Controversies in the Management of Abdominal Crohn's Disease
Robert R. Cima, MD, FACS, FASCRS*
Assistant Professor of Surgery
Consultant, Division of Colon and Rectal Surgery
Mayo Clinic College of Medicine
Mayo Clinic, Rochester, MN
Crohn’s disease (CD) is a chronic inflammatory disease of the intestinal tract that that often results in the need for surgical intervention in order to treat complications of the disease. The transmural nature of the inflammation leads can lead to intestinal perforation, intraabdominal abscesses, intestinal strictures and fistula development. Since there is no cure for CD, many patients will require multiple operations during their lifetime. Index surgery and re-operative surgery in these patients is often complex and challenging. There are many preoperative planning and technical aspects of Crohn’s surgery that can be helpful in achieving a successful clinical outcome. This syllabus will review some areas of current controversy in the management of abdominal Crohn’s disease.
Introduction
Crohn’s disease (CD) and ulcerative colitis (UC) are the major categories of non-specific inflammatory bowel diseases. The etiology of both diseases is unknown. However, unlike UC which is limited to the rectum and colon, CD can involve any point along the entire intestinal tract. While CD affects the small bowel predominantly, it frequently involves the colon, rectum, and perianal region. Sometimes these areas may be involved simultaneously or at separate times during the course of the patient’s disease. Unlike UC in which the intestinal manifestations of the disease are cured with removal of the rectum and colon, surgery for CD is directed at relieving symptoms related to or treatment for complications of the disease. Surgery should not be considered curative but rather as an adjunct to maximal medical therapy. The decision to proceed with operation should only be entertained after careful consideration and consultation with the patient and a gastroenterologist experienced in the medical management of the patient’s disease.
Nearly all of the CD complications that require surgical intervention are related to the transmural inflammation of the bowel wall that characterizes the disease. When this inflammatory process is allowed to progress it may lead to abscess formation, entero-entero or entero-cutaneous fistula, acute bowel perforation, and intestinal strictures. As noted previously, CD is not cured by surgery. Therefore, patients may require multiple surgical procedures over their lifetime due to recurrence of the disease. In the pre-immunomodulator period, nearly 50% of CD patients who underwent surgery would require surgery again to treat their disease within 5 years. The influence of newer therapeutic agents, such as azothioprine and Infliximab, on the need for future surgery is currently unknown. Since there is a relatively high reoperation rate in CD patients at present, another primary concern of the surgeon should be to minimize the amount of intestine that is resected especially small bowel. The purpose of this syllabus is not to extensively discuss the management of abdominal CD but rather to highlight some specific issues that remain areas for discussion or controversy.
Limited Colonic Resections for Crohn’s colitis
Traditionally, management of the Crohn’s limited to the colon had been removal of the entire colon with either construction of an ileorectosomy if the rectum and perianal region where not involved with disease or a total proctocolectomy with a permanent end ileostomy. The rationale for removal of the entire organ rather than segmental resection of involved region of the colon was that preserving the remaining colon did add any significant physiologic benefit to the patient by retaining the colon. Furthermore, the risk for recurrence was thought to be high, although there was no data to support this assumption, therefore the patient would need a subsequent operation to address the colonic recurrence. Over the last decade, a number of authors have reported that a “colon preserving” approach is feasible, safe and postpones the need for a permanent ileostomy. Prabhakar, et al. reviewed their experience with 699 patients with Crohn’s colitis over a 10 year period. Fifty-three patients underwent a segmental colon resection and 49 were available for analysis. With a mean follow-up of 14 years, 45% of patients required no further surgical therapy for their CD. Of the 27 patients who needed some type of therapy, 11 patients required medical therapy and 16 patients were surgically managed. Six of those patients had a completion proctectomy with permanent ileostomy while the remaining 10 had a repeat limited colonic resection. Overall, 86% of patients of patients suitable for a segmental colon resection for CD were stoma free at the end of the study. In a similar study published by Martel and colleagues, 84 patients underwent segmental colectomy for colonic CD. With a mean follow-up of 9 years, only 36 patients required reoperation. Twenty-six patients had colonic recurrences which were treated with repeat segmental resections in 17 patients and total colectomy in 9 patients. Using this “colon-preserving” approach 75% of patients with limited colonic CD were stoma free at the end of the follow-up period. Furthermore, these authors looked at quality of life and found that the majority of patients were having less than 3 bowel motions a day and over 80% were very satisfied with their functional result. Neither studied demonstrated any increased risk of post-surgical complications after segmental colon resection.
It is very clear from these fairly large retrospective series and other smaller ones that a conservative surgical approach in the management of colonic CD avoids the need for a permanent stoma in the vast majority of patients for at least a decade. The impact of modern immunomodulator or biologic medical therapy on extending the period of time without the need for subsequent surgical intervention or stoma rates is unknown. However, one would hope it will further extend the period of time that a patient may avoid repeat operation or a stoma.
