Newsletters - February 2010
Surgical Management of Inflammatory Bowel Disease in Children
by Thomas T. Sato, MD, FACS, FAAP, Professor of Surgery, Medical College of Wisconsin, Children’s Hospital of Wisconsin
Inflammatory bowel disease (IBD) describes a spectrum of idiopathic, lifelong and progressive intestinal inflammatory conditions that includes Crohn’s disease, ulcerative colitis and indeterminate colitis. Genetic and environmental factors appear to have significant impact on IBD pathogenesis, and there has been a worldwide increase in the incidence rates of Crohn’s disease and ulcerative colitis. In comparison to adults, IBD occurring in the pediatric population has a number of unique clinical characteristics and surgical management considerations.
A recent systematic, statewide population-based study in Wisconsin demonstrated a pediatric IBD incidence rate of 7.05 per 100,000.1 This is the highest pediatric IBD incidence rate reported in the world to date. The incidence rate of Crohn’s disease was 4.56 per 100,000, more than twice the rate of ulcerative colitis, and significantly more boys were diagnosed with Crohn’s disease compared to girls. Additionally, there was equal disease distribution across all ethnicities, and no effect of population density on disease incidence (rural versus urban living environment). Data from the prospective, statewide pediatric IBD registry demonstrated that the majority of children with ulcerative colitis present with symptomatic pancolitis, and that extraintestinal manifestations in newly diagnosed children with IBD are uncommon.
Pediatric Crohn’s Disease: Indications for Operative Management
Current medical and surgical management of Crohn’s disease is designed to control intestinal inflammation and improve the quality of life in children. Contemporary treatment of Crohn’s disease requires multidisciplinary management involving pediatric gastroenterologists, pediatric surgeons, radiologists and dietitians. Effective management must account for age-specific issues such as growth and development, delayed puberty, school attendance and ability to interact with family and peers on a social and emotional level.
Surgical management of pediatric Crohn’s disease is targeted toward disease control and treatment of disease complications. The major indication for operative management is failure to control disease activity despite optimal medical management. Virtually all children with established IBD will be medically managed with immunomodulating drugs, and potential drug effects must be accounted for in the perioperative period. Operative intervention is indicated when complications of the disease occur, such as intestinal perforation, abscess, bowel obstruction, fistula or hemorrhage. Unique to the child or adolescent, growth failure, nutritional deficiency and delayed puberty are clinically significant issues that may lead to operation despite medically controllable symptoms. The use of chronic steroids to suppress IBD symptoms has been largely abandoned. Finally, quality of life issues such as subjective pain, school attendance, and self-esteem are relative indications for operative intervention [See Table 1].
Table 1. Indications for Operative Management in Pediatric IBD
Children and their families should be counseled that Crohn’s disease is a lifelong inflammatory condition and there is currently no curative treatment. Therefore, operative management is directed at providing symptom relief or treating disease complications while preserving intestinal length. Successful operations for Crohn’s disease require accurate localization of clinically significant disease. A careful history and clinical examination may help to identify the presence of a palpable right lower quadrant mass from an inflammatory phlegmon or the presence of perianal or enterovesical fistula. Pediatric patients require esophagogastroduodenoscopy and colonoscopy with biopsy under general anesthesia for definitive diagnosis. In the presence of persistent symptoms despite escalating medical therapy, helical CT scan imaging of the abdomen and pelvis with intravenous and oral contrast is the most helpful diagnostic imaging modality. More recently, wireless capsule endoscopy has been successfully used to identify small intestinal disease in children with equivocal endoscopic results or contrast studies. Retention of the capsule from intestinal stricture in Crohn’s disease is an indication for prompt, but not emergent, operative exploration.
Other than endoscopy, the most commonly performed operation for pediatric Crohn’s disease is ileocecal resection. Resection of involved bowel is limited to grossly involved disease, as frozen section analysis to clear microscopic margins does not improve outcome.2 Open or laparoscopic segmental intestinal resection with primary anastomosis is preferred when feasible.3 There are no pediatric-specific, evidence-based data comparing hand-sewn to stapled anastomosis in childhood Crohn’s disease. Additionally, it remains unclear whether a minimally invasive approach will lead to a lower incidence of postoperative adhesive small bowel obstruction. In the setting of established intra-abdominal infection or inflammatory phlegmon with fistula, resection of the grossly diseased intestine with temporary diverting enterostomy may be required. For a chronically inflamed, fibrotic stricture seen more commonly in older adolescents and adults, site-directed stricturoplasty is preferred. Children undergoing exploration for suspected appendicitis who are found to have Crohn’s disease should be referred to a pediatric gastroenterologist for complete work-up. An appendectomy can safely be performed in this setting if the appendiceal base and cecum are not inflamed.
