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Key Points

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  1. Inflammatory bowel disease is a serious but treatable condition in children.

  2. Differentiating between Crohn disease and ulcerative colitis (UC) is extremely important.

  3. In Crohn disease, surgical management should emphasize preservation of bowel length and relief of obstructing lesions.

  4. Restorative proctocolectomy with colectomy is the treatment of choice for children with UC.

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Introduction

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Inflammatory bowel diseases (IBD) comprise a heterogeneous group of chronic inflammatory disorders of the gastrointestinal tract. Crohn disease (CD), ulcerative colitis (UC), and indeterminate colitis (IC) are thought to develop from dysregulation of the immune system in a genetically susceptible host. Incident symptoms occur in childhood in up to one third of patients with CD and UC. Despite the fact that most pediatric patients with IBD initially respond to steroid therapy, surgical intervention is required on an average 2 years later. Establishing the correct diagnosis of CD versus UC has important implications for surgical care.

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Pediatric surgeons must be aware that the majority of children present with colitis regardless of their eventual diagnosis. Epidemiologic studies suggest that up to 80% to 90% of children with CD experience colonic disease (colon only or ileocolonic), whereas only approximately 50% of adults have colonic involvement. Additionally, pediatric UC patients present more often with pancolitis as compared to adult patients who present more commonly with left-sided disease or proctitis. Adding to these disease similarities is the finding that pediatric CD presents predominantly with inflammatory and nonpenetrating phenotypes. Complicated forms of CD, such as stricturing and penetrating phenotypes, do occur in childhood; however, complicated CD more commonly results from a progressive disease despite contemporary treatment. Thus, the diagnostic dilemma of colitis in a child may not be differentiated to CD or UC until the teenage years when the adult distribution of IBD evolves. Interestingly, the incidence of CD appears to be increasing by population studies, whereas UC has remained essentially unchanged over time. To further cloud the situation, boys are at higher risk than girls for CD by a ratio of 2:1, whereas gender distribution is nearly equal in adult patients. Ultimately, the diagnosis of CD versus UC changes the indications for operation and the choice of surgical procedure.

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Clinical Presentation

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The classic presentation for CD is abdominal pain, diarrhea, poor appetite, and weight loss. Children with UC may have similar symptoms that include chronic (>2 weeks) abdominal pain, hematochezia, and tenesmus. Inflammatory extraintestinal manifestations occur in 25% to 35% of patients with either CD or UC and commonly affect the skin, joints, liver, eye, and bone. Indeed, extraintestinal manifestations of CD are more common in children with colonic disease as compared to isolated small-bowel CD involvement. Growth delay, which is commonly associated with CD, also occurs in severe UC, which is more common in pediatric patients.

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A genetic predisposition has been found in pediatric patients with IBD. Population-based studies have shown that 10% ...

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