The Place of Proctocolectomy with Ileostomy in the Era of Restorative Proctocolectomy

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Introduction

Over the second half of the last century, proctocolectomy in regards to terminal ileostomy has played an important role in the management of inflammatory diseases affecting the colon and rectum. For a long time it has been considered the gold-standard operation and all the results of alternative procedures have been compared with it. Before the era of restorative proctocolectomy, the possibility of removing all the diseased tissue in a one-stage procedure while avoid- ing the risk of cancer made this technique very successful. However, although this procedure has a low rate of morbidity and mortality and allows the patients an early return to normal activities [1], there are some important drawbacks that have spurred surgeons on to develop restorative proctocolectomy.

First of all, the patients perceive the ileostomy as an unnatural condition which impairs their relation- ships and social life. Although modern stoma care allows an easier management of the stoma appliance, the unpredictable faecal and noisy gaseous discharge may be of such an extent that they restrict patient activities to the point of becoming a psychological barrier. Sexual complaints of the patients depend on both the functional and psychological impairment.

Permanent impotence is rare with the intersphinteric technique and some authors have reported no permanent male dysfunction, either partial or complete, when using a perimuscular rectal excision during the proctocolectomy [2]. Women often complain of vagi- nal discharge and dyspareunia because of the perineal scar. However, psychological lability and the perception of the self-image are the factors that mainly affect sexual intercourse and the relationship of the patient. Moreover, some bladder dysfunctions may occur [3] and the perineal wound healing may take a long time, particularly in patients with unde- tected Crohn’s disease [4]. Finally, the cost of the management of the ileostomy should be considered.

Indications

The choice of the ideal surgical procedure for treating these patients is based upon the following con- siderations:

– the need of definitive treatment with a one-stage procedure that allows a complete removal of the diseased tissue and avoids the risk of cancer.

– The possibility of restoring the anatomy as well as the bowel function and the faecal continence.

– The outcome and the complications of different surgical procedures.

– The patient’s skills in managing the new condition and coping with the possible complications.

– And, above all, the possibility of improving the quality of life of the patient, which implies the evaluation of multiple functional and psychological factors.

Nowadays, restorative proctocolectomy is certainly the gold-standard technique for treating patients with ulcerative colitis or familial adenomatous polyposis [5, 6]. Nevertheless, there are still a few patients in which the proctocolectomy along with definitive ileostomy may be considered a good indication. The proctocolectomy with perineal excision and definitive ileostomy may be an indication of necessity in patients presenting with cancer or severe dysplasia of the very low rectum or anus [7]. In these cases, the choice of this surgical procedure should be based on the tumour stage and the need for radiotherapy. In other cases, the indications are more controversial and are still being debated. Provided that all the alter- natives are discussed with the patient, the decision of the surgeon should be drawn from a sort of balance between the factors mentioned above. In elderly patients with a longstanding disease and a weak sphincter, a one-stage procedure with definitive ileostomy could be beneficial and better accepted by the patient. Similarly, all the patients with sphincter damage, particularly women with post-obstetric neu-

The Place of Proctocolectomy with Ileostomy in the Era of Restorative Proctocolectomy

Giovanni Romano, Francesco Bianco, Vittorio D’Onofrio

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ropathic sphincter trauma, might be good candidates for a definitive ileostomy. In these patients, the excision of the rectum to the level of the perineal muscles and the removal of the diseased anal mucosa could lead to disappointing results in terms of continence with much more discomfort and distress symptoms for the patient than a well-managed ileostomy. All the patients should be correctly informed about the outcome and the possible complications after restorative proctocolectomy such as diarrhoea, noc- turnal soiling and recurrent pouchitis, so that patients who refuse to take these risks can be candidates for a proctocolectomy with a definitive ileostomy. Other patients are too psychologically weak and unstable to cope with the uncertainties of the outcome of a restorative procedure or are not available to attend the strict follow-up that is mandatory when an ileal pouch-anal anastomosis has been performed [8]. Furthermore, the definitive ileostomy, as salvage surgery, constitutes the last option for patients who previously underwent a restorative proctocolectomy in which the ileal pelvic pouch had to be removed [9].

The emergency operations are a particular problem.

