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Restoration of the continuity of the intestinal tract is one of the key concepts for maintaining the quality of life in patients with Crohn's disease. Restorative procedures have an important role in the scope of operative modalities for these patients. The authors review operative options aimed at fulfilling these goals including restorative partial small bowel resection; segmental, subtotal, and total colectomies; and ileal pouch anal anastomosis in patients with Crohn's disease.
Crohn's disease is a chronic, relapsing, incurable inflammatory condition that can affect the entire digestive tract, which is characterized by transmural inflammation and skip lesions. Indications for surgical intervention include acute and chronic complications of the disease such as toxic colitis, megacolon, perforation, hemorrhage, fistula, obstruction, stricture, carcinoma, and cases of failed medical therapy.1,2 Whenever surgical treatment is indicated, it is crucial to consider that the disease cannot be cured medically or surgically. Consequently, any type of treatment (medical or surgical) is directed at appropriately controlling symptoms, improving quality of life, and preserving the length and continuity of the intestinal tract.
The majority of patients with Crohn's disease ultimately require surgical intervention at some point in their life. In 1979, the National Cooperative Crohn's Disease Study3 reported that the risk of a patient with Crohn's disease to undergo any surgical procedure was 78% and 90% at 20 and 30 years from diagnosis, respectively. These numbers may have decreased slightly with the widespread use of newer medical agents; however, this risk has not yet been eliminated. The relapsing and chronic nature of the disease is also demonstrated by the substantial recurrence rates following surgical procedures. Froehlich et al4 reported that one year following the initial resection, up to 80% of patients have endoscopic signs of recurrence, 10 to 20% have clinical relapse, and ~5% of patients have surgical recurrence.
Excluding anorectal procedures, surgical options in patients with Crohn's disease can be broadly divided into procedures in which a segment of intestine is resected, or nonresectional procedures (internal bypass, fecal diversion, strictureplasty). The resectional procedures can be further divided into restorative or nonrestorative procedures. Nonrestorative procedures (construction of a stoma) are preferred by most surgeons in emergency operations, in cases of fecal peritonitis, and in cases in which the patient is hemodynamically unstable, severely malnourished, or otherwise critically ill.
The focus of this review is restorative operations in patients with Crohn's disease.
The Vienna Classification5 is the most widely accepted classification scheme for Crohn's disease. It incorporates age at diagnosis (A1: <40 years, A2: ≥40 years), location (L1: terminal ileum, L2: colon, L3: ileocolon, L4: upper gastrointestinal), and behavior of the disease (B1: nonstricturing, nonpenetrating, inflammatory; B2: stricturing; B3: penetrating). Approximately 20 to 30% of patients have Crohn's colitis, 20 to 30% of patients have Crohn's ileitis, and 50% of patients have ileocolitis—disease that involves both the terminal ileum and the colon. When surgical treatment is indicated, all of these factors should be taken into consideration.
Isolated disease of the small bowel that necessitates surgical resection should theoretically be approached in a routine manner: The diseased segment of small bowel should be resected, with construction either of an anastomosis or of a stoma. However, there are several issues that require special consideration.
For decades, the “appropriate” margin of resection was a debatable issue. Initially, only margins that were pathologically free of Crohn's disease were considered adequate; surgeons sent resected specimens for intraoperative frozen section examination until clear histologic margins were ultimately achieved. Such practice resulted in extensive resections, especially of small bowel, with significant risk of subsequent short bowel syndrome. The length of the resected specimen and the margins of resection are more important when resecting small bowel because the issue of short gut syndrome usually does not apply to the length of the large bowel. In 1980, Pennington et al6 compared the anastomotic recurrence rates of Crohn's disease in patients in whom the histologic margins were free, to the rates of recurrence in patients in whom the margins had histologic signs of Crohn's disease, and demonstrated that recurrence rates were similar in both groups irrespective of the histopathology of the resected margin. The authors concluded that the findings supported conservative resection to achieve grossly uninvolved margins rather than sacrificing functional intestine in an attempt to achieve histopathologically uninvolved margins. Subsequently, similar findings were reported by other groups7,8,9; with the realization that Crohn's disease is a panenteric inflammatory process, most surgeons who currently operate on patients with Crohn's disease, perform intestinal resection in macroscopically normal margins.
