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Tracy L. Hull, M.D.
Staff Surgeon, Department of Colorectal Surgery
The Cleveland Clinic Foundation
Cleveland, Ohio

Crohn's disease is a chronic inflammatory condition that can affect any part of the gastrointestinal tract from the mouth to the anus. The etiology is unknown. Symptoms depend on what portion of the gastrointestinal tract is affected and may include pain, bleeding, obstruction, abscess and infection, diarrhea, weight loss, and others. Neither medical nor surgical treatment is curative. Except in emergent situations, medical treatment is initiated until it fails to alleviate symptoms, produces unacceptable side affects, or a complication of the disease occurs.

Operative Indications

Most patients with Crohn's disease ultimately require one or more operations in their lifetime. Operative indications are the same no matter where the disease manifests itself. They include:

1. Failure of medical therapy

2. Obstruction

3. Fistula or abscess

4. Hemorrhage

5. Growth Retardation (in the pediatric population)

6. Perforation

7. Carcinoma

8. Extraintestinal manifestations

Preparation of the Patient

Patient preparation is similar no matter the site of disease. Unless contraindicated due to other problems, a thorough endoscopic and roentgenographic evaluation is performed of the large and small bowel to define all disease and plan the operative strategy. Dehydration, electrolyte deficiencies, coagulation defects, and anemia are repleted. Comorbid medical diseases are optimized and may require input from medical colleagues.

It is common for patients with Crohn's disease to present with varying degrees of malnutrition. Nutritional optimization with the use of total parenteral nutrition is controversial. Most reports in the literature are retrospective and uncontrolled and results on either side of the issue can be found (Higgins 1981, Irving 1990, Lashner 1989. In cases where surgery is elective or semi-elective and malnutrition is considered severe, total parenteral nutrition is used preoperatively for 5 to 7 days (Fazio 1976). If significant hypoalbuminemia persists, serious consideration is given to avoiding a bowel anastomosis in favor of a temporary ileostomy after resectional surgery.

If there is any indication that a stoma may be needed, preoperative marking is essential. The major factor influencing satisfactory rehabilitation of an ostomy patient is the correct location and construction of the stoma. The principles of stoma siting include the following:

1. The site is clearly visible to the patient

2. The surrounding zone of skin is undisturbed for a distance of 5 cm

3. The site is located at the summit of the infraumbilical fat mound

4. The surface marking is within the rectus abdominis muscle

5. The site is away from bony prominences, skin creases, or scars

Patients will often benefit from discussing ostomies with an enterostomal therapy nurse or lay ostomate.

Unless an emergency exists, withdrawal of immunosuppressives such as azathioprine, 6-mercaptopurine, or cyclosporine for 3-4 weeks prior to surgery should be considered. There is concern that these agents may negatively influence tissue and anastomotic healing.

Patients who are not obstructed should receive a bowel preparation with agents such as polyethylene glycol (4L) or Fleet phosphosoda (90 ml). Partially obstructed patients may require a modification with clear liquids or an elemental diet for 3-5 days. In this situation magnesium citrate over several days may be tolerated and help cleanse the bowel. Patients with distended small bowel may require preoperative hospitalization for nasogastric decompression as well as hydration and correction of electrolyte imbalances. Additionally if distal colon or anorectal surgery is to be done intraoperative washout of the rectum through a large rectal (34 Fr) catheter is done until the effluent is clear.

Additional preparation includes intravenous antibiotics preoperatively with postoperative antibiotics tailored to the operative findings and cultures. Stress steroids are administered perioperatively if the patient has been treated with steroids within the 6 months prior to operation. After anesthesia a nasogatric tube and foley catheter are placed. In patients with poor peripheral access a central catheter may be beneficial especially during the postoperative phase. Ureteric stents are considered when reoperative pelvic surgery is done or when an abscess or large phlegmon is suspected along the right or left gutter which may make identification of the ureter very difficult. Finally, prophylaxis against deep venous thrombosis is employed using pneumatic stockings with or without mini-dose heparin.

Strategic Planning for Surgery

It is important to remember that surgery does not "cure Crohn's disease" and the long term outlook for the patient should always be kept in mind. Ninety percent of individuals with Crohn's disease will ultimately need operative treatment (Whelan 1985). Forty to seventy percent of patients will require at least an additional operation by 15 years (Farmer 1975,Sales 1983). Therefore, with few exceptions a midline incision should be done to avoid destroying potential stoma sites that may be needed during the patient's lifetime.

