Oral lesions may be treated with topical prednisolone syrup (5 mg/5 mL) or dexamethasone (0.5 mg/5 mL) 5 mL painted to lesions or swished and spit twice daily. Triamcinolone 0.1% may be used on lips two to four times per day. If disease is limited to the rectum, topical therapy is used. Rectal hydrocortisone enemas or foam or topical 5-aminosalicylate (5-ASA) enema/suppository may be used.
In both pediatric CD and UC, corticosteroids are used for induction but not maintenance of remission. Steroids are used for 2 to 4 weeks and then tapered off and ideally used for less than 4 months to diminish side effects and growth disturbances. Oral corticosteroids are used with mild disease (Prednisone 1–1.5 mg/kg per day, max 40–60 mg). Budesonide is less effective but has less adverse effects due to its high first-pass hepatic metabolism and decreased systemic effect. Oral corticosteroids are escalated to IV steroids (methylprednisolone 1 mg/kg per dose q12 h, max 60 mg per day) if the patient is unresponsive to oral corticosteroids in 1 to 2 weeks. IV steroids are initially started with severe disease (see ED care below).
5-ASA is used for both remission induction and maintenance in pediatric IBD though there are no randomized controlled trials published in children. Examples include sulfasalazine, mesalamine, olsalazine, and balsalazide. 5-ASA agents inhibit synthesis of proinflammatory prostaglandins and leukotrienes in the colon. Slight exacerbation of watery diarrhea is common during the first few weeks of 5-ASA preparations, but initial worsening of colitis symptoms (cramps, diarrhea, and rectal bleeding) indicates an adverse reaction and most commonly occurs with mesalamine. These patients are typically then termed “allergic” to 5-ASA preparations. 5-ASA agents are ineffective during an acute exacerbation and should be discontinued during a flare.
Immunomodulators (6-mercaptopurine, 6-MP, azathioprine, AZA, methotrexate): 6-MP and AZA show efficacy in inducing remission in UC and maintaining remission in pediatric CD. Both 6-MP and AZA have been shown to induce remission in 70% to 80% of steroid dependent CD but may take 2 to 4 months to work. Methotrexate has quicker onset of action but there are only small trials of its use in children.48
Biologic Agents (Infliximab, cyclosporine, and tacrolimus)
Infliximab (most commonly used) is a chimeric monoclonal antibody directed against the cytokine tumor necrosis factor alpha. It is used both to induce and maintain remission in IBD resistant to steroids and Immunomodulators. Success rates are high but a patient may require repeated doses and treatment for a long period of time.14,49 Infusion reactions occur in 15% to 25% of patients. Initial symptoms appear like anaphylaxis including laryngospasm, shortness of breath, acute chest discomfort, hemodynamic instability, and mucosal irritability. Delayed symptoms occur 2 to 14 days after an infusion and include fever, arthritis/arthralgias, myalgias, malaise, urticarial rash. Premedication does not prevent initial infusion reaction but may decrease subsequent reactions.50,51 Reactions are typically managed by epinephrine, antihistamines, corticosteroids, and decreased infusion rate. Long-term side effects are also associated with infliximab use. Immunosuppression places patients at risk for sepsis, opportunistic infections, and lymphoma. Drug-induced lupus may occur.
Antibiotics are not indicated in acute colitis unless underlying infectious colitis is suspected.
Emergency Department Care—Acute Presentation and Management
Severe acute flares are characterized by more than six bloody stools per day, abdominal pain/distension, fever, tachycardia, and inflammatory changes on laboratory values. The inflammatory response from IBD alone may cause a low-grade fever. Toxic megacolon occurs in approximately 5% of adults with IBD but is less common in children. It is a surgical emergency and associated with an increased risk of perforation, sepsis, electrolyte abnormalities, and hemorrhage. Escalating abdominal pain should alert the clinician to the possibility of toxic megacolon and/or perforation. Symptoms may be masked by corticosteroid use. Bowel perforation is more common in UC than CD and typically associated with toxic megacolon. Acute flares require intravenous fluids and typically intravenous steroids. High-dose steroids (methylprednisolone 1 mg/kg/dose q12 for maximum of 30 mg q12) are most commonly used. Laboratory evaluation should include a complete blood count, electrolytes, C-reactive protein, erythrocyte sedimentation rate, albumin, and liver enzymes and function tests. Blood transfusion may be necessary. In severe colitis, blood and stool cultures including evaluation for C. difficile should be obtained to evaluate for concurrent infection. Five to 25% of patients with UC flare have underlying C. difficile and have a worse outcome.17,46,52 Steroid-resistant disease often requires endoscopy evaluation for CMV infection.53 Suspected infectious colitis, toxic megacolon, or perforation are indications for antibiotics. Routine use of opioids is discouraged because of its increased association with toxic megacolon. This presents a treatment dilemma for pain control. There have been reports of benzodiazepine use to manage tenesmus and low-dose ketamine use for pain control.46 Severe disease or complications may require surgical intervention. Adult trials show that in patients with a severe UC flare, 67% response to IV steroids, 29% required colectomies.54
Surgical intervention is reserved for patients with IBD that have not responded to medical therapy or for certain complications such as an abscess, fistula, or stricture. Surgery is also indicated in acute emergencies such as uncontrolled hemorrhage, bowel perforation, and toxic megacolon. Overall, three-fourths patients with IBD will require surgery for complications. Disease in UC is limited to the colon and a colectomy essentially “cures” the disease. Medical management is first maximized (see Surgery Complications). Risk factors for surgery in children include female gender, poor growth at presentation of disease, signs of malnutrition such as hypoalbuminemia, or those with complications such as abscess, fistula, or stricture formation.55 Endoscopic balloon dilation and steroid injection treatment is used as alternative to surgery. Success rates are higher in patients with simple strictures. Perforation is a complication.56
Overall, the incidence of surgical complications in children is between 13% and 55%.2,57–59 Early postsurgical complications include wound infections, dehiscence, pouch ischemia, and anastomotic leakage. Late complications include pouchitis and fistula formation. Intestinal obstruction may occur initially or years after initial surgery. Some patients (10% after operation) have CD diagnosed after the surgery (usually 5–10 years later).