The characteristic thickened Crohn's mesentery can preclude optimal visualization and control of the mesenteric vessels during the resection, which may increase the risk of a mesenteric hematoma. This potential problem can be managed either by serial overlapping clamping of the mesentery of the excised bowel, or by dividing the mesentery with an energy-based vascular sealing device. The thick mesentery can also make laparoscopic resection difficult.
The most common configurations of a small bowel and a small-to-large bowel anastomosis are a stapled side-to-side or a sutured end-to-end anastomosis, according to the surgeon's preference. Several studies have reported that stapled anastomoses resulted in significantly lower rates of disease recurrence10,11 and anastomotic leaks12; however, stapling may not be possible or safe in cases where the intestinal wall is significantly thickened, in which case suturing may be a better option.
It is not uncommon that adjacent normal bowel and other structures (bladder, uterus) are affected by adhesions or internal fistulas, and it is sometimes very difficult to distinguish the diseased loop of bowel from the “innocent bystander.” As a general principle, normal small bowel should not be resected. A fistulous tract targeting a normal structure should usually be excised either by a simple wedge resection and closure, or by a limited segmental resection and anastomosis. An extensive adhesiolysis usually is not required unless treatment of an interloop abscess is attempted.
The terminal ileum is the most common site of Crohn's disease and concomitant cecal involvement is not uncommon. In cases where the cecum is not involved, the diseased terminal ileal segment might be too distal to allow a safe stapled enteroenterostomy with macroscopically normal distal margins. Therefore, a handsewn enteroenterostomy or a stapled ileocolic (or ileocecal) resection should be performed, with the latter being the most commonly performed procedure for this distribution of the disease. The principles of resection and anastomosis that were described in the previous section apply to ileocolic resection as well. Nonaffected ascending colon usually should not be resected, and the newly constructed anastomosis should be physically separated from the underlying duodenum to reduce the risk of complex fistulas in cases of recurrent anastomotic disease; this goal can be accomplished by positioning the omentum between the anastomosis and the duodenum.
Approximately one third of patients with Crohn's disease have isolated colitis or proctocolitis. The optimal surgical approach in these patients is controversial. It seems logical that patients with relative rectal sparing and limited segmental, localized colitis should undergo a segmental colectomy; however, due to the recurrent nature of the disease, additional subsequent operations may be required. Conversely, patients with diffuse proctocolitis should undergo a total proctocolectomy, which will eliminate the possibility of future colitis, but will also subject the patient to a permanent ileostomy. In addition to the extent of the disease, important factors to be considered are the patient's nutritional status, number and extent of prior resections, and whether the patient has fecal incontinence. Variables such as patient's age, comorbidities, and urgency of the procedure should also be taken into account.
As mentioned above, the decision as to which procedure to perform is an individualized process that should be independently tailored to each patient. A hypothetical elderly patient with localized colitis and preexisting fecal incontinence may have worse functional outcomes following a segmental colectomy, whereas a young patient with diffuse colitis, adequate anorectal function, and relative rectal sparing may benefit most from a total colectomy with an ileorectal anastomosis.
Colitis limited to the ascending and/or proximal transverse colon is best treated by a right or extended right colectomy. Colitis of the left and distal transverse colon potentially could be treated by a segmental resection with a colocolic anastomosis, or by a total or subtotal colectomy with an ileorectal or ileosigmoid anastomosis.