The small intestine is a precious commodity and conservation is imperative to prevent short bowel syndrome in the short or long term. Nonobstructing, nonhemorrhaging segments of Crohn's disease usually do not warrant resection as medical therapy can usually provide a reasonable quality of life. One modification of this principle involves strictures. If a stricture is identified at operation, even if it is not felt to be symptomatic at that time it should be addressed with strictureplasty or resection, as they may become symptomatic after other obstructing lesions upstream are relieved.

Imperative to the concept of small bowel preservation is the issue of resection margins. Controversy has existed as to how much normal bowel should be resected proximal and distal to the diseased segment. Recent evidence shows that extended resection margins are unnecessary and show no advantage to patients in reducing cumulative recurrence rates. Also frozen sections of the margins are unnecessary. The presence of residual microscopic Crohn's disease at the resection margin does not appear to increase the recurrence rates (Fazio 1996). Additionally, minute apthous ulcers may be seen within the intestine at sites remote from the resected specimen. These ulcers can be ignored as there exists no evidence that they increase the incidence of recurrence. While it is highly desirable to perform anastomosis with normal appearing bowel ends, compromise may be required in patients with foreshortened intestine (i.e. less than 200 cm). In this situation it is acceptable to join segments that are involved or even inflamed with Crohn's disease as long as there is no stricturing or deep ulceration present.

Situations occur when resecting bowel for Crohn's disease which make anastomotic healing compromised. Examples include incompletely drained sepsis when a phlegmonous rind of inflammation representing a residual pyogenic membrane in proximity to an anastomosis exists, excessive blood loss during a long operation (i.e. >3-4 hours), severe hypoalbuminemia (i.e. <2.5 g/dL), or malnutrition. In these situations thought should be given for using a temporary stoma. Additionally the use of steroids does not mandate the use of a temporary stoma.

At the completion of the operation the peritoneal cavity is thoroughly irrigated with warm saline. Drains are used if a septic pyogenic membrane is left, when an infected retroperitoneal space has been found (i.e. psoas abscess), or if presacral mobilization of the rectum has been done. Usually the drains are brought out away from the main incision as close as possible to the area being drained. Drains placed over residual septic foci are removed after about two weeks. If a residual cavity is possible (such as with a psoas abscess), (14 to 16 Fr) mushroom or de Pezzer catheters are placed for an additional 3-6 weeks to prevent premature healing of the external skin. Prior to drain removal, sinograms may be indicated to assure shrinking of the abscess cavity. For presacral mobilization of the rectum, sump drains are used to minimize blood and fluid collection in the pelvis. These collections can act as excellent culture media for abscess-forming bacteria. They are removed when the drainage decreases to <50 cc in 24 hours.

Surgical Procedures

As would be expected the surgical procedure depends on the site of disease.

Ileocolic
Ileocolic disease is the most common disease pattern in Crohn's disease affecting 40-50% (Farmer 1975). Resection of the diseased bowel is the preferred method of surgical treatment. An anastomosis is performed if there is no confounding problem such as frank perforation and diffuse peritonitis. Sometimes an anastomosis with proximal loop ileostomy can be done. Even though this still requires another operation in the future, closure of a loop ileostomy is much less complicated than closure of an end ileostomy and usually does not require a full open laparotomy.

When an anastomosis is done, consideration is given if the distal end is at the ascending colon. In that situation the anastomosis sits over the duodenum and a recurrence of disease risks adherence to the duodenum with the risk of fistula or injury during future operative mobilization of the ileocolic anastomosis. To avoid these problems, wrapping the anastomosis with omentum can be done.

Bypass of ileocolic disease is done less in most recent times. A reasonable indication for this operation would be a complicated ileocecal phlegmon densely attached to the iliac vessels or retroperitoneum. However there should be plans to do a definitive resection months later after the inflammation has subsided. With this type of bypass, the proximal end of the ileal segment should be exteriorized as a small mucus fistula to vent the mucosal secretions that could cause ileal stump blow out.

Ileostomy alone is rarely used for ileocolic disease. It could be used in the example given in the previous paragraph. In cases of free perforation, ileostomy alone would almost never be used. It would be used in combination with resection of the perforated segment.

