pouches and stomas

6
Pouches and stomas Myles Fleming Neil Mortensen Abstract A third of patients with ulcerative colitis will need colectomy. This can be followed by ileal pouch formation to enable restoration of normal bowel continuity. Patients with familial adenomatous polyposis may also undergo pouch formation. The commonest long-term problem with pouches is pouchitis though this can often be treated with antibiotics. Stomas come in many forms but the commonest types are ileostomy or colostomy and these can be permanent or temporary. The expertise of a stoma nurse is vital to the management of these patients. Keywords adenomatous polyposis coli; colostomy; ileal pouches; ileo-anal pouches; ileostomy; J pouch; pouchitis; restorative proctoco- lectomy; ulcerative colitis Pouches A pouch, or more correctly an ileal pouch anal anastomosis (IPAA), is formed during a restorative proctocolectomy. During this operation, the entire colon plus rectum from the ileocaecal valve to 2 cm above the dentate line are removed, and an ileal pouch is formed and anastomosed to the ano-rectal remnant. Since this operation was first described in 1978, 1 it is estimated that worldwide over 30,000 pouches have been formed. Reasons for pouch formation The commonest pathologies for forming a pouch are ulcerative colitis (UC) and familial adenomatous polyposis (FAP). Ulcerative colitis: up to one-third of patients with UC may require colectomy long term. This can be in an urgent setting, during an episode of acute severe colitis that has failed to respond to intravenous corticosteroids and rescue therapy; or elective, when medical management has failed to control colitis or dysplastic/cancerous changes have occurred. The cancer risk is related to the degree and extent of inflammation in the colon, the presence of post-inflammatory polyps or colonic strictures, primary sclerosing cholangitis or family history of colorectal cancer. 2 It has been estimated that 18% of patients found to have UC will develop cancer 30 years from diagnosis. 3 Familial adenomatous polyposis: FAP is an autosomal domi- nant condition in which a defect in the adenomatous polyposis gene leads to at least 100 colonic polyps forming in the colon. This can occur as early as the teenage years, with the potential of cancer change by the mid-twenties. As it is not possible to remove all the polyps endoscopically, a prophylactic colectomy has to be performed, normally before the age of 25 years. Patients with a milder phenotype, with a lower polyp count, can be offered deferred surgery or subtotal colectomy with ileorectal anastomosis. Compared to pouch surgery, ileorectal anastomosis gives better bowel function and, as it avoids pelvic dissection, decreases the risk of pelvic nerve injury and/or impaired fertility. The rectal remnant will require regular screening as it has a 12e29% risk of developing cancer change. 2 Contraindications Crohn’s disease (CD): used to be an absolute contraindication to pouch formation as it results in multiple pouch fistulae and high failure rates. However, in patients who have only colonic disease it can be successful. 4 Indeterminate colitis: recently renamed to IBD unclassified (IBDU), is the pathological description given to the 5e10% of IBD patients in whom histology cannot distinguish CD from UC. Many patients with IBDU subsequently turn out to have CD, so it is a relative contraindication to pouch formation. However with careful preoperative assessment to exclude features linked to CD, a good outcome can be achieved. 5 Pouch design J pouch: (Figure 1) this is the commonest type of pouch formed today. The end of the ileum is stapled closed and the small bowel is looped back on itself to create a J that is 20 cm long. An opening is made in the apex of the J to allow the passage of a 10 cm long linear stapler. This is fired twice, converting the parallel lumens into one large lumen. 6 The apex is then anasto- mosed to the rectal remnant. W pouch: (Figure 1) another type of pouch is the W pouch. In this pouch the small bowel is looped three times, so four lengths of small bowel lie next to each other. These are then joined together to form a pouch. The argument for this design is that it gives a large reservoir size, so pouch emptying is less frequent, but in our practice we have found that a J pouch is adequate. S pouch: historically, the first described pouch was an S pouch. In this pouch, the most distal part of the small bowel is anasto- mosed to the anus with the pouch being just proximal. This pouch frequently has problems with pouch emptying and so has fallen out of favour. Rectal remnant There has been debate about the mucosa left in the rectal remnant. As the rectum is normally the worst affected part of the bowel in UC and as in FAP cancer can develop in any colonic mucosa, some have argued that a mucosectomy should be routinely performed. The main benefit of keeping this mucosa is better sensation and less damage to the sphincter, leading to a better functional outcome. 7 Mucosectomy with a hand-sewn anastomosis is also Myles Fleming MRCS is a Surgical Registrar at the John Radcliffe Hospital, Oxford, UK. Competing interests: none declared. Neil Mortensen MD FRCS is Professor of Colorectal Surgery in the Nuffield Department of Surgery, University of Oxford and Consultant Colorectal Surgeon for the Oxford Radcliffe Hospitals, Oxford, UK. Competing interests: none declared. POUCHES AND STOMAS MEDICINE 39:5 259 Ó 2011 Elsevier Ltd. All rights reserved.