In patients with a previously detected severe inflammatory anorectal involvement, continuous bleeding from the ultra-low rectum or a perforation at this site may occasionally constitute an indication for a radi- cal proctocolectomy without anastomotic reconstruction. Nevertheless, some authors suggest that even in these cases the rectum should be mobilised at the level of the levator ani and transected at the anorectal junction so that a pelvic pouch might be subsequently performed [4]. However, it should be stressed that, recently, many authors have reported that, in emergency, the primary procedure is more frequently a total colectomy with an ileostomy. They suggest that in these circumstances the surgeon should do a minimal intervention, and subsequently the patient may undergo a restorative proctectomy or an ileo-rectal anastomosis [10]. Emergency operations certainly have more complications such as a higher risk of fistula and pelvic nerve damage, and in these cases the surgeon’s experience plays an important role in the choice of surgical options [11].

The surgeon and the stoma care nurse should arrange a pre-operative meeting with the patients whenever an elective procedure is performed. In order to make the right choice, the patient and the partner have to be aware of the alternative procedures such as restorative proctocolectomy with an ileal pelvic pouch and total colectomy with ileo-rectal anastomosis. The procedures have to be carefully described to the patients with particular attention to technical details, drawings and literature data. More- over, they should be correctly informed about the quality of life and the possible implications of these

operations. Particularly, some aspects of both the procedures have to be discussed such as complications, continence, soiling, diarrhoea, dietary restrictions, social restrictions and the necessity of long- term follow-up. The patient should be reassured that a stoma care unit is always available for all his needs.

However, above all, the information given to the patients must be as objective as possible since, patients potentially unsuitable for the restorative operation may be persuaded by the excessive enthu- siasm of the surgeons for the pouch procedure [8].

Once a decision to perform a definitive ileostomy has been taken, the surgeon is requested to make a second choice: whether or not to perform a continent reservoir ileostomy according to a modified Kock technique instead of a conventional Brooke ileostomy [12, 13]. Nowadays, the Kock ileostomy is rarely used as the primary treatment for ulcerative colitis or familial adenomatous polyposis due to the other alternative surgical options that are available and the surgical and metabolic complications associated with the reservoir ileostomy [14]. Nevertheless, besides the above-mentioned indications for conventional ileostomy, patients who have a poorly functioning ileoanal pouch and who are unsatisfied with their continence and quality of life, may be suitable for a conversion to a continent reservoir ileostomy [9, 15].

Similarly, the continent reservoir may be proposed to a limited cohort of selected and strongly motivated patients who have previously had a terminal conventional ileostomy. However, it is necessary to stress that there is an important learning curve for this operation and, just because nowadays the indications are rare, only a few centres have acquired sufficient experience with the procedure.

Even in this case, a detailed description of the procedure and its aims should be given to the patient, since a failure of the reservoir exposes the patient to the risk of a reoperation, a further loss of 50–60 cm of the ileum along with consequent metabolic derange- ment and, finally, the need for wearing a conventional appliance for the stoma. Therefore, it is obvious that the possibility of evaluating whether or not the procedure will be successful depends on a clear and objective definition of the aims of the reservoir ileostomy. Some authors have described these aims as the possibility of achieving a pouch completely continent to the gas and faeces with a capacity of 800–1 000 ml. This pouch should be emptied by a catheter no more than two or three times a day, without the urgency of draining at night, and the catheterisation should take no longer than 15 min.

The exit conduit of the stoma should be invisible under the clothes and there should be no need of wearing a stoma bag as the mucous discharge should be minimal and there is complete control achieved by

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the application of a disposable dressing over the stoma. Finally, there should be no restrictions of food intake, sexual activities, work or any other social functions [8].

A careful selection of these patients is of para- mount importance. A psychological assessment should be made in order to verify the real motivation of the patient and the psychological profile, thus excluding psychologically unstable patients who are seeking attention. The social environment should be evaluated as well as the physical skill of the patient to manage the pouch. Some patients have been described as having badly managed and perforated the pouch during catheterisation, others, with psychological lability, manipulate and deteriorate the pouch themselves; likewise, the elderly patients should not be considered for this procedure. Patients with pathological and psychological dietary disturbances such as the alcoholic, the anorexic, the bulim- ic, the obese and the very thin should be excluded.

Particular attention should be paid to the patients who have previously undergone abdominal surgery, because the presence of thick adhesions may be a contraindication. Moreover, previous small-bowel resections, gastrectomy or pancreatic insufficiency are criteria for exclusion since in these cases there is an increased risk of electrolyte deficiency and metabolic complications. Finally, the emergency operation may be considered a contraindication for this procedure.

In 1997, Williams reported ten cases of Kock pouch construction. Four patients had a primary conventional proctocolectomy with a Kock pouch for ulcerative colitis with good long-term results. Six patients who had previously undergone restorative proctocolectomy for ulcerative colitis were converted to reservoir ileostomy. Among these latter, the reservoir was successful in four patients and failed in two:

one due to sepsis and one due to obesity [8].