Jejunal Ileal Disease
Approximately 10-20% of patients will have jejunal ileal disease (Farmer 1975). Bypass surgery is almost never done for this pattern of disease. The most common surgical procedure is resection with or without anastomosis depending on the clinical situation. However strictureplasty which was pioneered by Lee and Papaioannou (1982) is the procedure of choice to preserve small in appropriate setting. These include:

1. Diffuse involvement of the small bowel with multiple strictures

2. Stricture(s) in a patient who has undergone previous major resection(s) of small bowel (>100 cm)

3. Rapid recurrence of Crohn's disease manifested as obstruction

4. Stricture in a patient with short bowel syndrome

5. Nonphlegmonous fibrotic stricture

Relative contraindications for strictureplasty include:

1. Free or contained perforation of the small bowel

2. Phlegmonous inflammation, internal fistula, or external fistula involving the affected site

3. Multiple strictures within a short segment

4. Stricture in close proximity to a site chosen for resection (one would include this in the resected specimen)

5. Colonic strictures (as they may be cancerous)

6. Hypoalbuminemia (<2.0 g/dL)

Strictureplasty has proven effective therapy to relieve obstructive symptoms despite diseased bowel being left in situ. Two thirds of patients are able to be weaned from steroids and recurrence of disease is usually not at the strictureplasty site (Ozuner 1996) . The operation is safe in properly selected patients. In one study septic complications occurred in 7116 patients (6%) and were associated with an albumin less than 3.0g/dL. Only 2% required reoperation for sepsis (2 patients) (Fazio 1993). Additionally hemorrhage (transfusion > 3 units occurred in 4% and was usually managed successfully with selective mesenteric angiography and intraarterial vasopressin infusion (Ozuner 1995).

The long term results of strictureplasty are also quite promising. Of 162 patients undergoing 191 operations with 698 strictureplasties, followed for a mean of 42 months, 98% were relieved of obstructive symptoms and 2/3 ceased taking steroids (Ozuner 96). Reoperative recurrence affected only 28%. Of those requiring reoperation, 78% had new strictures at a location remote from the original strictureplasty site. The reoperative recurrence rate was statistically similar between strictureplasty alone (31%) and strictureplasty with resection (27%)

Colonic Disease
Colonic disease affects 20-30% of patients (Farmer 1975). In patients with pancolitis a total proctocolectomy with permanent ileostomy is the usual operative choice. In toxic patients or those with severe perianal disease or sepsis, a colectomy without proctectomy and with ileostomy is done. The rectum is then removed at least six months later after the sepsis/inflammation has subsided or the patient has recovered from their toxic state. If a colectomy only is done, we prefer to staple the colon in the mid to distal sigmoid and then sew it in the distal aspect of the incision. Therefore if the staple line breaks down, the patient is left initially with a wound infection and later with a colocutaneous out-of-circuit fistula instead of peritonitis from the leaking stump. If the colon is too diseased to "hold the staples", the distal end is brought onto the skin surface through the distal aspect of the incision and the fascia and skin are closed around it. The bowel is wrapped with gauze and then amputated and sewn to the skin in about a week.

When a proctectomy is done for Crohn's disease, the initial dissection starting at the pelvic brim is done in a similar manner for other rectal dissections. The presacral space is entered and the dissection is continued circumferentially to the pelvic floor. Since the operation is not typically done for cancer, the removal of the anus does not have to be radical. The two options used in handling the anal dissection include amputating the rectum at the level of the anorectal ring then 1) doing an anal canal mucosectomy and sewing the anal sphincter closed or 2) removing the mucosa and the internal sphincter by dissecting in the intersphincteric groove (the external sphincter is then sutured closed). Either of these approaches allows for preservation of tissue in an effort to avoid an unhealed perineal wound. Scammell and Keighley (1973) reported about 25% of their patients had an unhealed perineal wound at one year and 10% failed to ever heal. There is a spectrum of unhealed perineal wounds ranging from a fistula to a total open cavity.

After a proctocolectomy we prefer to drain the pelvis via closed suction drains placed lateral to the abdominal incision. Additionally if omentum is available, placing it along the gutter into the pelvis to obliterate the raw open space can be done. The drain is usually removed when the output is less than 50 cc in 24 hours.

For patients with rectal sparing, a colectomy with ileorectal anastomosis prevents or delays the need for a permanent ileostomy. For this operation to be optimal, anal sphincters should be normal and there should be no or minimal anal disease. The rectum must be compliant and distend to serve as a satisfactory reservoir. Usually this can be assessed with rigid proctoscopy examing for distensibility with insufflation of air.