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POUCHES AND STOMAS

Pouches and stomasMyles Fleming

Neil Mortensen

AbstractA third of patients with ulcerative colitis will need colectomy. This can be

followed by ileal pouch formation to enable restoration of normal bowel

continuity. Patients with familial adenomatous polyposis may also

undergo pouch formation. The commonest long-term problem with

pouches is pouchitis though this can often be treated with antibiotics.

Stomas come in many forms but the commonest types are ileostomy or

colostomy and these can be permanent or temporary. The expertise of

a stoma nurse is vital to the management of these patients.

Keywords adenomatous polyposis coli; colostomy; ileal pouches;

ileo-anal pouches; ileostomy; J pouch; pouchitis; restorative proctoco-

lectomy; ulcerative colitis

Pouches

A pouch, or more correctly an ileal pouch anal anastomosis

(IPAA), is formed during a restorative proctocolectomy. During

this operation, the entire colon plus rectum from the ileocaecal

valve to 2 cm above the dentate line are removed, and an ileal

pouch is formed and anastomosed to the ano-rectal remnant.

Since this operation was first described in 1978,1 it is estimated

that worldwide over 30,000 pouches have been formed.

Reasons for pouch formation

The commonest pathologies for forming a pouch are ulcerative

colitis (UC) and familial adenomatous polyposis (FAP).

Ulcerative colitis: up to one-third of patients with UC may

require colectomy long term. This can be in an urgent setting,

during an episode of acute severe colitis that has failed to

respond to intravenous corticosteroids and rescue therapy; or

elective, when medical management has failed to control colitis

or dysplastic/cancerous changes have occurred. The cancer risk

is related to the degree and extent of inflammation in the colon,

the presence of post-inflammatory polyps or colonic strictures,

primary sclerosing cholangitis or family history of colorectal

cancer.2 It has been estimated that 18% of patients found to have

UC will develop cancer 30 years from diagnosis.3

Myles Fleming MRCS is a Surgical Registrar at the John Radcliffe

Hospital, Oxford, UK. Competing interests: none declared.

Neil Mortensen MD FRCS is Professor of Colorectal Surgery in the Nuffield

Department of Surgery, University of Oxford and Consultant Colorectal

Surgeon for the Oxford Radcliffe Hospitals, Oxford, UK. Competing

interests: none declared.

MEDICINE 39:5 259

Familial adenomatous polyposis: FAP is an autosomal domi-

nant condition in which a defect in the adenomatous polyposis

gene leads to at least 100 colonic polyps forming in the colon.

This can occur as early as the teenage years, with the potential of

cancer change by the mid-twenties. As it is not possible to

remove all the polyps endoscopically, a prophylactic colectomy

has to be performed, normally before the age of 25 years.

Patients with a milder phenotype, with a lower polyp count,

can be offered deferred surgery or subtotal colectomy with

ileorectal anastomosis. Compared to pouch surgery, ileorectal

anastomosis gives better bowel function and, as it avoids pelvic

dissection, decreases the risk of pelvic nerve injury and/or

impaired fertility. The rectal remnant will require regular

screening as it has a 12e29% risk of developing cancer change.2

Contraindications

Crohn’s disease (CD): used to be an absolute contraindication to

pouch formation as it results in multiple pouch fistulae and high

failure rates. However, in patients who have only colonic disease

it can be successful.4

Indeterminate colitis: recently renamed to IBD unclassified

(IBDU), is the pathological description given to the 5e10% of

IBD patients in whom histology cannot distinguish CD from UC.

