eMedicine Specialties > Pediatrics: Surgery > General Surgery

Crohn Disease: Surgical Perspective: Treatment

Author: Patricia A Valusek, MD, Pediatric Surgical Scholar, Department of Surgery, Children's Mercy Hospital; Staff Physician, Department of Surgery, Hennepin County Medical Center
Coauthor(s): Amina M Bhatia, MD, Fellow, Department of Pediatric Surgery, Emory University School of Medicine; George W Holcomb III, MD, Surgeon-in-Chief, Professor, Department of Pediatric Surgery, Children's Mercy Hospital, University of Missouri-Kansas City School of Medicine
Contributor Information and Disclosures

Updated: Jul 6, 2006

Treatment

Medical Therapy

Treatment strategies for patients with Crohn disease consist of attempts at medical remission and supportive medical management of chronic symptoms and exacerbations. Surgery is reserved for complications of intestinal disease or cases in which medical management is unsuccessful. Numerous medications are available to control acute inflammatory symptoms and prevent recurrence of intestinal disease. Although numerous studies regarding postoperative prophylactic therapy have been performed in adults, no prospective studies have been conducted in children. Nutritional therapies are focused on preventing the weight loss and malnutrition that frequently accompany the disease.

Aminosalicylates

Oral and topical salicylates are often first-line medications in the treatment of acute exacerbations of Crohn disease. Sulfasalazine was the first salicylate to be used in inflammatory bowel disease. It consists of a sulfapyridine moiety (the carrier compound) linked to 5-aminosalicylic acid (the active ingredient). After reaching the colon, sulfapyridine is released from the 5-aminosalicylic acid by means of colonic bacteria. The salicylate compound acts locally to prevent inflammation by inhibiting various elements of the inflammatory cascade. Sulfasalazine is effective in active ileocolonic and colonic disease but less effective in isolated small-bowel disease. It is often helpful in preventing recurrence after surgery to treat active disease.

Numerous adverse effects, including gastrointestinal symptoms (eg, nausea, vomiting, abdominal pain) are associated with the sulfapyridine moiety and limit patients' tolerance of this medication. Newer oral compounds, such as mesalamine (Asacol, Pentasa), olsalazine (Dipentum), and balsalazide (Colazal), lack the sulfapyridine carrier; they are better tolerated than sulfapyridine. In addition, topical aminosalicylates in the form of suppositories and enemas may be of benefit in patients with distal colonic Crohn disease.

Corticosteroids

Glucocorticoids have long been known to be highly effective in the treatment of acute episodes of Crohn disease. They induce remission in all disease locations. However, long-term corticosteroid treatment does not have a role in maintaining remission in patients with quiescent disease or in management after medical or surgical treatment of active disease. High-dose systemic steroids may be administered for weeks to months as treatment of active disease.

Corticosteroids have many adverse effects, including the development of cushingoid features, hypertension, hyperglycemia, cataracts, osteoporosis, osteonecrosis, and psychological effects. In children, growth retardation, delayed onset of puberty, and delayed bone maturation are particular concerns related to repeated steroid treatments. Newer steroids, such as budesonides, are rapidly metabolized during their first pass through the liver; therefore, their potential for adverse effects is limited. Topical steroid enemas may be used in distal Crohn colitis without the systemic effects.

Antibiotics

Enteric bacteria may promote intestinal inflammation, and antibiotics aimed at the intestinal flora have been successfully used in both intestinal and perianal Crohn disease. Metronidazole is most widely used in perianal Crohn disease; it can promote complete healing of perianal fistulas. If administered after ileal resection, metronidazole can effectively decrease postsurgical disease recurrence, as shown in randomized trials. However, symptoms often recur after the drug is stopped, and adverse effects often limit its use. Ciprofloxacin or other broad-spectrum antibiotics may be used as alternatives, with similar effectiveness.

Immunosuppressants

Azathioprine and its metabolite, 6-mercaptopurine, are inhibitors of purine synthesis. They are used in the treatment of both active and quiescent Crohn disease. In a randomized controlled trial, mercaptopurine offered significant benefit in the treatment of active disease and disease with fistulas. Moreover, azathioprine and 6-mercaptopurine therapies allow for early reduction and cessation of corticosteroid treatment during acute episodes. Unlike salicylates and steroids, the immunosuppressants are effective as maintenance therapies for quiescent disease. Several months of treatment are usually required in order to achieve an effect. The optimal duration of therapy remains unclear.

Dose-dependent adverse effects include nausea, rash, marrow toxicity, hepatitis, and acute pancreatitis. Although no evidence suggests an increased risk of solid tumors in adults, concerns about lymphoreticular malignancies have limited the use of these medications in children.

