In the past, proctocolectomy (complete removal of the colon and rectum, Table 42.2) with permanent ileostomy was the standard operation for UC and in many ways remains the benchmark by which all other operations are compared. The operation removes the organ responsible for nearly all of the symptoms of the disease and can be performed in even the sickest patients with a low complication rate and negligible mortality. Patients are usually able to resume normal activities fairly soon after surgery and most adapt well to having an ileostomy. Nevertheless, many patients find the idea of a permanent ileostomy objectionable or even unbearable, which led surgeons to develop operations that remove the colon and rectum but allow near-normal bowel function without the use of a permanent ileostomy – the ileal pouch-anal anastomosis (IPAA). In current practice, the operation is typically performed in two or sometimes three stages, depending on the certainty of the diagnosis, the overall health of the patient, and whether the procedure is performed electively or more urgently.

Most patients with UC who require an urgent operation are offered abdominal (subtotal) colectomy, in which the surgeon removes the colon, creates an ileostomy, and closes the intra-abdominal end of the rectum (Hartmann procedure). The rectum is preserved so that a restorative procedure can be performed on an elective basis after the patient has stabilized and can be prepared properly for such a delicate and demanding operation. Urgent colectomy is traditionally performed through a long midline incision but at some centers is routinely done laparoscopically. The principal risks are surgical site infection and bleeding, though the majority of children do well and recover very quickly. The advantages of a laparoscopic approach include smaller incisions, a shorter recovery time, a more rapid return to normal activities, and less scarring [9–11]. Regardless of the technique, the goal of this urgent operation is to remove most of the diseased colon as quickly and as safely as possible and to allow the patient to return to a state of good health until a more definitive restorative operation can be performed electively 2–3 months later. It also provides a surgical specimen that can be examined histologically when the true diagnosis remains uncertain.

Currently, the most popular restorative operation for children with UC is ileal pouch-anal anastomosis (IPAA): the colon and rectum are removed, the ileum is brought down through the pelvic floor musculature, and an anastomosis is created between the ileum and the anus. This creates an ileal reservoir that is intended to function as a replacement for the rectum. The ileum may be unmodified (straight pull-through) or can be fashioned to create a more capacious reservoir, the ileal pouch. The most commonly used pouch configuration is the J-pouch, in which the ileum is folded back on itself for a distance of approximately 8–12 cm and the common wall is obliterated using a surgical stapling device (Fig. 42.1). Other options include the S-pouch, in which the ileum is folded twice to create a reservoir that is three times the diameter of the ileum, and the W-pouch, in which the ileum is folded yet again, resulting in an even larger reservoir. In general, the larger the pouch, the sooner the patient achieves a pattern of relatively normal bowel habits, but the higher the degree of stasis and bacterial overgrowth, factors that many believe increase the likelihood of pouch inflammation and infection. Given its relative ease of construction and proven track record of excellent functional results, most surgeons currently prefer the J-pouch.

There are two accepted methods of creating the ileoanal anastomosis, both of which produce excellent results. Although the decision is based on the preference of the operating surgeon, it is an important consideration, especially in children. One involves mucosal proctectomy with a true ileoanal anastomosis; the other is total proctectomy with double-stapled anastomosis, in which case the anastomosis is made between the pouch and the rectum 1–2 cm above the start of the anal mucosa (Fig. 42.2). Mucosal proctectomy involves dissecting along a submucosal plane and removal of the rectal mucosa all the way down to the anal transition zone, with circumferential preservation of a short portion of the muscular wall of the rectum. This was originally designed as a way to remove the mucosa and submucosa, which is where the inflammation in patients with UC is found, while preserving the presumed motor and sensory function of the rectal musculature. The submucosal dissection can be arduous and rather difficult, especially in patients with severe or long-standing rectal inflammation. The ileoanal anastomosis was traditionally created using a hand-sewn technique through the anus, which is laborious but effective. Recently, a new technique has been developed that allows the creation of a circular stapled anastomosis of the pouch to the anal mucosa without having to leave a remnant of diseased rectum [12].