Many patients with IBDU subsequently turn out to have CD, so it

is a relative contraindication to pouch formation. However with

careful preoperative assessment to exclude features linked to CD,

a good outcome can be achieved.5

Pouch design

J pouch: (Figure 1) this is the commonest type of pouch formed

today. The end of the ileum is stapled closed and the small bowel

is looped back on itself to create a J that is 20 cm long. An

opening is made in the apex of the J to allow the passage of

a 10 cm long linear stapler. This is fired twice, converting the

parallel lumens into one large lumen.6 The apex is then anasto-

mosed to the rectal remnant.

W pouch: (Figure 1) another type of pouch is the W pouch. In

this pouch the small bowel is looped three times, so four lengths

of small bowel lie next to each other. These are then joined

together to form a pouch. The argument for this design is that it

gives a large reservoir size, so pouch emptying is less frequent,

but in our practice we have found that a J pouch is adequate.

S pouch: historically, the first described pouch was an S pouch.

In this pouch, the most distal part of the small bowel is anasto-

mosed to the anus with the pouch being just proximal. This

pouch frequently has problems with pouch emptying and so has

fallen out of favour.

Rectal remnant

There has been debate about themucosa left in the rectal remnant.

As the rectum is normally the worst affected part of the bowel in

UC and as in FAP cancer can develop in any colonic mucosa, some

have argued that a mucosectomy should be routinely performed.

The main benefit of keeping this mucosa is better sensation and

less damage to the sphincter, leading to a better functional

outcome.7 Mucosectomy with a hand-sewn anastomosis is also

� 2011 Elsevier Ltd. All rights reserved.

Pouch design

Stapled ileal J-pouch Stapled anastomosis to the top of the anal canal

Hand-sewn W-pouch Hand-sewn pouch–anal anastomosis in the mid-anal canal

20 cm

10 cm

Anal sphincter

Dentate line

Anal sphincter

Dentate line

Figure 1

POUCHES AND STOMAS

associated with increased septic complications.8 Furthermore, in

the Cleveland series of pouches, mucosectomy was not protective

against pouch neoplasia in IBD patients.9

Staged surgery

Figure 2 Endoscopic view inside a normal, healthy pouch. The seam of

one of the vertical stricture lines in this J pouch can be seen (lower left).

Though some centres have reported good results from one-stage

surgery, it is more commonly done as a two-stage or three-stage

procedure. This is because anastomotic leak is the major early

complication of pouch formation and can lead to long-term

pouch dysfunction and failure.10

Two-stage: if the patient is having the pouch for UC that has

escaped medical therapy in a chronic setting, or for FAP, surgery

is performed in two stages. The first stage is panproctocolectomy,

ileal pouch formation and a defunctioning loop ileostomy. The

second stage, closure of the loop ileostomy, is performed around

3 months later after a pouchogram has shown no leaks.

Three-stage: if the colectomy is being performed for acute severe

colitis, the procedure is performed in three stages. Here, the first

stage is a subtotal colectomy and end ileostomy with the rectum

left untouched. Several months later, when the patient no longer

requires corticosteroids, the inflammatory response has resolved

and nutritional status has returned to normal, a completion

proctectomy, pouch and defunctioning loop ileostomy is per-

formed. Finally, as with the two-stage approach, the loop ileos-

tomy is subsequently closed.

MEDICINE 39:5 260

Laparoscopic versus open

Increasingly, the colectomy is performed as a laparoscopic proce-

dure. Laparoscopic colorectal surgery has been shown to have

short-term benefits over open surgery, though long-term advan-

tages have been harder to demonstrate. In our experience, like that

of others,11,12 there is a feeling that laparoscopy leads to fewer

adhesions, and so a smaller future risk of adhesional obstruction

and easier pouch formation if the operation is three-stage.

As laparoscopic colorectal surgery has matured, the proctec-

tomy and pouch anal anastomosis has increasingly been formed

laparoscopically.13 However, if difficulties are encountered using

this approach, it is important to convert to open operation, as

leaving a long rectal stump leads to a poorer long-term outcome.

Alternatives

Though pouch surgery is viewed as the gold standard in those

requiring colectomy there is no need to form a pouch if the

patient is happy to be left with an end ileostomy.