Although many authors consider a diagnosis of Crohn’s disease as an absolute contraindication for a Kock pouch construction, this particular topic is still being debated [16]. In most of the reported cases, the patients with Crohn’s disease who had a reservoir ileostomy as the primary operation, had a diagnosis of ulcerative colitis at the time of the first operation and, only subsequently, was the underlying diagnosis of Crohn’s disease confirmed. In these patients, the diagnosis is usually achieved by the histological examination of the colon specimen in the early post- operative period or, later—via the analysis of the removed ileal pouch as a consequence of complications. In these latter cases, the histological examination of the colon, at the time of the first operation, has features similar to those of the ulcerative colitis, but the subsequent pouch complications reveals

Crohn’s disease. Some of these patients with Crohn’s disease develop complications in the early post-operative period such as sepsis, fistula, bleeding and poor functional results requiring pouch excision; others, after years of good functional results, begin to have the typical symptoms of pouchitis. These patients complain of abdominal pain, fever, diarrhoea with high volume of fluid discharge and loss of electrolytes, bleeding, obstruction and difficult catheterisation. Frequently, they are classified as having chronic pouchitis and only the endoscopic biopsy allows the correct diagnosis of the underlying Crohn’s disease. In these recurrent forms of Crohn’s disease, medical treatment rarely achieves good results and, often, the patients end up with the removal of the pouch and a conventional ileostomy.

Myrvold and Kock, in their important study that was a milestone in this specific area, reported only 27% of complications out of a total of 52 patients with Crohn’s disease who underwent a reservoir ileostomy. The same incidence of complications was found in patients operated on for ulcerative colitis or familial adenomatous polyposis [17]. Other authors reported similar results in a small subset of patients with Crohn’s disease. These authors suggested that patients with Crohn’s disease confined to the colon and no evidence of disease to the small bowel, after a minimum follow-up of 5 years might be candidates for a reservoir ileostomy. Similarly patients with indeterminate colitis without evidence of disease to the ileum might be suitable for the Kock ileostomy [18]. On the contrary, Handelsman reported four pouch excisions out of eight patients operated on with Crohn’s disease compared to only two pouch excisions in 87 patients with ulcerative colitis, so that he concluded that the suspicion of Crohn’s disease is a contraindication to continent reservoir ileostomy.

Management, Outcome and Complications

The management of a conventional Brooke ileostomy is quite easy, both for the surgeon and the patient.

As soon as possible, the patient is allowed to drink to easily achieve a correct balance of fluid intake.

Beginning in the early post-operative period, a stoma care nurse should instruct the patient and his partner to empty and change the bag and to apply the flange.

Although the rehabilitation mainly depends on patient motivation, the availability of a stoma care unit greatly helps the patient. They have to be informed about the correct volume of fluid intake, the need of wound dressing until complete healing is achieved and the risk of intestinal obstruction due to stoma impairment or post-operative adhesions.

Mortality is related to the proctocolectomy and

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ranges between 2 and 3%, but for emergency procedures, a mortality of 23% has been reported. A depression may occur in the first period probably due to the new perception of the self-image and to the suspension of steroids medication. The most frequent complications in the early post-operative period are perineal sepsis and bleeding from the perineal wound, which are conservatively treated; a persistent sepsis or bleeding from the abdomen or the pelvis may require a second laparotomy. The loss of a large volume of fluid and electrolytes through the ileostomy may represent a serious problem, particularly in the first days after the post-operative ileus has resolved; therefore, it may be necessary to continue intravenous infusion to replace fluid loss. In some cases, chronic ileostomy diarrhoea occurs and the patients need to increase the volume of fluid and electrolyte intake. As the follow-up lengthens, an increased incidence of urolithiasis is reported, probably related to the extent of bowel excision. As in all, the patient who undergoes a bowel resection, an intestinal obstruction due to adhesions may compli- cate the early and late post-operative course; it must be remarked that this occurrence may be disastrous for these patients since a reoperation may lead to a further loss of bowel.