Similar to small bowel resections, surgeons speculated that limited or segmental colonic resections when clinically appropriate could be advantageous for patients with limited Crohn's colitis. More than two decades ago, Sanfey et al13 reported their experience with 41 patients with various degrees of Crohn's colitis. Twelve patients had a total proctocolectomy with a permanent ileostomy, and 29 patients had segmental resections. Of these 29 patients, 13 had a subtotal colectomy with 7 having an ileorectal anastomosis and 6 had an ileostomy. Only 3 of these 13 patients (23%) had the continuity of the bowel eventually restored. The remaining 16 patients had limited colectomies; in 12 patients (75%) the continuity was restored in the long term. Despite the fact that these patients required more subsequent operations due to recurrent disease, the authors concluded that limited colitis should be treated by a limited resection, and that diffuse or distal colitis should be treated with total colectomy or proctocolectomy, and that these patients had a low chance of having their stomas reversed. Longo et al14 noted that after 5.5 years of follow-up, 62% of patients who had segmental colectomy and 67% of patients who had total colectomy had recurrent disease compared with 20% recurrent small bowel disease in patients who had total proctocolectomy. Intestinal continuity was maintained in 81% of the patients treated by segmental resection. The authors concluded that although recurrence was likely, segmental colectomy improved the quality of life by delaying the need for a stoma and by preserving functioning bowel. Prabhakar et al15 from the Mayo Clinic described 49 patients who underwent a segmental or a total colectomy and were followed for a mean of 14 years. In 22 patients (45%) no further treatment was required; of the remaining 27 patients, 16 required additional surgical procedures due to recurrent disease that occurred after a mean of 51 months, and 5 had a third procedure. After 14 years of follow up, 42 patients (86%) remained stoma-free, and 7 (14%) ultimately required permanent ileostomy, with a mean stoma-free interval of 23 months.
A recent meta-analysis16 compared the results of segmental and total or subtotal colectomy for Crohn's colitis. No prospective randomized trials compared these procedures, so the authors reviewed six comparative trials, which included 488 patients of whom 223 underwent a total colectomy with an ileorectal anastomosis and 265 had a segmental colectomy. There were no differences in rates of postoperative recurrence, postoperative complications, or need for a permanent stoma between the two groups, however, the time to recurrence of Crohn's disease was significantly shorter in the patients who underwent a segmental resection by a mean of 4.4 years (p<0.001). The authors concluded that both procedures were equally effective as treatment options for colonic Crohn's disease, with recurrence occurring significantly earlier in patients who undergo limited resections. The choice of operation should be dependent on the extent of colonic disease; however, because no prospective randomized studies have been undertaken, a clear view about which approach was more suitable for localized colonic Crohn's disease could not be determined.
With the introduction and widespread use of more aggressive and effective agents for postoperative maintenance and prophylaxis, recurrence may be reduced and delayed even more than previously reported. Surgeons should keep in mind that depending on the extent of the colonic inflammation, a limited resection should be considered and supplemented with appropriate maintenance therapy. This may significantly delay recurrence, potentially reduce the need for subsequent surgical interventions, and potentially reduce the risk of a permanent stoma, which is one of the major factors that influence the quality of life, especially in this young patient population.
Restorative proctocolectomy (RPC) and ileal pouch-anal anastomosis (IPAA) is the procedure of choice for patients with ulcerative colitis (UC) and familial adenomatous polyposis (FAP). However, most surgeons consider Crohn's colitis to be an absolute contraindication for performing a RPC and IPAA due to high complication and failure rates, which may lead to excision of the pouch and result in a permanent ileostomy and short bowel syndrome. Currently, few centers offer a RPC and IPAA to patients with an established preoperative diagnosis of Crohn's disease.17,18 In one recent report,18 the authors selected 41 patients with isolated Crohn's proctocolitis without anal manifestations to undergo a RPC with IPAA. After 10 years of follow-up, 14 patients (35%) had Crohn's-related complications and 4 patients (10%) had their pouch excised. The authors suggested that due to the relatively low complication rates, this procedure could be offered to selected patients with Crohn's disease; it may delay the need for a permanent ileostomy. Despite these rather promising results, most studies reported significantly worse outcomes. Brown et al19 described 36 patients who were followed up for a mean of 83 months. The pouch failure rate in this report was 56% (20 patients), with 16 patients (45%) having their pouch excised; 64% of patients had pouch-related