Kock pouch: in those who do not want, or cannot cope with, an

ileostomy but are prevented from having an IPAA due to poor

sphincter function, an alternative is the Kock pouch (continent

ileostomy).14 The Kock pouch is similar in shape to an S pouch,

except that the distal end is intussuscepted into the pouch,

forming a ‘nipple valve’. The distal small bowel is then sutured

low on the abdominal wall as a flat ileostomy. The ‘nipple valve’

prevents the stoma discharging any contents unless a Medena

catheter is passed. This form of pouch was first described in 1969

but went out of favour with the creation of the IPAA, since it still

requires a stoma, and because technical difficulties with the

nipple valve led to its slippage and leakage of stoma effluent.

Normal function

A mature well-functioning pouch will empty 5e6 times a day

(Figure 2). The contents are liquid, though after the pouch has

been in place for some years the effluent may thicken. Nocturnal

pouch emptying is common.

� 2011 Elsevier Ltd. All rights reserved.

POUCHES AND STOMAS

Some patients don’t require any dietary modification or

medication but most patients do need to be careful with their diet

(see Table 1), and it is common to use varying amounts of

loperamide to slow pouch emptying.

In some patients, pouch leakage can be problematic and, as

a routine in our centre, all patients have their anal sphincters

assessed before having pouch surgery. This is especially impor-

tant in women who have suffered obstetric trauma, as weak

sphincter function can result in problems with pouch continence.

The UK National Pouch Registry reported nocturnal seepage

occurring in 8% of patients at 1 year, rising to 15.4% at 20

years.15

Fertility and sexual function

All men undergoing pouch surgery need to be warned of the

possibility of erectile dysfunction secondary to damage to rectal

nerves during the rectal dissection. This risk has been reported to

be small,16 but all men at our centre are routinely offered sperm

storage. Male sexual function scores seem unaffected by pouch

surgery.17

Pouch surgery has an adverse impact on female fertility,

although the scale of this is debated. One meta-analysis reported

a threefold increased risk of infertility,18 though a later study

from Finland found that the frequency of childbirth was reduced

only to 80% of that in their control group.19

The mode of childbirth is controversial. Though several

studies report that vaginal delivery is safe after pouch formation,

a third of vaginal deliveries can be associated with occult

sphincter injury, and we believe that there may still be problems

in the long term that have not yet become apparent.8

After pouch surgery, female sexual function scores are worse

than male,17 despite being better than female scores before pouch

surgery.20

Foods that may affect pouch and stoma output

Increase and loosen output Beans, beer, caffeinated

beverages, chocolate, leafy green

vegetables, raw fruits and

vegetables, spicy food,

wholemeal food, cereal, alcohol,

citrus fruits and juice

Decrease and thicken output Apple sauce, bananas, boiled

rice, cheese, smooth peanut

butter, tapioca, white bread,

potatoes, suet pudding, pasta

Increase flatus Beer, carbonated beverages,

dried beans and peas, milk and

milk products, onions, cabbage,

broccoli, sprouts

Can obstruct ileostomy, pouch

inlet or pouch outlet

Mushrooms, sweetcorn, potato

skins, nuts, tomato skins, raw

fruit skins, celery strings

May cause anal irritation Citrus fruits, popcorn, oriental

vegetables, bran, coconut

Table 1

MEDICINE 39:5 261

Long-term complications

Pouch complications are best managed in a stepwise manner.

The first step is clinic assessment with pouchoscopy to assess the

mucosa, size of pouch, length of rectal stump, and presence of

anastomotic stricturing. If needed this can be followed with stool

culture, MRI of the pelvis to check for small abscesses, poucho-

gram to look for sinuses or fistula, and examination under

anaesthetic to assess the pouch more fully.

Pouchitis: the most common problem is pouchitis. This presents

with bleeding (Figure 3), pain and increased pouch frequency. It is

believed to be a reactivation of the immune abnormality that led to

the ulcerative colitis, as the frequency of pouchitis is up to 50% in

those who have a pouch formed for UC but only 10% in those

with FAP.21 In most patients it is an inconvenience that settles

after a two-week course of ciprofloxacin and/or metronidazole.

Chronic pouchitis, which affects 5e10% of patients, is diffi-

cult to manage; options comprise maintenance ciprofloxacin, or

the probiotic, VSL#3,22 which has achieved good results in some

centres. Metronidazole cannot be used long term due the risk of

peripheral neuropathy.