What is more difficult, is the management of a reservoir ileostomy in the early and late post-operative periods; however, the selected patients are usually very strongly motivated to actively cope with the new condition. At the end of the operation, and as long as the ileus has completely resolved, a permanent catheter is placed in the reservoir in order to drain the pouch and avoid disruption, leakage and nipple valve desusception. Subsequently, the catheter is periodically occluded and the time of drainage is progressively reduced. Some irrigation may be necessary to avoid faeces and food particles obstructing the catheter both in the early post-operative period, when the patient starts eating, and then at the time when the pouch is functioning. After a few weeks, the patient becomes aware of the necessity of emptying the reservoir and, usually, this manoeuvre is easily accomplished three or four times a day. Besides the complications already mentioned as being associated with any ileostomy and major laparotomy, there are a few others that are specific to a stoma reservoir construction. Some patients complain of continuous abdominal bloating and pain and others need to spend too much time in frequent catheterisations and irrigations to wash out the pouch from smelly particulate contents. These complaints are likely to be more frequent in patients with slow small-bowel transit. Accordingly, some authors consider that patients having a chronic constipation with a slow bowel transit, for which a conventional total procto-

colectomy has been indicated as the last option, should have a Brooke ileostomy rather than a reservoir ileostomy [8, 19]. In the past years, valve slippage was reported as the most common complication, up to 44% of incidence, but this problem has been almost completely solved by using stapling machines. More important complications are the ischaemia or the fistula of a part of the ileostomy such as the exit conduit, the nipple valve or, at worst, the pouch. The fistula may be a consequence of an ischaemic tract, a suture line dehiscence or a perforation subsequent to catheterisation. Obviously, the most serious complication is the leakage in the abdomen of bowel contents particularly from the pouch since, if the fistula is not immediately recog- nised, the life of the patient may be threatened. In such circumstances, salvage surgery is mandatory. In case of a simple slippage of the valve, a new nipple valve can be refashioned [20]. When the valve or the exit conduit are ischaemic with a possible fistula, or the bowel tract is not sufficient, a reconstruction procedure is needed such as a rotational procedure or an interposed loop, in which a new bowel segment is adopted for the new valve and conduit. However, in the worst cases, the pouch cannot be saved. In these circumstances the reservoir must be entirely removed, and a completely new pouch ileostomy may be performed or, in some cases, a conversion to a Brooke ileostomy. Failes reported a 21% of reoperation rate and most of the revisions were undertaken within 1 year from the first operation [21]. In order to improve the functional results and reduce the early and late complications, over the last years a series of novel techniques for performing a continent ileostomy have been described such as the Barnett reservoir, the T pouch, and the ileocecal valve-preserving ileostomy [14, 22, 23, 24]. These new procedures are reported as improving the continence and the quality of life of the patients. However, although the preliminary reports are encouraging, a long-term follow-up is not yet available for correctly comparing the results.

The incidence of pouchitis has been reported to range between 4 and 40% in patients with a Kock reservoir for ulcerative colitis [25, 26]. The diagnosis is based on the typical above-mentioned symptoms and on an endoscopic biopsy that reveals diffuse bleeding inflammation and/or a villous atrophy.

However, the histological features are often non-specific and may hide Crohn’s disease. It is likely that this pouchitis rate is higher in patients who underwent the same operation for Crohn’s disease than in those with ulcerative colitis, and much lower in patients operated on for familial adenomatous polyposis; besides, the more the follow-up lengthens, the more the incidence of pouchitis increases. In 1993,

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the group from Göteborg University compared the pouchitis rate after a long-lasting follow-up of patients with a Kock reservoir to that of patients with a pelvic reservoir. Pouchitis was found to be more frequent in the pelvic pouches as it occurred in 34%

of the Kock procedures and 51% of the pelvic pouches; and in 64 and 76% of the cases there was only a single episode or a slight and short form of disease [26]. Nevertheless, patients with a Kock reservoir more frequently developed a chronic form of pouch- itis than those with a pelvic pouch: 18 vs. 6%. Pouch- itis is likely to be due to a bacterial overgrowth and a delayed small-bowel transit, and thus in most of the cases it quickly responds to conservative treatment with metronidazole and pouch catheterisation. Some metabolic disturbances such as fluid and electrolyte deficiencies, megaloblastic anaemia due to the deple- tion of vitamin B12, fat or bile-salt malabsorption, as well as villous atrophy, may occur both in patients with pouchitis and those with a normal functioning reservoir. Moreover, the same metabolic complications have been found in patients with a conventional Brooke ileostomy, so it is likely that these metabolic imbalances depend on the volume of the ileostomy output, regardless of the type of ileostomy.