Prabhakar LP, et al. Avoiding a stoma: role for segmental or abdominal colectomy in Crohn’s colitis. Dis Colon Rectum. 1997;40:71-78.
Martel P, et al. Crohn’s colitis: experience with segmental resections; results in a series of 84 patients. J Am Coll Surg 2002;194:448-453.
Fate of the Rectum after Ileorectal Anastomosis
Most patients wish to avoid the need for a permanent stoma. As noted previously, a colon-preserving approach to limited colonic CD did result in a prolonged period without recurrence or the need for a permanent stoma. For patients with extensive colonic disease but without any rectal involvement or perianal disease, the surgical choices are limited to total abdominal colectomy with either end ileostomy or an ileorectal anastomosis (IRA) or a total proctocolectomy and permanent end ileostomy. Previously, many surgeons avoided rectal preservation fearing a high recurrence rate of the CD and concern for a poor functional outcome. However, a number of studies have demonstrated that the recurrence rate and functional outcome of an IRA for the treatment of extensive colonic CD are very reasonable.
Cattan and colleagues reviewed their experience with 144 patients with extensive colonic CD. An IRA was performed in 118 patients while 26 had an ileostomy and rectal preservation rather than an IRA because of severe anorectal CD at the time of operation. After ten years of follow-up, the probability of clinical recurrence after an IRA was 83% with a 10 year rectal preservation rate of 86%. Of those patients who had an ileostomy with rectal preservation, 63% still had their rectum in place at 10 years. One of the main predictors of failure of both the IRA and rectal preservation was the presence of extra-intestinal manifestations of CD at the time of the original surgery. However, presence of active perianal CD at the time of IRA was not associated with a worse outcome. Similar findings were reported by Pastore and colleagues in their review of 42 CD patients that underwent an IRA and Yamamoto et al who reviewed 130 patients that underwent IRA or colectomy with ileostomy and preservation of the rectum.
Again these studies support a very conservative bowel sparing approach to management of the colonic CD.
Cattan P, et al. Fate of the rectum in patients undergoing total colectomy for Crohn’s disease. Br J Surg. 2002;89:454-459.
Yamamoto T, et al. Fate of the rectum and ileal recurrence rates after total colectomy for Crohn’s disease. World J Surg. 2000;24:125-129.
Pastore RL, et al. Total abdominal colectomy and ileorectal anastomosis for inflammatory bowel disease. Dis Colon Rectum. 1997;40:1455-1464.
Ileal pouch anal anastomosis (IPAA) in Crohn’s Disease
The IPAA is the preferred restorative surgical option for appropriately selected patients with chronic ulcerative colitis (CUC). Across a large number of institutions with over 20 years of experience, the IPAA in CUC patients has been shown to be a safe and durable procedure with high patient satisfaction that avoids the need for a permanent stoma. Traditionally, the diagnosis of CD was an absolute contraindication for an IPAA even if the patient had a clinical history of disease limited to the colon. The rationale for this view was that those patients who were thought to have CUC and underwent an IPAA that were later found to have CD had a much higher rate of pouch related complications and failure rates.
In recent review by Braverman and colleagues, who evaluated 32 patients with an IPAA who later had their diagnosis changed to CD. In this small series, 28% of patients had to have their pouch either removed or diverted with a proximal ileostomy with a median follow-up of 12 years. Those patients with a pouch in place did have a much higher rate of peri-pouch complications including perianal abscesses and fistulas as compared to the expected result for patients who had IPAA for CUC. In regards to functional outcomes, the CD pouch patients had on average 6 bowel movements per 24 hours with a majority of patients reported pouch leakage and the need for pad use. However, the majority of patients in the series still had their pouch in place avoiding the need for a permanent stoma with good function although many required medication to manage their CD. A similar review of patients was performed by Hartley et al. In their group of 60 patients diagnosed with CD after IPAA, at a median follow-up of nearly 4 years 21 patients (35%) had developed recrudescent CD. In this sub-set, of patients eventually 7 patients required pouch excision or permanent defunctioning of the pouch. For the entire cohort, the overall pouch failure rate was only 12% but with a 33% failure rate in those patients that had recrudescent CD in the pouch. These results are comparable to a study by Colombel and colleagues who looked at patients with CD and an IPAA that were medically managed with infliximab. In this small study, 8 out of 26 patients ultimately required pouch excision or defunctioning while 67% had a well functioning IPAA.
Panis et al reported the only large series of patients that underwent IPAA with a known diagnosis of CD. This highly selected patient population none had a prior history of anorectal or small bowel CD. With up to 5 years of follow-up, they reported that there was no increase in immediate postoperative complications as compared a contemporary data set of CUC patients undergoing IPAA. The overall pouch function as measured by stool frequency, continence, leakage, and sexual activity were all similar. There were 21 CD patients in the study with at least 5 years of follow-up and in this group only 2 (10%) required pouch excision.
