Inflammatory bowel disease includes both Crohn disease and ulcerative colitis. Pediatric-onset inflammatory bowel disease differs from adult inflammatory bowel disease in disease type, location, progression, and sex preponderance, and 20% to 30% of inflammatory bowel disease is diagnosed in childhood. Children are more likely than adults to present with extraintestinal manifestations of inflammatory bowel disease (with aphthous ulcers, joint involvement, and growth delay being the most common). Inflammatory bowel disease flares typically require treatment with intravenous steroids and inpatient admission. Acute emergencies include toxic megacolon, intestinal obstruction, and perforation. The use of steroids may obscure diagnosis of an underlying abdominal emergency by masking signs and symptoms. The emergency clinician must be cognizant of such complications and diagnostic challenges when evaluating inflammatory bowel disease.
A previously healthy 12-year-old boy is brought to the ED by his mother for evaluation of fatigue and skin rash. She has noticed “red bumps” on his legs for the past few weeks, but she reports no other associated symptoms. The patient says he has been feeling tired lately and can’t seem to keep up with his friends on the playground. He states that he sometimes has abdominal cramping after eating but denies diarrhea. He does not recall being bitten by insects on his legs and says those red bumps “hurt just a little.” On physical examination, you note a thin and mildly pale-appearing boy, who is small for his age. He has normal affect and behavior. Vital signs are: oral temperature, 36.3°C; heart rate, 89 beats/min; respiratory rate, 18 breaths/min; blood pressure, 103/65 mm Hg; and oxygen saturation, 99% on room air. Several well-circumscribed, minimally tender, nonweeping and nonblanching erythematous nodules are noted on the bilateral anterior tibia. There is no surrounding fluctuance, crepitus, or bullae. The only significant finding on review of his past medical history is his growth chart. You are concerned that the nodules on his legs may be indicative of systemic disease, and you consider your diagnostic options...
You are then called to evaluate a 17-year-old adolescent girl who is brought in by ambulance for abdominal pain and fever. She has a history of Crohn disease and is currently taking sulfasalazine and prednisone. She reports diffuse abdominal cramping for 2 days, fever and chills, vomiting, and bloody diarrhea. She has been unable to tolerate any oral intake since 3:00 AM that day. She denies any recent antibiotic use, history of travel, or medication changes, although she has not been consistently compliant with her medications. Vital signs are: temperature, 39.1°C; heart rate, 132 beats/min; respiratory rate, 23 breaths/min; blood pressure, 94/57 mm Hg; and oxygen saturation, 95% on room air. She appears pale and diaphoretic, and her mucous membranes are dry. Physical examination is notable for diffuse abdominal tenderness with guarding, tympany on percussion, and decreased bowel sounds. No rebound tenderness is elicited. Capillary refill is 3 seconds. The remainder of the examination is within normal limits. You ask your nursing staff for 2 large-bore intravenous lines for fluid resuscitation. You are concerned about an acute flare and you consider appropriate treatment options...
Inflammatory bowel disease (IBD) includes both Crohn disease and ulcerative colitis. Approximately 2 million people worldwide are afflicted with IBD, and 20% to 30% of all patients with IBD are diagnosed during childhood.1,2
Childhood incidence of ulcerative colitis is estimated at 0.5-4.3/100,000 and Crohn disease at 0.2-8.5/100,000.3 The peak incidence of initial presentation for IBD occurs between the ages of 15 and 25 years, and approximately 20% of patients with ulcerative colitis and 25% to 30% of patients with Crohn disease present before the age of 20 years.4 Crohn disease and ulcerative colitis occur equally in the first 8 years of life, but Crohn disease is more common in older children.5 The incidence of ulcerative colitis has remained relatively stable, whereas the incidence of Crohn disease has increased.6-9 Utilization of colonoscopy in developed countries may have led to greater differentiation of Crohn disease from ulcerative colitis and relatively more diagnoses of Crohn disease. The number of emergency department (ED) visits per year is unknown; however, the public health burden of disease is significant in patients with IBD, due to utilization of outpatient resources, ED visits, and inpatient care.10
Certain clinical presentations may be similar to adults, but pediatric-onset IBD differs in disease type, location, progression, and sex preponderance. While there is no difference in sex predominance in adult IBD, studies have shown that Crohn disease is more prevalent in pediatric male populations.11 Importantly, a subset of children with IBD may present only with extraintestinal manifestations (particularly joint involvement, apthous ulcers, poor weight gain, growth failure, and delayed puberty). Of all extraintestinal manifestations, joint involvement is the most common in children with IBD.12
Both medical and surgical interventions have the goal of inducing and maintaining remission in IBD. However, these treatments are not without side effects, the most significant of which are immunosuppression, infusion reaction, and postsurgical complications.
Potential complications of IBD include intestinal obstruction and perforation, sepsis, and toxic megacolon. Long-term disease burden includes an increased risk of developing malignancies and recurrent thromboembolic events.
A literature search was performed using PubMed, Ovid MEDLINE®, Google Scholar, and the Cochrane Database of Systematic Reviews with the search terms pediatric inflammatory bowel disease, epidemiology, emergency, treatment, complications, and malignancy. Over 200 articles met the selection criteria. Of those, 109 articles with full texts were reviewed, and 87 are cited in this review. Clinical cohort and systematic review studies were also analyzed. Few prospective trials have been conducted for treatment in the pediatric population. Further research is needed in the management of the child with IBD.
Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.
To help the reader judge the strength of each reference, pertinent information about the study will be included in bold type following the reference, where available. In addition, the most informative references cited in this paper, as determined by the authors, will be noted by an asterisk (*) next to the number of the reference.
Margaret Huang, MD; Emily Rose, MD, FAAP, FAAEM, FACEP
July 1, 2014