Many authors have reported a better quality of life in patients who underwent a restorative proctocolectomy than those who underwent a conventional proctocolectomy with ileostomy [5, 6]. In 1991, in order to assess whether the improvement of the quality of life in patients with restorative proctocolectomy was due to the absence of a stoma or to a better faecal continence, Kohler and Pemberton examined functional and performance activities in 406 patients with Brooke ileostomies (stoma present, inconti- nent), 313 with Kock pouches (stoma present, continent), and 298 with ileal pouch-anal anastomoses (stoma absent, continent). All the patients had been operated on for ulcerative colitis or familial adenomatous polyposis. Patients with ileal pouch-anal anastomoses had fewer restrictions in sports and sexual activities than those with Kock pouch, whereas those with Kock pouches, in turn, had fewer restrictions in these activities but more restrictions in trav- el than those with Brooke ileostomies. Performance in the categories of family, work and social life were similar between the groups. They concluded that both the presence of a stoma and faecal incontinence impair the quality of life after proctectomy, so therefore the ileal pouch-anal anastomosis allows the best quality of life [6]. On the contrary, more recently, some other authors reached more controversial con- clusions. Mikkola evaluated the clinical differences between conventional and restorative proctocolectomy among 240 patients, the reoperation rate was 38 and 36% respectively; but as major complications

were more frequent in the pouch group, he therefore concluded that ulcerative colitis can safely be managed with either conventional or restorative proctocolectomy, and more remarkable, in most cases the patient’s preference should dictate the choice of procedure [27]. Likewise, in 2003, Camilleri-Brennan [28] analysed morbidity and quality of life in two matched groups of patients who underwent a restorative proctocolectomy or a conventional proctocolectomy with a Brooke ileostomy. The restorative proctocolectomy was found to be associated with a significantly better perception of body image than a permanent stoma, although the quality of life in gen- eral was similar in both groups and the patients with the pelvic pouch had more long-term complications than patients with ileostomy: 52.6 vs. 26.3% respec- tively. Therefore, because of the higher complication rate and the relatively small advantage in terms of quality of life associated with the restorative proctocolectomy, patients should be thoroughly advised before agreeing to this operation [28]. Moreover, conventional ileostomy usually represents the ulti- mate procedure after unsuccessful salvage surgery for patients who have experienced the failure of an ileal anal-pouch. As an alternative, the group from Göteborg University converted 13 patients with a failed previous restorative proctocolectomy to a continent ileostomy with reservoir. Subsequent revisional surgery was required in eight patients but, after a follow-up of 6 years, ten patients with intact ileostomy were fully continent and none had to use a stoma appliance. Provided that this operation should certainly be done in specialised units, it may be considered as an alternative salvage surgery to the Brooke ileostomy in motivated patients [29].

Closing Remarks

Although in the era of restorative proctocolectomy it is hard to accept that a reservoir ileostomy may be a satisfactory alternative to the ileal anal-pouch, it cannot be denied that, recently, many have reported rewarding results with the continent ileostomy even after a long-term follow-up. In 2004, Berndtsson et al.

analysed data from 68 patients operated on with continent ileostomy between 1967 and 1974 to assess the long-term pouch durability and the health-related quality of life. The median follow-up was 31 years.

The majority of patients reported good physical condition and satisfactory pouch function. Patients emptied the pouch a median of four times every day and 65% of patients had at least one post-operative revision to restore continence. The quality of life scores were compared to those of a control group. Seventy- eight percent of the patients rated their overall health

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as good, very good or excellent and the scores were comparable to the reference values [30]. Likewise, in 2005, Castillo reported a retrospective study in which the results of 24 patients operated on with a modified Kock pouch between 1993 and 2003 were evaluated.

The median follow-up was 66 months. The underlying disease was ulcerative colitis in 71% of the patients, 20 patients had already been operated on:

13 patients were converted from a Brooke ileostomy to a continent ileostomy and 7 patients were operated on for a failure of a previous ileal anal-pouch.

Revisional operations were performed in 58% of patients. The failure rate with reconversion to conventional ileostomy was only 8.3%, and 90% of the patients were satisfied with the continent pouch [31].

Although a high rate of reoperations may be needed to restore continence, continent ileostomy has good durability, satisfactory pouch function or quality of life, which are, in most cases, similar to that of the normal population. Therefore, the results reported make this procedure a viable option for patients with a previous restorative proctocolectomy that has failed, or whenever a restorative proctocolectomy is not likely to be advisable for the reasons discussed above. However, the patients must be carefully selected and before performing a reservoir ileostomy, they should always be advised of the high risk of revisional operations. Moreover, despite the high reoperation rate, most patients are reported to be pleased with continent ileostomy and, even in the case of reservoir failure or dysfunction, when they are asked to choose, most of them prefer to cope with revision operations rather than manage a conventional ileostomy for their entire lifetime [8].

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