Persistent inflammatory symptoms of Crohn’s colitis despite optimal medical management is well-treated by segmental or total abdominal colectomy. Current data support the concept of performing segmental or total abdominal colectomy with primary anastomosis, avoiding diverting colostomy or ileostomy when possible.4 While ileoanal pouch reconstruction is the procedure of choice for children with ulcerative colitis, results for pouch procedures with Crohn’s disease have been discouraging secondary to disease recurrence and higher reoperation and complication rates.5 Perianal disease is observed in approximately 13 percent of pediatric patients with Crohn’s disease and may be anatomically complex. Severe, intractable perianal Crohn’s disease generally occurs with established colorectal disease and may require colectomy with diverting colostomy for symptom control.
Postoperative complications following operations for Crohn’s disease are, unfortunately, common and include small bowel obstruction and intra-abdominal infection.6 Recurrent disease should be anticipated, particularly in children with colonic disease or severe, aggressive disease at the time of initial operation. Recurrence rates of symptomatic disease following initial operation have been reported to be 17 percent at one year, 38 percent at three years and 60 percent at five years.7 Whether contemporary recurrence rates are lowered by the use of maintenance therapy and biological modifiers remains to be determined.
Surgery for Ulcerative Colitis
Compared to adults, at least 25 percent of children with ulcerative colitis require urgent or emergent operation for failure of optimal medical management, gastrointestinal bleeding, intestinal perforation, toxic megacolon or systemic sepsis [See Table 1]. Hematochezia requiring transfusion is common in severe pediatric ulcerative colitis. In the emergent setting, typically occurring with concomitant high-dose corticosteroids and immunosuppressive agents, most surgeons prefer subtotal colectomy with diverting end ileostomy. Restorative procedures are performed electively weeks to months later. Colon cancer is rare in the pediatric population. The estimated risk of developing carcinoma with ulcerative colitis is approximately 1 percent per year after eight to 10 years of active disease; therefore, ongoing endoscopic surveillance and biopsy is required. Similar to adults, dysplasia is an absolute indication for colectomy.8
In contrast to operations for Crohn’s disease, ulcerative colitis can be surgically cured with acceptable morbidity and excellent long term outcome. Therefore, timing of operative intervention for pediatric ulcerative colitis should account for symptom severity, quality of life issues, surgeon experience and patient/parent preferences. Operative management of ulcerative colitis is aimed at removing the colon and rectum with preservation of anal sphincter integrity and function. Definitive management of pediatric ulcerative colitis includes removal of the rectal mucosa—the use of endorectal dissection in children with ulcerative colitis was popularized in 1978 by Martin and LeCoultre, applying the dissection technique described for the treatment of Hirschsprung’s disease by Soave.9
The most commonly performed operation for pediatric ulcerative colitis is total abdominal proctocolectomy with either a direct (straight) ileoanal anastomosis or construction of an ileoanal pouch (J-pouch) anastomosis. Pouch reconstruction was driven by the desire to reduce stool frequency and urgency. Data support this concept as stool frequency and rates of urgency and nocturnal incontinence tend to be lower following ileoanal pouch procedures compared to straight ileoanal reconstruction. This difference tends to diminish over time, and therefore, decisions between these two approaches should reflect consideration of the patient’s age, lifestyle, preference and surgeon experience. Excellent outcomes have been reported using total abdominal proctocolectomy with ileoanal pouch reconstructive procedures in children and adolescents. These procedures are currently being performed using minimally invasive surgical techniques with similar excellent outcomes.10 There are no pediatric-specific data comparing hand-sewn to stapled ileoanal anastomosis, but the latter approach has gained increased popularity in the setting of minimally invasive approaches. Postoperative complications are common and include anastomotic leak, stenosis, infection, small bowel obstruction and pouchitis. Less commonly, bladder dysfunction, impotence, dyspareunia, infertility and ileovesical or ileovaginal fistula have been observed.