Ileal pouchitis occurs in 7% to 44%. The majority of patients have a single initial attack after surgery. Symptoms include acute onset of diarrhea (may be >30 episodes per day), urgency, incontinence, rectal bleeding, abdominal cramping, and malaise in a patient after restorative proctocolectomy. Endoscopic examination of the pouch demonstrates mucosal hemorrhage, ulcers, and contact bleeding in the ileal mucosa. EIM of colitis often relapse with pouchitis. Treatment includes antibiotics (typically metronidazole) and corticosteroid enemas. Most cases resolve within 72 hours of treatment. Chronic pouchitis may less commonly occur. The etiology of pouchitis is unknown but theories include ischemia, bacterial overgrowth, immunologic abnormality, or recurrent disease in colonic metaplasia.
Long-Term Complications of IBD
In IBD, overall, there is a small increase in mortality of 0.5% overage matched controls.60 There does not appear to be a gender difference in the clinical outcome of IBD.61
The risk of colorectal cancer in patients with IBD overall has decreased in the past 30 years. Those with IBD onset prior to age 25 years, particularly males, still have increased risk.62 Children who develop UC before age 14 years have a colon cancer incidence rate of 5% at 20 years and 40% at 35 years. The risk of dying from colon cancer is 8% after 10 years from diagnosis.3 Cancer has been reported in children younger than 18 years of age. In addition, CD has a 40-fold increased risk of small bowel cancer.63 A diagnosis of UC and primary sclerosing cholangitis portends a high risk for colorectal cancer.64,65
IBD medication use has varying association with mortality.66 Immunocompromise from various agents increases risk of disseminated and opportunistic infections. Steroid use is associated with masking signs and symptoms of toxic megacolon and perforation which can increase both morbidity and mortality. Use of azathioprine and mercaptopurine increase the risk for nonmelanoma skin cancers67 and immunomodulators increase risk of lymphoma. A rare fatal form of lymphoma, hepatosplenic T-cell lymphoma is associated with 6-MP and AZA, but not with infliximab monotherapy.14
IBD is a well-established risk factor for recurrent venous thromboembolism. The pathophysiology behind this risk is not completely understood. It is likely multifactorial and frequently related to acquired risk factors such as surgery, immobilization, dehydration, and indwelling central venous lines.68
Pediatric IBD has unique manifestations with respect to the growth and development of a child. Consequences of IBD include malnutrition, failure to thrive, delayed puberty, and short stature. Proinflammatory cytokines exert deleterious effects on growth in IBD via various mechanisms either systemically or at the level of the growth plate.69
The psychosocial effects of a developing child with IBD can be significant and complex. Fear of strangers, separation anxiety, fear of loss of love and approval, fear of loss of control of bodily functions, and fear of pain and humiliation are common in children. Not surprisingly, up to 25% of adolescents with IBD are depressed.70 Social support, education, and skills to cope with stress are important in caring for a child with IBD.71
Premature subclinical atherosclerosis may occur in pediatric IBD. There are early reports that microvascular endothelial dysfunction occurs similar to lupus and rheumatoid arthritis (Table 75-2, Fig. 75-2).72
TABLE 75-2Differential Diagnosis of IBD |Favorite Table|Download (.pdf) TABLE 75-2 Differential Diagnosis of IBD
Peptic ulcer disease
Urinary tract infection
Irritable bowel syndrome
Algorithm for emergency department management.