Cyclosporine is commonly used as an immunosuppressant with organ transplantation. In a randomized prospective trial, high-dose oral cyclosporine was effective in the treatment of active Crohn disease. Its rapid onset of action (<2 wk) makes it an attractive bridge therapy until azathioprine or 6-mercaptopurine become effective. The effects of cyclosporine continue for several months after cessation of its administration. Nephrotoxicity, hypertension, electrolyte abnormalities, gingival hyperplasia, and paresthesias are the most common adverse effects.

Alternative immunosuppressants such as tacrolimus (FK-506) and mycophenolate mofetil are being explored as treatments for Crohn disease. Preliminary data suggest that these treatments have some benefit in patients with severe inflammatory bowel disease.

Biologic therapies

Tumor necrosis factor-alpha (TNF-alpha) is an inflammatory cytokine that acts as a primary mediator of intestinal inflammation and injury in inflammatory bowel disease. Preliminary evidence from clinical trials involving antibodies against TNF-alpha has shown promise in the treatment of patients with active and quiescent disease.

Approved by the US Food and Drug Administration (FDA) in 1998, infliximab is a chimeric antibody that specifically targets TNF-alpha. Data from noncontrolled studies suggest an improvement in the endoscopic and histologic appearance of chronic active disease after a single infusion of infliximab. A benefit has also been observed in refractory enterocutaneous and perianal fistulae.

CDP571 is a human monoclonal anti-TNF-alpha antibody that may be advantageous in patients with refractory Crohn disease. Other potential therapies being evaluated include thalidomide, antisense oligonucleotides against intercellular adhesion molecule-1, recombinant interleukin-10 and interleukin-11, and anti-CD4 antibodies.

Nutritional therapy

Although ineffective as a primary therapy, nutritional manipulations, which allow the bowel rest, can be effective adjuncts in the treatment of active Crohn disease. Both parenteral and enteral nutrition are effective. However, because of the associated risks and costs of parenteral nutrition, it is typically reserved for the postoperative period. Enteral nutrition has the benefit of altering the bacterial flora and providing direct nourishment to the intestinal mucosa. The effectiveness of enteral nutrition in active disease was shown in several randomized trials. However, relapse commonly occurs after the cessation of enteral feeding. Supplemental enteral nutrition at night without dietary restrictions during the day is beneficial in maintaining disease remission. To the authors' knowledge, no benefit of elemental diets compared with conventional enteral nutrition has been shown.

Surgical Therapy

Most patients with Crohn disease require surgical intervention during their lifetime. Unlike ulcerative colitis, Crohn disease has no surgical cure. Within 20 years of the onset of symptoms, 80% of patients with Crohn disease require surgery, and many require multiple procedures. About 20-30% of patients have a recurrence of disease within the first postoperative year. Hence, every attempt at conserving the small bowel should be made in the surgical approach to Crohn disease. Despite this approach, repeated intestinal resection for Crohn disease is a major cause of short-bowel syndrome.

The most common complication of Crohn disease is small-bowel obstruction, which occurs in 30-50% of patients. The obstruction is typically due to intestinal strictures from repeated bouts of inflammation and subsequent fibrosis. In the case of a complete obstruction or a partial obstruction refractory to nonsurgical management, surgical intervention is required. Surgical options for intestinal strictures include resection of the strictured bowel or stricturoplasty. In cases of long strictures (>12 cm) or multiple strictures in close proximity, surgical resection with primary anastomosis is often required.

Stricturoplasty for multiple short strictures has the benefit of bowel conservation. A Foley catheter (inflated to 25 mm) can be passed through the lumen to detect additional distal strictures. The strictured bowel is incised longitudinally to a point 1-2 cm beyond the narrowing and then closed transversely without resection. For long or multiple confluent strictures, a stricturoplasty that resembles a Finney side-to-side pyloroplasty can be used to conserve bowel length. Hydrostatic balloon dilatation of ileocolic strictures has been performed, but its effects may not be long-lasting. Bypass procedures are usually reserved for duodenal obstructions.

Other complications of Crohn disease that may require surgical intervention include free perforation, abscesses, fistulas, toxic megacolon, and massive hemorrhage. More than 10% of patients with Crohn disease have an intra-abdominal or pelvic abscess during their lifetime. Abscesses must be drained either surgically or percutaneously. Although surgical drainage is most often successful, an attempt at percutaneous drainage may spare some patients an operation.

Because of the transmural nature of the inflammation, fistula formation is common. Enteroenteric, enterocutaneous, enterovesical, and rectovaginal fistulas may often be initially treated using the principles of fistula healing and medical therapy. If medical therapy is unsuccessful, resection of the involved bowel is required in symptomatic patients.

Toxic megacolon and massive hemorrhage are uncommon complications of Crohn disease. However, they may require urgent bowel resection. Total abdominal colectomy with a Hartmann pouch has been advocated for fulminant toxic megacolon. This allows future restoration of bowel continuity with a sphincter-preserving ileorectal anastomosis. However, a permanent ileostomy may ultimately be required due to recurrent rectal disease.