The classic double-stapled approach involves removing the entire thickness of the rectum, which is transected using a linear stapling device somewhere above the anal transition zone. An ileorectal anastomosis is then created using an end-to-end circular stapling device, creating a circular anastomosis. The advantages of this approach include an easier plane of dissection, a less anal sphincter dilatation (placing anastomosis stitches by hand transanally requires more anal stretching than simply inserting a stapling device), and a shorter operating time. The functional results also appear to be the same or better than those achieved using the rectal muscle-sparing hand-sewn technique. An important distinction, however, is that the traditional double-stapled technique necessitates the retention of a short segment of rectal mucosa, usually approximately 1–2 cm in length, which is at risk for ongoing inflammation and malignant degeneration [13, 14] (Fig. 42.2). Although it is recommended that all patients be followed indefinitely regardless of the surgical technique used, those with retained rectal mucosa require more diligent surveillance, including frequent routine biopsies, for their entire lives, which is probably why it is the less attractive alternative for younger people [15].

At the time of ileoanal reconstruction, surgeons have traditionally also performed a temporary ileostomy, which is supposed to decrease the risk of anastomotic leak, pelvic abscess, and other postoperative septic complications. These complications have been associated with poor pouch function and a significantly diminished long-term quality of life [16, 17]. Although pelvic sepsis is relatively uncommon even without diversion, some surgeons prefer to minimize the risk as much as possible by performing a temporary ileostomy. Others feel that the risk of a severe complication is low enough that protective ileostomy can be avoided in certain patients [18, 19]; however, this remains somewhat controversial. The ileostomy can usually be reversed 6–8 weeks after surgery, usually after a water-soluble contrast enema confirms good healing, a normal pouch configuration, and good evacuation (Fig. 42.3). For most patients, this will be the second or third stage of the operation, depending on whether a subtotal colectomy was done at the first stage.

A procedure that is rarely performed anymore but deserves mention is the Kock pouch (or continent ileostomy) operation [20, 21]. The colon and rectum are completely removed, and the ileum is used to create a reservoir that resides within the abdomen. The end of the ileum is brought out through a small abdominal incision in the manner of an ileostomy, but a small intussusception is created just proximal to the outlet, essentially creating a valve that prevents the leakage of stool. The patient does not wear a standard ileostomy appliance and instead uses a plastic tube to evacuate the pouch several times daily as needed. The concept is certainly appealing; however, the long-term results of the Kock pouch have been somewhat disappointing and the complication rate has been unacceptably high in most centers. The procedure has for the most part been abandoned, except at a small number of institutions worldwide.

Recent worldwide experience has confirmed that children with UC whose disease is refractory to medical management are best served by the combination of colectomy, proctectomy, and ileal pouch-anal anastomosis, with or without a temporary protective ileostomy [22, 23]. Though classically considered a three-stage operation, and still perhaps best conceptualized as a three-stage operation, there is sometimes pressure, mostly from patients and their parents, to combine all three stages in a single operation, namely, colectomy, proctectomy, and IPAA without a temporary protective ileostomy (Fig. 42.4). There are reports in adults and in children that a one-stage approach is generally safe and appears to produce similar long-term results and functional outcomes as the two- or three-stage approaches [24, 25]. These studies are certainly promising and somewhat compelling; however, it must be noted that a true side-by-side comparison or randomized trial has not been, and probably never will be, done. This means that there is likely some selection bias on the part of surgeons who must decide whether they feel comfortable proceeding with a single-stage procedure or not. The decision is complex and involves consideration of several factors: (1) the overall health status of the patient, especially nutrition and corticosteroid dependence; (2) whether the operation is being performed electively or emergently; (3) confidence in the diagnosis (UC or Crohn disease); (4) intraoperative factors such as the length and difficulty of the operation, blood loss, degree of soiling of the pelvis with rectal contents, blood supply of the ileal pouch, and degree of tension at the anastomosis; and (5) recent administration of biological agents such as infliximab – some [26, 27] but not all [28, 29] studies suggest an increased risk of surgical complications for up to 8 weeks. There is also the overriding concern, based on the early published series of the results of IPAA, that pouch complications such as leak and pelvic sepsis diminish pouch function for the life of the patient, [16, 17] which in children could reasonably be expected to be more than 50 years after the operation. Therefore, because most pediatric surgeons would consider long-term functional results more important than the short-term inconvenience of multiple operations or time with an ileostomy, they are more likely to prefer to err on the side of caution. Nevertheless, the progressive pediatric surgeon should evaluate the published data objectively, consider the individual risk factors and overall status of the patient on a case-by-case basis, and discuss all options with patients and their families in a frank but compassionate manner.