complications and 4 additional patients (11%) required permanent diversion. Tekkis et al20 reported on 26 patients who had RPC and IPAA for Crohn's disease. After 70 months of follow-up, 14 patients (54%) underwent a pouch excision, and another patient was permanently diverted for a total pouch failure rate of 58% (15 patients). These patients also were 2.6 times more likely to develop pouch-related fistulae compared with patients who had a diagnosis of UC. The authors of these reports concluded that Crohn's disease should remain a contraindication to RPC and IPAA. The outcomes reported in these and other studies are summarized in Table Table11.19,20,21,22,23,24,25,26,27,28,29,30 The reported overall pouch failure rates range from 12 to 66% with pouch excision rates ranging from 10 to 54%. In a recent meta-analysis, Reese et al31 compared the outcomes of RPC and IPAA in patients with UC, Crohn's disease, and indeterminate colitis. Ten studies comprising 3,103 patients (Crohn's disease, n=225; ulcerative colitis, n=2,711; indeterminate colitis, n=167) were included. Patients with Crohn's disease had significantly more anastomotic strictures (p=0.05), higher pouch failure rates (p<0.001), higher rates of pouch inflammation after closure of protective ileostomy (p=0.05), higher rates of urgency (p=0.02), and higher rates of incontinence (p=0.01) compared with patients with non-Crohn's diagnosis. The pooled pouch failure rate in this study was 35% in patients with Crohn's disease compared with 4.8% in patients with UC and indeterminate colitis combined (p<0.001); the overall postoperative complication rates were significantly higher and functional outcomes were worse in patients with Crohn's disease. The authors recommended that patients with an established preoperative diagnosis of Crohn's disease who are planning to undergo a RPC and IPAA should be fully aware of these results and their consequences. Nevertheless, they also suggested that a carefully selected, special subset of patients, who have isolated Crohn's colitis without any evidence of small intestinal or perianal manifestations, may benefit from RPC and IPAA with acceptable complications and function. Similarly, patients who undergo a RPC and IPAA for presumed UC or indeterminate colitis, whose diagnosis is subsequently changed to Crohn's disease, but do not have perianal or terminal ileal manifestations, also may have acceptable outcomes and function. Hartley et al30 described a series of 60 such patients in whom the pouch failure rate was only 12%; in the 53 patients with functioning pouches, the median number of daily bowel movements was 7 (3 to 20) with 50% of patients rarely or never experiencing urgency and 59% (35 patients) reporting perfect or near-perfect continence. The authors suggested that such results could potentially be improved even further with the continued development of medical strategies for the long-term maintenance and suppression of Crohn's disease.
Currently, RPC and IPAA is not considered as the standard of care for patients with an established diagnosis of Crohn's disease; however, it may be followed by a successful outcome.
Since the first laparoscopic colectomy nearly 17 years ago, almost every abdominal procedure has been laparoscopically attempted; the scope of indications is constantly increasing. Currently, a mounting body of evidence demonstrates the advantages of laparoscopy over traditional laparotomy: Smaller incisions cause less postoperative pain, earlier recovery of bowel function and toleration of diet, shorter hospitalization, and reduced costs.32,33,34 Early reports of the application of laparoscopy in patients with inflammatory bowel disease (IBD) discussed mainly the feasibility of creating stomas and performing limited resections laparoscopically,35 but the indications were gradually broadened to include ileocolic resections, other segmental resections, and subtotal and total colectomies in patients with Crohn's disease, and even laparoscopic RPC and IPAA in selected patients with UC, and emergency operations.
Indications for laparoscopic surgery in patients with Crohn's disease are the same as for conventional surgery as mentioned in the beginning of this review. Potential contraindications for the laparoscopic approach include diffuse peritonitis, multiple previous laparotomies with known diffuse intraabdominal adhesions, acute intestinal obstruction with massive dilation of bowel loops, and uncorrectable bleeding disorders. The laparoscopic approach can and should be utilized in the same manner as conventional open surgery: (1) for establishing diagnosis—diagnostic laparoscopy, (2) for fecal diversion—laparoscopic creation of a colostomy or ileostomy, and (3) for performing bowel resections. However, the characteristic features of Crohn's disease—skip areas; thick and short mesentery; large, friable inflammatory masses; enteric fistulae and adhesions from previous operations—make the laparoscopic approach in these cases significantly more challenging, even for the most experienced surgeons. Nevertheless, because most patients with Crohn's disease are relatively young, and most will require surgery at some point,3 the laparoscopic approach is very appealing for patients and surgeons alike, despite these difficulties.