The argument for segmental colonic resection in patients with Crohn's colitis is that it preserves part of the colon. However, while there is a high rate of recurrence of colonic disease it is reported in some studies that as many as 60% will maintain intestinal continuity for more than a decade after initial resection with anastomosis (de Dombal 1971, Prabhakar 1997). This operation is particularly considered in older patients and patients who have had a significant amount of small bowel resected. The amount of disease should be less than 10-20 cm of the colon with the remaining colon normal.

Some patients present with severe colonic disease and are either almost too ill to tolerate an abdominal operation, pregnant, or the colon is so friable that resection would risk perforation and spillage of fecal contents. In this situation an ileostomy with blowhole colostomy is done. This operation is contraindicated with known perforation. In recent times it is rarely needed, but it should be part of the armamentarium of any surgeon operating for Crohn's disease, as it may be life saving in some situations.

Perianal disease
The perianal abnormalities associated with Crohn's disease include edematous skin tags, hemorrhoids, cyanotic discoloration, fissures, canal ulceration, abscesses, fistulas, and anorectal strictures. Perianal disease is usually associated with colonic disease, but can be the only manifestation or associated with ileocecal or small bowel disease. A conservative approach is usually used with treatment of any of these problems and surgery is reserved for symptomatic problems. All abscesses need adequate drainage. Medical treatment with antibiotics is not enough. A mushroom-tipped catheter can be placed into the apex to further aid drainage.

The management of fistula represents one of the most challenging dilemmas. Most low lying fistula can be managed by fistulotomy. Occasionally, despite careful selection, the fistulotomy site will not heal and the patient will be left with an asymptomatic anal ulcer. Further operative treatment should be avoided and medical management employed. If the fistula encompasses a large amount of sphincter muscle and fecal incontinence is a concern, a chronic loose seton may be used to ensure adequate drainage. These non-cutting setons adequately satisfy the goals of therapy by reducing perianal drainage and pain without worsening fecal continence or risking proctectomy. The soft, nonreactive nature of Silastic vessel loops makes them ideal seton material for long-term fistula management .As with any chronic fistula the risk of malignant generation has been described and therefore the patient should be examined periodically.

Mucosal advancement flaps have been successfully used in patients without rectal disease, cavitating ulceration or anal stenosis.. More recently sleeve advancement flaps have been described for fistula associated with anal canal ulceration, or stricture, but a normal rectum (Marchesa 1998). This operation involves stripping off the anal canal mucosa (similar to a mucosectomy done for a handsewn pelvic pouch) and continuing the dissection cephalad to mobilize a 360 degree "sleeve" of full thickness rectum. The rectum is then advanced down to accomplish a formal procto-anal anastomosis. This operation is usually combined with a diverting stoma. Although the mobilization can usually be done transanally, the patient is warned about the possibility of a transabdominal mobilization if necessary.

Rectovaginal fistula presents an additional type of problematic fistula that can be treated with an advancement flap (Hull 1997). The associated disease dictates whether a simple advancement flap can be used or if a sleeve of rectum will need to be advanced. Additionally surgical treatment with a transvaginal flap has successfully been used.

Controversy exists regarding proximal disease when treating perianal disease. For example, do you surgically address the ileocecal disease when doing an advancement flap? The answer to this question has not been discovered, but it intuitively seems correct to treat proximal disease with medication until it is in remission or with surgery before expecting an advancement flap to be successful.

An additional dilemma is when to use a diverting stoma when doing an advancement flap. A stoma does not guarantee success with a flap repair. It is reasonable to consider a diverting stoma if there is technical difficulty, it is a redo operation, the experience of the surgeon is limited, or other adverse features exist.

Duodenal Crohn's disease
Symptomatic duodenal Crohn's disease is rare affecting 1-2% of cases. The most frequent problem is stricture leading to obstructive symptoms. Incontinuity bypass has been the preferred method of treatment. Some controversy exits if a vagotomy should be added to prevent marginal ulceration. In this day of highly potent acid eliminating drugs it is unknown if the vagotomy is necessary. If one is done a highly selective vagotomy may be a better choice over a truncal vagotomy to minimize diarrhea. Recently Worsey and Hull (1999) reported on performing strictureplasty for strictures amendable. Depending on the location of the stricture, a Finney or Heineke-Mikulicz strictureplasty can be done. They found the results were favorable to a bypass and avoided the question of needing a vagotomy.