Patients with UC who are nutritionally robust and whose symptoms are relatively well controlled tend to be considered responsive to medical management and therefore are not considered candidates for surgical intervention. As a result, those patients who are considered for surgery tend to be acutely ill (bleeding, pain, intractable diarrhea) or severely chronically unwell (malnutrition, corticosteroid dependency, growth failure). These patients are perhaps best served by abdominal colectomy, preferably performed laparoscopically, and temporary end ileostomy. The vast majority feel better immediately, wean off corticosteroids (and all medications) rapidly, gain weight nicely, and are pleased to have had the operation. In patients for whom the diagnosis remains uncertain, this also provides a large specimen (the colon) to examine carefully for signs of Crohn disease without having “burned a bridge” by removing the rectum. (Interestingly, very few patients have symptoms due to inflammation in the diverted rectum even off all systemic medications.) On the other hand, under these same circumstances, there are some surgeons who would consider going ahead with proctectomy and IPAA and perhaps even consider avoiding the ileostomy. Provided that parents understand the risks of short-term complications are probably higher and that long-term pouch function could be affected, this might be a reasonable approach in select cases.

In some cases a compromise in the form of a two-stage approach is a reasonable option. The traditional two-stage approach is to combine the colectomy and proctectomy/IPAA but to perform a temporary protective ileostomy. (This is also the fallback position when one believes a one-stage procedure is feasible going in but then encounters technical difficulties or is unhappy with the viability or tension of the pouch during the operation.) Then again, some surgeons have found that if they perform abdominal colectomy up front and allow the patient to become healthy and come off all medications, the proctectomy/IPAA becomes technically much easier, and they are therefore more likely to forego the ileostomy. Other advantages of performing the ileostomy after the first operation include better patient acceptance of an ileostomy at a time when they are ill but are expected to begin to feel better (they often associate their improved sense of well-being with the ileostomy rather than with the colectomy); the diverted rectum is “cleaner” and often less inflamed at the time of proctectomy, and it avoids having to create a mid-ileal anastomosis at the time of the ultimate ileostomy closure – the ileostomy placed after colectomy is an “end” ileostomy, which ultimately becomes part of the pouch, while the ileostomy placed after proctectomy/IPAA is a “loop” ileostomy, closure of which entails a bowel anastomosis and potentially the increased risk of subsequent stricture or adhesive bowel obstruction.

Finally, though it might be considered “old-fashioned” and it certainly should no longer be considered the standard approach in children with UC, it is reasonable to recommend a three-stage operation when appropriate [30]. The chronically ill patient who has intraoperative complications or a pouch that is under significant tension is probably not best served by a heroic attempt at a one-stage operation. As always, surgical decision making for patients with UC demands careful consideration of the overall balance of potential risks and anticipated benefits. The opinions of patients and their parents should also be considered whenever possible.

Patients who need an urgent operation are prepared for surgery in the usual fashion, with intravenous fluid resuscitation and broad-spectrum prophylactic antibiotics. Patients who are anemic may require a blood transfusion, depending on the surgical and anesthetic standards of the institution. Typically 1–2 units of packed red blood cells are made available for possible use during or after the operation. For patients receiving corticosteroids, it is still standard practice at some institutions to administer a “stress dose” of corticosteroids. Lastly, if time allows, the patient should be evaluated by an enterostomal therapist so that an optimal ileostomy site can be identified and marked.