Perianal Crohn disease presents a particularly difficult management challenge. Fissures, fistulas, and abscess may be multiple and recurrent, and repeat operations may lead to sphincter damage and incontinence. True abscess require drainage. When a fistula tract can be identified, a silicone Seton can be used to prevent premature skin closure and recurrent abscesses. These indwelling Setons should be left in place for at least 12 months to allow complete epithelialization of the tract. This approach leads to a chronically draining fistula tract. In patients in whom severe perianal disease has destroyed the sphincter, proctectomy with permanent ileostomy may be necessary.

Laparoscopic resection

The laparoscopic approach to Crohn disease has been shown to be feasible as well as safe. Complications of Crohn disease such as abscesses, phlegmons, and recurrent disease have been safely treated laparoscopically and are not contraindications to laparoscopy in these patients.

Although many surgeons still perform open resection and though it should be considered the criterion standard, the laparoscopic approach is being used with increasing frequency. In children, laparoscopic intestinal resections have usually been reserved for proctectomy and pull-through procedures in Hirschsprung disease. Segmental intestinal resections in Crohn disease can easily be accomplished as well. No difference in recurrence rates are found in adults undergoing laparoscopic versus open ileocolic resection. To this authors' knowledge, no data have been published regarding recurrence rates in children undergoing open versus laparoscopic resection.

Over a 5-year period at Children's Mercy Hospital, 18 patients underwent laparoscopic segmental resection of the terminal ileum and cecum with primary anastomosis of the ileum to the colon.

Surgical technique

After the patient is given general endotracheal anesthesia and after a urinary catheter is introduced, the abdomen is prepared and draped widely. A 12-mm incision is made in the umbilicus through which a 12-mm cannula is introduced for future insertion of the endoscopic stapling device. Two 5-mm incisions are made, 1 in the left mid-abdomen and 1 in the left suprapubic region; through these, grasping forceps are inserted for retraction.

The final port is initially 5 or 10 mm long and is placed in the right lower abdomen in a location similar to that used for open appendectomy incision. This incision is subsequently enlarged to approximately 2 cm, and the specimen is extracted from the abdominal cavity through this incision (see Image 2). In addition, the 2 ends of the intestine to be anastomosed are exteriorized through this incision and a 2-layer extracorporeal anastomosis is created.

The first step in the operation is ligation and division of the proximal ileum with the endoscopic stapler. Next, with either an ultrasound-activated scalpel (UltraCision Harmonic Scalpel; Ethicon Endosurgery, Cincinnati, OH) or sealing device (Ligasure; Valley Lab, Boulder, CO), the mesentery of the proximal right colon is coagulated and transected (see Image 3). Then, the right lower abdominal incision is enlarged to 2 cm, and the specimen is exteriorized. With this technique, the distal margin of resection is most precisely determined, and the distal resection margin can be divided with the surgical stapler. This procedure may also be performed intracorporeally with an endoscopic stapler.

Once the resected specimen is removed, the proximal small intestine is delivered through the right lower abdominal incision and a 2-layer extracorporeal anastomosis is created between the proximal and distal margins. The bowel is then returned to the abdominal cavity, and all incisions are closed.

In the limited experience at Children's Mercy Hospital, all patients were discharged home on either the fourth or fifth postoperative day. Nasogastric tubes were not placed to improve the patients' comfort during postoperative convalescence. No intraoperative or postoperative complications have developed in these patients.

Preoperative Details

A recent evaluation of the extent of intestinal disease with appropriate radiologic and endoscopic studies is essential. Steroids are tapered as much as tolerated, and the patient's nutritional status is optimized.

Thorough bowel cleansing is required in patients without intestinal obstruction. As an alternatively, patients with partial chronic obstructions for whom bowel preparation is unsafe can drink clear liquids for several days.

In patients who may receive stomas, preoperative counseling best prepares the patients and their families. A stomal therapist should be involved with the patient's care before surgery. Patients should also be counseled about their expectations for surgery because future recurrences are likely.

Intraoperative Details

Most patients will have recently received corticosteroids. Therefore, perioperative steroid dosing is likely to be required.

Perianal, rectal, and sigmoidoscopic examinations are often performed while the patient is under anesthesia to determine the presence and extent of perianal disease.

The goal of surgical resection is to remove the grossly involved bowel; microscopic disease at the resection margins is acceptable. Primary anastomosis of bowel can usually be achieved. On occasion, a proximal functioning stoma or Brooke ileostomy is required in patients in whom an anastomosis is unsafe.

Postoperative Details

After surgery, steroids are appropriately tapered. Patients who were receiving a low-dose or short-term steroid before surgery may be treated with a relatively rapid taper. Parenteral nutrition is often continued until bowel function returns.

Complications

The most common complications of surgery for Crohn disease are intraperitoneal adhesions. Patients with Crohn disease who are undergoing abdominal surgery are also at increased risk for developing enterocutaneous fistulas as a result of surgery.

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