Pediatric Crohn Disease Surgery Guidelines

Updated: Oct 26, 2020
  • Author: Patricia A Valusek, MD; Chief Editor: Harsh Grewal, MD, FACS, FAAP  more...
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SAR/SPR/AGA Guidelines on Enterography in Small-Bowel Crohn Disease

In 2017, an expert panel, which included contributors from the Society of Abdominal Radiology (SAR) Crohn’s Disease–Focused Panel, the Society of Pediatric Radiology (SPR), and the American Gastroenterological Association (AGA), issued the following guidelines on the use of computed tomography enterography (CTE) and magnetic resonance enterography (MRE) in patients with small-bowel Crohn disease (CD) [22] :

  • The number of involved bowel segments and their location, as well as the length and degree of upstream dilatation of Crohn strictures, should be reported by radiologists to help gastroenterologists and surgeons determine the best therapeutic plan.
  • Radiologists should state if mural inflammation is present when describing areas with stricture or penetrating disease.
  • Cross-sectional enterography should be performed at CD diagnosis.
  • Consider cross-sectional enterography for disease monitoring in patients with small-bowel disease or penetrating complications.
  • While a dedicated pelvic magnetic resonance (MR) study is needed in patients with perianal disease, all CTEs and MREs should also include imaging of the anus.
  • Radiologists should comment on and describe intramural T2 hyperintensity, restricted diffusion, perienteric stranding, wall thickness, and mural ulcerations seen on imaging, because they typically correlate with disease severity.
  • MRE is preferred over CTE to estimate response to medical treatment in patients with asymptomatic disease.
  • Noncontrast MRE with T2-weighted and diffusion-weighted imaging is an “acceptable alternative” when intravenous contrast agents cannot be used.
  • Radiologists should evaluate CTE and MRE examinations for signs of mesenteric venous thrombosis, occlusions, or small-bowel varices.

ESPGHAN Guidelines for Crohn Disease Surgery in Children

In 2017, the European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) pediatric inflammatory bowel disease (IBD) Working Group developed the following guidelines for surgical management of CD in children. [71]

Considerations for surgery

Surgery may be considered as an alternative to medical therapy for pediatric CD when a patient has active disease limited to a short segment(s) despite optimized medical treatment. 

Surgery should be considered in children in prepubertal or pubertal stage if growth velocity for bone age is reduced over a period of 6-12 months in spite of optimized medical and nutritional therapy. 

A complete assessment of the patient's general and bowel condition is recommended before elective surgery to optimize the surgical approach, minimize the length of bowel resection, and reduce the risk of complications. It should include history, physical examination, ileocolonoscopy, imaging studies, screening for concomitant infections, and nutritional deficiencies. 

Selection of type of surgery

Limited resection should be performed when a patient has localized small-bowel or colonic CD not responsive to medical therapy. 

Strictureplasty must be considered when a symptomatic patient has multiple short strictures in the small bowel.

Extensive resections of the small bowel should be avoided because they pose a long-term risk of development of short-bowel syndrome.

When a patient has pancolonic disease, the choice of surgery is subtotal colectomy and ileostomy. Later ileorectal anastomosis (IRA) can be performed if the rectum is spared and there is no significant perianal disease. One-stage IRA is generally not advised.

Ileal pouch-anal anastomosis (IPAA) is not recommended when a patient has CD.

Risk factors for complications of surgery and perioperative managememt

Corticosteroids exposure should be minimized before surgery to reduce surgery-related complications such as infections.

Anti–tumor necrosis factor alpha (TNF-α) administration during the immediate perioperative period is discouraged because it is associated with an increased risk of infection.

Nutritional status should be optimized and anemia corrected to reduce the risk of postoperative complications.

A patient should cease smoking before surgery, given the strong association of smoking with postoperative recurrence.

Long-term postoperative complications

Vitamin B12 levels (active vitamin B12) should be routinely monitored in patients who undergo resection of >20 cm of terminal ileum.

Bile acid malabsorption should be suspected in patients with persistent diarrhea despite clinical remission or limited disease activity after ileal resection.

The patient should be informed that after surgical resection, the risk of bowel obstruction is increased (secondary to either recurrent disease or adhesions).

Follow-up after surgery

Postoperative medical treatment should be based on ileocolonoscopy assessment and not solely on symptoms or serum inflammatory markers. Repeated fecal biomarkers testing may aid in deciding on the timing of endoscopy. 

Prevention of recurrence

Thiopurines may be used for preventing postoperative recurrence when a child has a moderate risk of CD recurrence.

When thiopurines have failed preoperatively, their postoperative use requires careful risk-benefit analysis.

Treatment with anti–TNF-α is recommended when a CD patient has a high risk of postoperative recurrence or has widespread disease.

When a patient experiences endoscopic recurrence after intestinal resection despite optimal thiopurine treatment, the therapy should be escalated to anti–TNF-α medication.

Perianal disease

Pelvic magnetic resonance imaging (MRI) and evaluation under anesthesia by a surgeon with experience in pediatric anorectal disease should be among the initial procedures in evaluating a child with suspected complex perianal CD.

Disease extension should be reevaluated by means of ileocolonscopy in all patients with complex perinanal disease manifesting after primary diagnostic investigations of CD. Concomitant intestinal lesions (inflammation, stenosis) have both prognostic and therapeutic relevance.

Treatment of perianal CD should be based on a combination of surgery, antibiotics and biologics.

The presence of perianal abscess should be ruled out early, and when it is detected, drainage should be discussed with the surgeon.

Placement of a noncutting seton should be considered in all complex fistula tracts, especially those with recurrent abscesses.