Although all of these studies are small single institution, non-randomized retrospective reports, it would seem that in highly selected and motivated patients an IPAA may be offered for the management of Crohn’s colitis. The overall functional results seem relatively similar to that of patients with CUC but there is an increase in peri-pouch complications. Furthermore, while the rate of pouch failure is higher than in CUC patients the vast majority of patients have a functioning pouch for a number of years. Also, the impact of newer medical therapies has not been fully analyzed and the report from Colombel would suggest that these therapies may make be able to successfully manage reactivation of the CD.
Braverman JM, et al. the fate of the ileal pouch in patients developing Crohn’s disease. Dis Colon Rectum. 2004;47:1613-1619.
Hartley JE, et al. Analysis of the outcomes of ileal pouch-anal anastomosis in patients with Crohn’s disease. Dis Colon Rectum. 2004;47:1808-1815.
Colombel JF, et al. Management of Crohn’s disease of the ileoanal pouch with infliximab. Am J Gastroenterol. 2003;98:2239-2244.
Panis Y, et al. Ileal pouch/anal anastomosis for Crohn’s disease. Lancet. 1996;347:854-857.
Duodenal Crohn’s Disease
One of the hallmarks of CD is that may involve any segment of the intestinal tract. Fortunately, the duodenum is rarely involved with a reported frequency of 0.5-4.0%. However, when it is involved it poses a significant management challenge. If surgical intervention is required, the historical procedure of choice was some type of gastric-biliary by-pass procedure. Murray et al reported excellent long-term outcomes with a by-pass although a third of patients required some type of reoperative procedure. Recently, there have been a number of case reports or small series that have evaluated the role of strictureplasty in the management of duodenal strictures. Takesue and colleagues reported performing Finney type strictureplasties for proximal duodenal strictures in two patients without complications. In a larger case series of 10 patients, Yamamoto reviewed the results of surgery for 13 patients that underwent duodenal strictureplasties. In this series with long-term follow-up strictureplasty was associated with a high rate of reoperation for early post-operative complications or recurrent duodenal strictures. Overall, nine out of the 13 patients required reoperation either to have repeat strictureplasty or conversion to a by-pass procedure. Other surgical options to treat duodenal strictures have been described including a pedicle jejunal patch to the duodenum. In this case described by Eisenberger and colleagues, an 8 cm duodenal stricture was opened and the defect was closed with a pedicle flap of 10 cm of jejunum. Intestinal continuity was re-established by performing a jejuno-jejunoenterostomy.
Clearly, duodenal CD presents a complex management problem. By-pass procedures while larger operations provide a durable long-term option. For short strictures strictureplasty may be considered but is associated with a relatively high recurrence rate. Novel reconstructive procedures may be considered but do represent a high risk endeavor.
Murray JJ, et al. Surgical management of Crohn’s disease involving the duodenum. Am J Surg. 1984;147:58-65.
Takesue Y, et al. Strictureplasty for short duodenal stenosis in Crohn’s disease. J Gastroenterol. 2000;35;929-932.
Yamamoto T, et al. Outcome of strictureplasty for duodenal Crohn’s diease. Br J Surg. 1999;86:259-262.
Eisenberger CF, et al. Strictureplasty with a pedunculated jejunal patch in Crohn’s disease of the duodenum. Am J Gastroenterology. 1998;93:267-269.
Stricturing disease and the long strictureplasty
One of the most common causes of reoperation in the CD patient is for obstructive symptoms related to stricture formation. Symptomatic strictures often involve the small bowel. Strictureplasty is a useful surgical technique that relieves the obstructive symptoms caused by small bowel strictures while preserving intestinal length and thus hopefully avoiding the complications of repeated small bowel resection. Lee and Papaioannou first reported strictureplasties for the treatment of Crohn’s strictures in 1982. While any Crohn’s patient undergoing surgery for obstructive disease symptoms might be a candidate for a strictureplasty, those patients who will most benefit are those who develop recurrent strictures.
The nature of the stricture and the amount of the remaining bowel are key factors in determining whether a strictureplasty is appropriate. Strictureplasty is not an option in the setting of bowel perforation or extensive inflammatory phlegmon in the involved segment of bowel. On initial exploration of the abdomen, all evidence of disease should be carefully and obvious strictures marked. Each area of involved bowel should be examined for any other pathology that might preclude a strictureplasty such as a fistula or localized abscess. The length of bowel remaining in situ should to be assessed for the presence of other strictures. However, visual and tactile external evaluation of the bowel may not identify all small bowel strictures. A number of different techniques have been described to intraoperatively evaluate the internal diameter of the small bowel lumen. In essence, these techniques all rely upon the introduction of a device, most commonly a balloon tipped catheter, into the bowel that can be passed along its length in order to assess if there are any obstructions to easy passage.