Pediatric patients with symptomatic Crohn’s disease treated with operative resection will generally have a good to excellent outcome. Most children return to activity and school, and there may be significant postoperative improvement in weight for age, height for age, weight for height and resting energy expenditure.11 However, patients and parents must be educated that because surgical intervention is largely directed at complications of a life-long disease, continued medical treatment is required and a high likelihood of future operative procedures exists. Most children and adolescents report good to excellent quality of life following colectomy and ileoanal reconstruction for ulcerative colitis.12 A reasonable long-term functional goal is normal fecal continence, stool frequency of four to six bowel movements per 24 hours, with normal urinary and sexual function. Children and adolescents require modification of eating behavior for optimal outcome, and initially, anti-diarrheal medications such as loperamide or diphenoxylate and supplemental bulk fiber agents are commonly used.
IBD remains an infrequent but important surgical problem in the pediatric population. The contemporary treatment of pediatric IBD is a model for the multidisciplinary management of potentially life-long disorders. Well-planned surgical management of Crohn’s disease and ulcerative colitis can have a major impact on growth, development, and quality of life in children and adolescents with IBD.
The Inflammatory Bowel Disease Center at Children’s Hospital of Wisconsin is a nationally recognized center designed to provide comprehensive care for children and adolescents with IBD. The center is under the direction of Michael Stephens, MD, assistant professor of Pediatrics in the Division of Pediatric Gastroenterology. Faculty members of the Division of Pediatric Surgery have advanced pediatric laparoscopic and minimally invasive skills to provide contemporary surgical management of IBD. For an appointment, call Children’s Hospital Central Scheduling at 414-607-5280 or 877-607-5280.
Dr. Sato can be reached at 262-266-6550 or firstname.lastname@example.org.
Kugathasan S, Judd RH,Hoffman RG et al. Epidemiologic and clinical characteristics of children with newly diagnosed inflammatory bowel disease in Wisconsin: a statewide, population-based study. J Pediatr 2003;143:525-531.
Telander RL, Schmeling DJ. Current surgical management of Crohn’s disease in childhood. Semin Pediatr Surg 1994;3:19-27.
von Allmen D, Markowitz JE, York A, Mamula P, Shepanski M, Baldassano R. Laparoscopic-assisted bowel resection offers advantages over open surgery for treatment of segmental Crohn’s disease in children. J Pediatr Surg 2003;38:963-965.
Andersson P, Olaison G, Bodemar G, et al. Surgery for Crohn colitis over a twenty eight-year period: fewer stomas and the replacement of total colectomy by segmental resection. Scand J Gastroenterol 2002;37:68-73.
de Oca J, Sanchez-Santos R, Rague JM, et al. Long-term results of ileal pouch-anal anastomosis in Crohn’s disease. Inflamm Bowel Dis 2003;9:171-175.
Patel HI, Leichtner AM, Colodny AH, Shamberger RC. Surgery for Crohn’s disease in infants and children. J Pediatr Surg 1997;32:1063-1068.
Baldassano RN, Han PD, Jeshion WC, et al. Pediatric Crohn's disease: risk factors for postoperative recurrence. Am J Gastroenterol 2001;96:2169-76.
Fonkalsrud EW. Surgical management of ulcerative colitis in childhood. J Pediatr Surg 1994;3:33-38.
Martin LW, LeCoutre C. Technical considerations in performing total colectomy and Soave endorectal anastomosis for ulcerative colitis. J Pediatr Surg 1978;13:762-764.
Georgeson KE. Laparoscopic-assisted total colectomy with pouch reconstruction. Semin Pediatr Surg 2002;11:233-236.
Sentongo TA, Stettler N, Christian A, et al. Growth after intestinal resection for Crohn's disease in children, adolescents, and young adults. Inflamm Bowel Dis 2000;6:265-269.
Shamberger RC, Masek BJ, Leichtner AM,Winter HS, Lillehei CW. Quality of-life assessment after ileoanal pull through for ulcerative colitis and familial adenomatous polyposis. J Pediatr Surg 1999;34:163-165.