The most extensive data about laparoscopic restorative procedures in patients with Crohn's disease are about ileocolic resection. Since the first report of laparoscopic ileocolic resection,36 numerous studies compared its outcomes to the equivalent open procedure; interestingly, only two of these reports were prospective, randomized trials.37,38 Milsom et al37 prospectively randomized 60 patients to undergo either a laparoscopic (31 patients) or a conventional (29 patients) ileocolic resection. In 2 patients (6.5%) the laparoscopic procedure was converted; there were significantly less minor complications in the laparoscopic group (4 versus 8 in the open group; p<0.05); the operative time was significantly longer in the laparoscopic group (145 minutes versus 85 minutes in the open group; p<0.0001); recovery of pulmonary function as measured by forced expiratory volume in the first second (FEV1) and forced vital capacity (FVC) was significantly faster in the laparoscopic group (2.5 days versus 3.5 days in the open group; p=0.03). There were no significant differences between the two groups in terms of postoperative opiate requirements, recovery of normal bowel function, and length of hospital stay. The authors concluded that the laparoscopic approach offered some benefits without any apparent disadvantages other than the longer operative time. In a more recent study, Maartense et al38 also randomized 60 patients for laparoscopically assisted (30 patients) or conventional (30 patients) ileocolic resection. Primary outcome parameter was postoperative quality of life during 3 months of follow-up, and secondary parameters were operating time, morbidity, hospital stay, postoperative morphine requirement, pain, and costs. Conversion to an open procedure was required in 3 patients (10%). There was no statistically significant difference in quality of life between the groups during the follow-up period. Median operating time was longer in laparoscopic compared with open surgery (115 versus 90 minutes; p<0.003). Hospital stay was shorter in the laparoscopic group (5 versus 7 days; p=0.008). The number of patients with postoperative morbidity within the first 30 days was lower in the laparoscopic group (10% versus 33%; p=0.028). Median overall costs during the 3 months of follow-up were significantly lower in the laparoscopic group (p=0.042). The authors concluded that although there were no differences in the quality of life between the two groups, laparoscopy did offer obvious advantages over open surgery in terms of lower morbidity, shorter length of stay, and reduced costs. Long-term quality of life in patients with Crohn's disease has been shown to be reduced compared with the general population; recurrent disease was the only factor adversely affecting it, irrespective of the operative technique applied.39 Tilney et al40 recently conducted a meta-analysis of the results of 15 studies (one prospective randomized trial, six prospective observational trials, and eight retrospective trials) that compared the outcomes of laparoscopic with open ileocolic resection in Crohn's disease. The studies included 783 patients, 338 (43.2%) of whom had undergone laparoscopic resection, with an overall conversion rate of 6.8% (23 patients). The operative time was significantly longer in the laparoscopic group, by nearly 30 minutes (p=0.002); the blood loss and complication rates in the two groups were similar. The patients who had laparoscopic surgery had a significantly shorter time for recovery of their bowel function and a shorter hospital stay, by 2.7 days (p<0.001). For selected patients with uncomplicated ileocecal Crohn's disease, laparoscopic resection offered substantial advantages in terms of faster resolution of postoperative ileus and shortened hospital stay, with equivalent rates of morbidity compared with open procedures (Table 2).32,33,37,41,42,43,44,45,46,47,48,49,50,51,52 Lowney et al53 studied the effect of laparoscopy on the long-term anastomotic recurrence after ileocolic resection in a retrospective study that included 113 patients. Recurrence was defined as development of new anastomotic Crohn's disease requiring surgical intervention. There was no statistically significant difference in the recurrence rates between the two groups; hence, the anastomotic recurrence is probably a feature of Crohn's disease, which is not dependent on the surgical technique that was used for the bowel resection.
The ultimate goals of both the medical and surgical treatment Crohn's disease are optimal control of symptoms and maintenance of quality of life. Because the presence of a stoma is detrimental to quality of life, avoidance of creating stomas by performing restorative procedures is a key feature in maintaining a better quality of life. However, patients and physicians should keep in mind that it is natural for Crohn's disease to relapse, especially at or near previous anastomoses, and that recurrence has a negative impact on quality of life. Patients should be fully aware that at some point, creation of a stoma may be a better option for controlling symptoms and improving lifestyle.