The technique used for a strictureplasty is dependent upon the length of the stricture. For strictures less then 4-5 cm in length, the procedure is similar to the Heineke-Mikulicz pyloroplasty performed for pyloric stenosis. For longer strictures, >4-5 cm, the standard transverse closure of the enterotomy will not be possible. The Finney strictureplasty resembles a side-to-side anastomosis. This strictureplasty may be useful in a patient with a long stricture or for a segment with multiple short strictures closely group together with intervening dilated short segments of bowel. The bowel is folded at the stricture with the normal proximal and distal bowel brought along side one another. If a hand-sewn technique is used there are two options. First, if the strictured area is mildly stenotic and the bowel is of reasonable quality then the entire stricture may be opened along the antimesenteric border and a hand-sewn essentially side-to-side anastomosis may be performed along the length of the enterotomy. Second, if the stricture is too tight or the bowel is not suitable for suturing, then a true side-to-side anastomosis between the proximal and distal normal bowel can be performed leaving the strictured segment in place as a short by-passed segment.
When the bowel is markedly dilated proximal to a short stricture the size discrepancy between the proximal and distal normal bowel often precludes a Heineke-Mikulicz strictureplasty. In these instances, a Moskel-Walske-Neumayer strictureplasty is performed. This strictureplasty is essentially a Y-to-V advancement flap closure of the stricture. The stricture is opened along the antimesenteric border as a “Y”-shaped enterotomy with the “Y” portion in the dilated bowel just proximal to the stricture. The strictured segment is then pulled apart and the antimesenteric segment of the proximal bowel is advanced into the strictured area and closed in a transverse fashion with one side of the closure being normal bowel along the entire length the and the other being the two strictured bowel edges.
Michelassi has developed a unique procedure for dealing with the most difficult type of stricture, the very long (>20 cm) or a series of strictures in close proximity. In these highly unusual patients, a very long segment of bowel that would result in a prohibitively extensive resection can be retained as a side-to-side isoperistaltic strictureplasty. In this technique, the bowel is completely divided transversely at the middle of the strictured segment. Unlike other strictureplasties, the mesentery is divided perpendicular to the long axis of the bowel to permit the two segments of bowel to be overlapped and positioned side-to-side along the entire length of the divided segments. Both strictured segments are opened along the anti-meseneteric border and the antimesenteric faces of bowel are sewn one to the other in an isoperistaltic fashion. This technique has the advantage of not having any bypassed bowel. Because a small numbers of patients require this type of strictureplasty reports of long-term functional results are limited. However, Shatari and colleagues have reported on 21 patients that underwent long (>20 cm) small bowel strictureplasty compared to 41 patients that had short strictureplasties performed. They found no significant difference in postoperative complications between two groups. Furthermore, there was no difference in 3-, 5-, and 10-year recurrence rates between the long and short stiructureplasty groups.
Nearly all series of strictureplasties are single institution reports. Dietz et al. have reported the safety and long-term efficacy of strictureplasty in 314 patients with obstructing small bowel CD. They reviewed 1,124 strictureplasties performed in 314 patients. The overall morbidity was 18% and septic complications occurred in 5%. Crohns disease recurred in 34% of patients with a median follow-up of 7.5 years. The only significant predictor of recurrence was earlier age at the time of the index strictureplasty.
The primary role of strictureplasty is to preserve small bowel length in patients who have severe diffuse medically refractory disease or in patients who have recurrent stricture disease. Both short and long length strictureplasty are effective operations. When strictureplasty is used appropriately, it is an extremely important and useful operation for patients with complex Crohn’s disease.
Lee EC, Papaionnou N. Minimal surgery for chronic obstruction inpatients with extensive or universal Crohn’s disease. Ann R Coll Surg Engl. 1982;64:229-233.
Michelassi F. Side-to-side isoperistaltic strictureplasty for multiple Crohn’s strictures. Dis Colon Rectum. 1996;39:344-349.
Shatari T. et al. Long strictureplasty is a safe and effective as short strictureplasty in small bowel Crohn’s disease. Colorectal Disease. 2004;6:438-441.
Dietz DW, et al. Safety and long-term efficacy of strictureplasty in 314 patients with obstructing small bowel Crohn’s disease. J Am Coll Surg. 2001;192:330-338.
Summary
Abdominal Crohn’s is a complex disease process. The primary goal of surgery is to minimize to loss of small bowel length to avoid nutritional compromise. A secondary goal should be to avoid a permanent stoma. The impact of newer medical therapies for CD may permit a broader scope of operations than previously recommended for Crohn’s patients.