by bacterial proliferation caused by stagnation of the bowel contents. Medication
such as loperamide or the antibiotic metronidazole may improve the frequency of
bowel movements.
In most cases the creation of a “pouch” has to be accompanied by a temporary
prophylactic ileostomy. The ileostomy can be closed 2-3 months later.
The choice between a permanent ileostomy and a “pouch” is not an easy one
and it finally lies with the patient. Both operations have the same efficiency in
curing the risk of colorectal cancer. The advantages of the ileostomy are that it
involves a single operation and does not cause any diarrhoea or pelvic
inflammation problems. In that sense it is an operation suitable for people who
are anxious to put an end to their treatments as soon as possible and get on with
their lives. As long as the patient can accept the idea of living with an ileostomy,
panproctocolectomy is the fastest and safest option.
For patients who feel strongly against ileostomy the “pouch” is the preferred
option. They must accept the necessity for a temporary ileostomy and there is a
risk of pelvic sepsis complications for some time (due to leaks from the pouch and
creation of pelvic collections). This may be a problem for young women because
of pelvic sepsis may increase the risk of pelvic adhesions and infertility. Also the
patient may accept that they will have some form of , even mild, diarrhoea and
they should examine if this is compatible with their lifestyle. Last but not least,
some pouches fail and ultimately have to be taken down and substituted with a
permanent ileostomy.
Total colectomy and ileorectal anastomosis is a compromise between the two
previously examined options. The whole colon is removed but the rectum is left in
place. The small bowel is joined with the rectum. This results in loose stools but
generally the frequency of diarrhoea is much milder than the ileo-anal
anastomosis – most patients manage to have an acceptable quality of life. A
temporary ileostomy is not routinely required (only in a case of complications)
and therefore only one operation is required.
The disadvantage of ileorectal anastomosis is that, by leaving the rectum in
place, it does not remove the risk of rectal cancer. If this option is chosen then
sigmoidoscopy should be performed every 6 months and any polyps should be
destroyed. There are some available data showing that this policy can be efficient
for some patients and that the risk of rectal cancer on those patients is actually
lower than theoretically anticipated. There are no large series evaluating the long
term safety of this option however it is worth discussing with patients who are not
happy with either of the previous two.
If the option of ileorectal anastomosis is chosen then the patient should, apart
from six-monthly sigmoidosocpy, also have medical treatment with sulindac (an
anti-inflammatory), calcium supplements and vitamin E. This medication
decreases also the risk of duodenal and upper gastrointestinal polyps.
As is obvious above, surgery for Familial Adenomatous Polyposis is complex
major and it involves some important decisions which have to be made jointly by
surgeon and patient. It is important that the patient fully understands the
advantages and disadvantages of each surgical procedure because there is no
ideal option.
SURGERY FOR FAMILIAL ADENOMATOUS POLYPOSIS (FAP)
 | |  | |  | |  | |  | |  |