Occasionally, other pouchproblems (such as Crohn’s disease or

bacterial overgrowth) can be misdiagnosed as chronic pouchitis,

so it is important that patients are properly assessed. Importantly,

too small a pouch can present with increased frequency.

Anaemia: vitamin B12 absorption in the terminal ileum can be

disrupted by pouch formation. Iron deficiency anaemia can result

from bleeding secondary to pouchitis.

Fistula: in our practice, we tend to assume that a single fistula is

due to an operative complication, with anastomotic leak as the

commonest cause. If multiple fistulae develop, it is likely that the

initial pathology was wrong and the patient has Crohn’s disease.

Fistulae are managed initially with seton placement. If they

fail to heal, TNF-a antagonists can be tried. Surgical options

include advancement flaps, defunctioning ileostomy or, in the

Figure 3 Acute inflammation of a pouch.

� 2011 Elsevier Ltd. All rights reserved.

POUCHES AND STOMAS

most difficult cases, revision pouch surgery. If the patient has

multiple fistulae, the ultimate result is often pouch excision with

end-ileostomy formation.

Vaginal fistulae have a poorer outcome than other fistulae, only

47% of patients achieving resolution in one reported series.23

Difficulties with emptying: stenosis of the pouch anal anasto-

mosis can result in problems with emptying; this normally

results from the pouch being placed in the pelvis under too much

tension, damage to its blood supply or pelvic sepsis. Another

cause of emptying difficulties is too long a rectal stump.

Both can be managed with intermittent catheterization of the

pouch or pouch revision surgery.

Pouch cancer: the cancer risk is small, with only around 30

cancers reported worldwide.9 Recent guidelines advise 5-yearly

pouchoscopy in UC patients, 10 years from diagnosis, with FAP

patients and UC patients who had dysplasia on colectomy having

annual pouchoscopy.2

Pouch failure: the UK National Pouch registry reported failure

rates following primary pouch formation of 9.3% at 5 years and

16.1% at 10 years, with sepsis being the major cause.15 An older

Cleveland Clinic series showed failure rates of 9% at 5 years and

13% at 10 years.24

Quality of life

Despite the multiple problems that can be associated with

pouches, most patients have good function and would recom-

mend them to others.25,26

Stomas

Stomas, from the Greek stoma meaning mouth, are the exteri-

orization of the bowel through the abdominal wall. They come

in a variety of types and can be either temporary (defunction-

ing) or permanent. They are described according to the part of

the bowel from which they are formed and the material they

pass.

Stoma types/design

Ileostomies: these are made from ileum and pass liquid effluent.

All ileostomies today are Brooke ileostomies where they are

spouted with the mucosa being stitched to the skin surface.27

This prevents the alkaline effluent and activated small bowel

enzymes from contacting the skin, so preventing excoriation.

An ileal conduit, created when the bladder needs to be

removed for bladder cancer, is made from ileum onto which the

ureters are sewn.

Colostomies: colostomies are constructed from colon and pass

formed stool. As stool does not irritate skin, they are formed

flush with the skin surface.

End stoma: stomas can also be further described as being in the

form of an ‘end’ where the stoma has only one opening.

Loop stoma: with a ‘loop’ both proximal and distal parts of the

bowel are brought to the skin surface and are opened next to each

other. Loop ileostomies normally have the proximal end spouted

MEDICINE 39:5 262

and the distal part flat. Loop colostomies are often difficult to

manage, due to their size and (often) awkward position.

Reasons for stoma formation

Cancer: colon cancer operations can result in a stoma. In an

abdominoperineal resection for very low rectal or anal tumours,

the complete rectum and anus including sphincters are removed,

so a permanent end colostomy must be formed.

In low anterior resection for low rectal tumours where the

anastomosis is below the peritoneal reflection, a temporary

defunctioning loop ileostomy is often formed. This is done because

the risk of a leak from the low join is high, so the faecal streamneeds

to be diverted until the anastomosis heals.

Diverticular disease:Hartmann’s operationwas initially described

for rectal cancer but is now more commonly performed for perfo-

rated sigmoid diverticular disease. If, at operation, gross faecal

contamination is found, it is not safe to form an anastomosis as this

will often fail to heal in the presence of bacterial contamination. In

this case, the segment of colon containing the perforation is excised

and an end colostomy is brought out. The distal remnant of colon,

the rectal stump, is left in situ, either closed and free in the

abdominal cavity, closed and subcutaneous, or as amucus fistula in

the bottom part of the midline wound.

