In the past, proctocolectomy (complete removal of the colon and rectum, Table 38.2) with permanent ileostomy was the standard operation for UC, and to this day 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 being done electively or as an emergency.

Proctocolectomy with IPAA is a definitive operation for patients with UC; however, the clinical circumstances might dictate that a lesser operation be performed, at least initially. For example, a diverting ileostomy might be considered in a patient with severe and intractable colitis of unclear etiology. This is rarely indicated except perhaps in the unusual situation in which a young child has severe colitis, the diagnosis is uncertain, and an adequate trial of medical therapy has not been possible due to rapidity of onset. In these situations, it may be difficult for parents to accept the idea of taking such a dramatic and irrevocable step as colectomy. Patients sometimes improve dramatically after ileostomy diversion, but clinical decision making often becomes very difficult. Simple reversal of the ileostomy almost always results in prompt recurrence of symptoms and the true diagnosis may remain elusive. In these patients, one should anticipate that another surgical procedure will eventually need to be performed, usually involving removal of the colon. If the diagnosis is confirmed to be UC, the recommended operation is proctocolectomy with IPAA. The diagnosis of Crohn disease or indeterminate colitis might be an indication for partial colectomy and a restorative operation in which the rectum and part of the colon are preserved (ileocolostomy or ileorectostomy).

Most patients with UC who require an urgent operation are offered abdominal (or 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, with the idea that a restorative procedure will be able to 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 can also be 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]. Disadvantages might include longer operating times and greater cost. 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. It also provides a surgical specimen that can be examined histologically when the true diagnosis remains uncertain.

Despite the fact that the rectum is not removed, patients usually do quite well after abdominal colectomy. After approximately 4–6 weeks, plans can be made for restorative proctectomy and construction of an ileal reservoir. In the past, some patients were given the option of ileorectostomy, in which the rectum is preserved and an anastomosis is created between the ileum and the rectum. This preserves relatively normal rectal sensory and motor function but also retains the rectal mucosa, placing the patient at risk for persistent proctitis and carcinoma. These patients require frequent and meticulous endoscopic surveillance for dysplasia for the rest of their lives. Because of concerns about the risk of cancer and the burden of a lifetime of surveillance, ileorectostomy is no longer considered a suitable option for the definitive treatment of UC in children. However, due to a higher risk of infectious complications and fistulizing perianal disease after ileal reconstruction of the rectum, it is a reasonable consideration for children with Crohn disease or indeterminate colitis.

Currently, the most popular restorative operation for children with UC is 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 so as 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 6–10 cm and the common wall is obliterated using a surgical stapling device (Fig. 38.1). 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 ileo-anal 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 hand-sewn ileo-anal anastomosis; the other is total proctectomy with double-stapled anastomosis (Fig. 38.2). 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 ileo-anal anastomosis is then created using a hand-sewn technique through the anus. The submucosal dissection can be arduous and sometimes difficult, especially in patients with severe or long-standing rectal inflammation.

The double-stapled approach involves removing the entire thickness of the rectum, which is transected using a linear stapling device just above the anal transition zone. An ileo-anal anastomosis is then created using an end-to-end stapling device, which creates a circular anastomosis. The advantages of this approach include an easier plane of dissection, 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 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 (Fig. 38.2) [12, 13]. 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 [14]. At the time of ileo-anal 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 quality of life [15, 16]. Although pelvic sepsis is relatively uncommon even in the absence of fecal diversion, many surgeons prefer to minimize the risk as much as possible by performing a temporary ileostomy. Some surgeons feel that the risk of a severe complication is low enough that protective ileostomy can be avoided in certain patients [17, 18], 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. 38.3). 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 [19, 20]. 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, effectively creating a valve designed to prevent 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 IPAA, with or without a temporary protective ileostomy [21, 22]. Though classically considered a three-stage operation—and still perhaps best conceptualized as a three-stage operation—there is increasing 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. 38.4). 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 [23, 24]. 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, 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 to proceed 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) intra-operative 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, degree of tension at the anastomosis, and (5) recent administration of biological agents such as infliximab—some [25, 26] but not all [27, 28] studies suggest an increased risk of surgical complications for up to eight weeks. There is also the over-riding 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 [15, 16], 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 more 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 probably should no longer be considered the standard approach in children with UC, there is no shame in recommending a three-stage operation when appropriate. For example, the chronically ill patient who has intra-operative complications or a pouch that is under a dangerous amount of 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 (Table 38.3), namely 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. At a minimum, two units of packed red blood cells should be made available for possible use during or after the operation. For patients receiving corticosteroids, it may be 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 be formally assessed for the possibility of chronic malnutrition, which prolongs healing and increases the risk of complications after major surgery. Depending on the degree of malnutrition, 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 less than 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 a cathartic 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 [29, 30], but the potential benefit in patients who undergo proctectomy and ileo-anal 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 patients recover nicely and are able to tolerate a regular diet within seven days of surgery. The short-term results for patients who undergo a minimally invasive procedure might be slightly better and have 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 the IPAA procedures 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 be able to enjoy a normal lifestyle; however, there are inherent limitations in duplicating normal rectal function with a surgical construct [31]. The ideal functional result after an IPAA procedure can be summarized as (1) fecal continence during the day and at night, (2) four or fewer stools during the day, (3) no more than one stool at night, (4) the ability to postpone defecation for at least 30 min, and (5) the ability to distinguish between flatus and stool.

The J-pouch IPAA is currently the most popular operation for children and adolescents with UC who need surgical intervention, and several large studies in adults and children have confirmed that the majority of patients have good functional results [32–37]. In most large series, patients report an average stool frequency of three per day and once 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 a bowel movement. Many patients are able to participate in athletics and report being able to participate in a wide variety of normal activities [35]. Through the use of patient questionnaires, several studies have documented a very good quality of life for a majority of patients after IPAA with 90–95% of patients reported to be satisfied or very satisfied with the results of their operation [38, 39].

Because the anal dilatation required during completion of the mucosectomy and creation of the ileo-anal anastomosis might result in injury to the anal sphincter, some studies have assessed anorectal function after IPAA using rectal manometry [40, 41], although few have included children [42]. Most studies confirm that although there is usually 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 after surgery. Furthermore, although the rectal inhibitory reflex is often noted to be absent, nearly all patients regain normal rectal sensation for the presence of stool.