Laparoscopic Surgery for Crohn's Disease
Laparoscopy continues to find new frontiers. In selected patients with Crohn's disease, laparoscopic assisted resection in feasible particularly with ileocecal disease (Ludwig 1996). Anastomoses however have been done extracorporeal. It is excellent for diversion in patients with symptomatic perianal disease or those needing diversion after a flap repair. It has also been used for colectomy in selected patients. As with all laparoscopic bowel surgery, the comfort of the surgeon with laparoscopic surgery continues to be the deciding factor along with associated problems like extensive adhesions in redo surgery, abscesses, and entero-entero or other fistula.

Conclusion

Crohn's disease is a complex disease. New medications such as Remicaid(TM) may change the indications and surgical procedures done in the future. However, until then careful planning for operative intervention and remembering that surgery does not "cure" the disease will aid in management and planning.

References

De Dombal FT, Burton I, Goligher JC. Recurrence of Crohn's disease after primary excisional surgery. Gut 12:519-527, 1971.

Farmer RG, Hawk WA, Turnbull RB. Clinical patterns in Crohn's disease: a statistical study of 615 cases. Gastroenterology 68:627-635, 1975.

Fazio VW, Alexander-Williams J, Oberhelman HA Jr, Goligher JC, Brotman M. Parenteral nutrition as primary or adjunctive treatment. Dis Colon Rectum 19:574-8, 1976.

Fazio VW, Marchetti F, Church J, Goldblum J, Lavery I, Hull T, Milsom J, Strong S, Oakley J, Secic M. Effect of resection margins on the recurrence of Crohn's disease in the small bowel. A randomized controlled trial. Ann Surg 224:563-573, 1996.

Fazio VW, Tjandra JJ, Lavery IC, Church JM, Milsom JW, Oakley JR. Long-term follow-up of strictureplasty in Crohn's disease. Dis Colon Rectum 36:355-361, 1993.

Higgens CS, Keighley MRB, Allan RN. Impact of preoperative weight loss on postoperative morbidity. JR Soc Med 74:571-5, 1981.

Hull TL, Fazio VW. Surgical approaches to low anovaginal fistula in Crohn's disease. Am J Surg 173:95-8, 1997.

Irving MH. The management of surgical complications in Crohn's disease-abscess and fistula. In Allan RN, Keighley MRB, Alexander-Williams J, Hawkins C (eds): Inflammatory Bowel Disease. 2nd ed. Edinburgh, UK, Churchill-Livingstone, 1990.

Lashner BA, Evans AA, Hanauer SB. Preoperative total parenteral nutrition for bowel resection in Crohn's disease. Digestive Diseases Science 34:741-6, 1989.

Lee ECG, Papaioannou N. Minimal surgery for chronic obstruction in patients with extensive or universal Crohn's disease. Ann R Coll Surg Engl 64:229, 1982.

Ludwig K, Milsom JW, Church JM, Fazio VW. Preliminary experience with laparoscopic intestinal surgery for Crohn's disease. Am J Surg 171:52-56, 1996.

Marchesa P, Hull TL, Fazio VW. Advancement sleeve flaps for treatment of severe perianal Crohn's disease. Br J Surg 85:1695-1698,1998.

Ozuner G, Fazio VW, Lavery I, Milsom J, Strong S. Reoperative rates for Crohn's disease following strictureplasty. Dis Colon Rectum 39:1199-1203, 1996.

Ozuner G, Fazio VW. Management of gastrointestinal bleeding after strictureplasty for Crohn's disease. Dis Colon Rectum 38:297-300, 1995.

Prabhakar LP, Laramee C, Nelson H, Dozios RR. Avoiding a stoma: role for segmental or abdominal colectomy in Crohn's disease. Dis Colon Rectum 40:71-8, 1997.

Sales DJ, Kirsner JB. The prognosis of inflammatory bowel disease. Arch Intern Med 143:294, 1983.

Scammell BE, Keighley MRB. Delayed perineal wound healing after proctocolectomy for Crohn's colitis. Br J Surg 73:150-2, 1986.

Whelan G, Farmer RG, Fazio VW, Goormastic M. Recurrence after surgery in Crohn's disease: relationship to location of disease (clinical pattern) and surgical indication. Gastroenterology 88:1826-1833, 1985.

Worsey MJ, Hull T, Ryland L, Fazio V. Strictureplasty is an effective option in the operative management of Duodenal Crohn's disease. Dis Colon Rectum in press 1999.