Patients who are being prepared for an elective procedure should have a formal nutritional assessment. Moderate to severe malnutrition prolongs healing and increases the risk of complications after major surgery. Enteral or parenteral nutritional supplementation is sometimes necessary, even if this means delaying the operation for several weeks. Given that chronic, high-dose corticosteroid therapy can also adversely affect wound healing and increase the risks of an operation, attempts should be made to gradually decrease the dose for patients who are scheduled for surgery, preferably down to 15–20 mg of prednisone daily. Although gradually falling out of favor for most colorectal operations, some surgeons still believe that a mechanical bowel preparation decreases the risk of septic complications after major colorectal surgery. A typical regimen includes a clear liquid diet for 24–48 h and the administration of an osmotic laxative such as polyethylene glycol or magnesium citrate solution. Antibiotics are given intravenously immediately before incision, but oral antibiotics are also sometimes used. There are recent data that suggest bowel preparation does not improve the outcome for most kinds of elective colorectal surgery [31, 32], but the potential benefit in patients who undergo proctectomy and ileoanal reconstruction is not well known.

The technical results of the operations described for children with UC are generally quite good. Infectious complications and bleeding are uncommon and usually easily managed without sequelae. Even after the most complicated operations, most children recover nicely and are able to tolerate a regular diet within a few days of surgery. The short-term results for patients who undergo a minimally invasive procedure might be slightly better, with the added benefit of improved cosmesis [9–11]. Regardless of the technique, the overall risk of serious complications or death is very low.

The functional results of IPAA are generally very good, though there is a great deal of variation between patients and in the same patient over time. The ultimate goal of surgical intervention is for the patient to enjoy a normal lifestyle; however, there are inherent limitations in duplicating normal rectal function with a surgical construct [33]. The ideal functional result of IPAA includes (1) fecal continence day and night, (2) four or fewer stools throughout the daytime, (3) no more than one stool at night, (4) the ability to postpone evacuation for 30 min, and (5) the ability to distinguish between flatus and stool.

The J-pouch IPAA is still the most popular operation for children and adolescents with UC who need surgical intervention, and several large studies have confirmed that the majority of patients have good functional results [25, 34–38]. In most large series, patients report an average stool frequency of three per day and once or none at night. Fewer than 5% have soiling or staining, most of which occurs only at night. Approximately 90% of patients can delay defecation for at least 30 min, and most report being able to pass flatus without accidents. Many patients are able to participate in a wide variety of normal activities including athletics [36]. Studies using patient questionnaires document a very good quality of life for the majority of patients after IPAA with 90–95% of patients reported to be satisfied or very satisfied with the results of their operation [39, 40].

Because the anal dilatation required during completion of the mucosectomy and creation of the ileoanal anastomosis might result in injury to the anal sphincter, some studies have assessed anorectal function after IPAA using rectal manometry [41, 42], though few have included children [43]. Most studies confirm that although there is often a decrease in resting sphincter pressure and maximum squeeze pressure after IPAA relative to preoperative controls, these values gradually return to normal when patients are followed for more than a year. Furthermore, although the rectal inhibitory reflex is often noted to be absent, nearly all patients regain normal rectal sensation for the presence of stool.

Most large series of patients who undergo IPAA report good results regardless of pouch anatomy or method of pelvic dissection. Some surgeons continue to advocate the use of a straight ileoanal anastomosis [44, 45]. The purported benefits of avoiding a pouch include its relative ease of construction, the creation of less tension on the small bowel mesentery, and decreased stasis, which is thought to increase the risk of pouchitis. Nevertheless, the disadvantages of the procedure, including increased frequency and urgency of defecation, are felt by most surgeons to outweigh its potential advantages. A number of patients with a straight pull-through ask to be converted to a J-pouch because of the unacceptably high frequency of stooling [35]. When compared with colectomy and either Brooke or Kock ileostomy, most studies report improved functional results and quality of life in patients who have undergone colectomy with IPAA [46, 47]. However, at least one group has suggested that it is the colectomy that is responsible for most of the improvement in the quality of life for patients with UC and that restoration of normal defecation actually contributes very little [39].