Inflammatory bowel disease: the initial surgical management of

acute severe ulcerative colitis is total colectomy and end ileos-

tomy. Ileostomies are also formed during pouch surgery.

In Crohn’s disease, stomas are formed after a pan-

proctocolectomy for Crohn’s colitis, if the rectum needs defunc-

tioning to attempt healing of complex perianal fistulas, or when an

intra-abdominal abscess is found when performing small bowel

resection for strictures or fistulae.

Other causes: stomas can be formed as part of the management of

patients with anal sphincter damage, paraplegia, bowel obstruc-

tion, and before downstaging chemoradiotherapy of rectal cancers.

Normal function

As there is no sphincter mechanism or rectum to store stool,

there is no control over the passage of effluent. An ileostomy

tends to pass its effluent continually as the small bowel has

constant peristalsis, whereas a colostomy is still subject to

colonic clearance waves so effluent is passed intermittently,

usually only a few times a day.

Diet: a variety of foods are recognized as causing loosermotions and

patients should be advised of them (Table 1). If patients find their

ileostomy effluent is too large or frequent, loperamide can be used.

Flatus: flatus passed from an ileostomy results from swallowed

air or carbonated drinks. With colostomies the flatus is mainly

generated by bacterial fermentation in the colon. If flatus is

problematic, root vegetables can be excluded from the diet or

oral charcoal taken.

Stoma nurses

All centres routinely creating stomas should have specialist stoma

nurses. These nurses have taken on a significant part of the

� 2011 Elsevier Ltd. All rights reserved.

Figure 4 Retracted ileostomy.

POUCHES AND STOMAS

workload associated with the preoperative and postoperative

management of stomas.

Before any surgery involving possible stoma formation, a stoma

nurse should counsel the patient. Patients should also be preoper-

atively marked, to avoid placing the stoma in a skin fold or an area

that is difficult to access.

Postoperatively, stoma nurses help to educate the patient

about stomas and the changing of stoma appliances. They can

subsequently offer advice if the patient develops difficulties and

advise on the large variety of stoma appliances.

Long-term complications

High output: this is usually a problem only with ileostomies, as

they are proximal to the absorptive function of the colon. It can lead

to marked and rapid fluid and electrolyte imbalance e particularly

during bouts of infective gastroenteritis. High output stomas can

result from a short proximal length of small bowel or diseased

proximal small bowel (for example, in Crohn’s disease).

Psychological morbidity: stomas can be associated with signif-

icant psychological morbidity and problems with body image,

which can be helped by giving appropriate information and

counselling before their formation and support after.

Parastomal hernia: this results from protrusion of bowel along-

side the stoma through the opening in the muscular abdominal

wall. Incidences of up to 50% have been reported in the literature.

They canmake fitting of a stoma appliance difficult, increasing the

chances of a leak, and can cause pain from stretching of the

mesentery or pressure affects on the abdominal wall. They can

prove difficult to fix as they have a high rate of recurrence.

Stenosis/retraction: (Figure 4) this can occur due to a poor

blood supply to the stoma or if it was formed under too much

tension. It is managed conservatively, with dilatation or by

surgical revision.

Prolapse: this is mainly seen with ileostomies, where the spout

elongates. If problematic it requires surgical repair.

Skin irritation: the solid stool from colostomies rarely causes

problems. However ileostomy effluent is very irritating and can

MEDICINE 39:5 263

cause marked skin excoriation. Barrier creams and films can help

mild cases. Stoma nurses can assist with stoma bag fitting but

a poorly spouted stoma will require surgical revision.

Bleeding: granulation tissue, which readily bleeds, can develop

at the mucocutaneous junction. It can be managed with silver

nitrate cauterization. If no external tissue can be seen, it will

need investigation with endoscopy via the stoma.

Bowel obstruction: adhesional obstruction is the commonest

cause and is normally treated with ‘drip and suck‘. If the

obstruction fails to resolve, surgery can be performed but runs

the risk of forming new adhesions. Infrequent causes include

internal hernia or food bolus, the latter causing obstruction

where the stoma passes through the abdominal wall. A

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