Pediatric Inflammatory Bowel Disease In The Emergency Department: Managing Flares And Long-Term Complications
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Pediatric Inflammatory Bowel Disease In The Emergency Department: Managing Flares And Long-Term Complications

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Table of Contents
 
Table of Contents
  1. Abstract
  2. Case Presentations
  3. Introduction For Pediactric Inflammatory Bowel Disease
  4. Critical Appraisal Of The Literature For Inflammatory Bowel Disease
  5. Etiology And PathophysiologyFor Pediatric Inflammatory Bowel Disease
    1. Genetic Susceptibility
    2. Ethnicity
    3. Infection
    4. Environment
  6. Differential Diagnosis For Inflammatory Bowel Disease
  7. Prehospital Care For Inflammatory Bowel Disease
  8. Emergency Department Evaluation For Pediatric Inflammatory Bowel Disease
    1. Clinical Presentation
    2. Indeterminate Colitis
    3. Ulcerative Colitis
    4. Crohn Disease
    5. Extraintestinal Symptoms
  9. Diagnostic Studies For Pediatric Inflammatory Bowel Disease
    1. Laboratory Testing
    2. Diagnostic Imaging
  10. Outpatient Imaging For Pediatric Inflammatory Bowel Disease
    1. Endoscopy
      1. Ulcerative Colitis
    2. Upper Gastrointestinal Contrast Study
    3. Video-Capsule Endoscopy
  11. Treatment For Inflammatory Bowel Disease
    1. Management Overview
    2. Management Of Inflammatory Bowel Disease
      1. Acute Flares
      2. Bowel Perforation And Toxic Megacolon
      3. Bowel Obstruction
    3. Other Management Options
      1. Medications
      2. Antibiotics
      3. Topical Therapy
      4. Corticosteroids
      5. Aminosalicylates
      6. Immunomodulators
      7. Biologic/Anti–Tumor Necrosis Factor Agents
    4. Surgery
  12. Long-Term Complications Of Inflammatory Bowel Disease
    1. Malignancy
    2. Thromboembolic Risks
    3. Medication Complications
    4. Growth
    5. Psychosocial Effects
    6. Other Long-Term Complications
  13. Time- And Cost-Effective Strategies For Inflammatory Bowel Disease
  14. <a href="#Risk-Management-Pitfalls>Risk Management Pitfalls For Inflammatory Bowel Disease
  15. Disposition And Summary For Inflammatory Bowel Disease
  16. Case Conclusions
  17. Tables and Figures
    1. Table 1. Differential Diagnosis For Inflammatory Bowel Disease
    2. Table 2. Extraintestinal Symptoms Of Inflammatory Bowel Disease
    3. Table 3. Medications And Side Effects Of Treatment For Inflammatory Bowel Disease
    4. Figure 1. Abdominal X-ray Showing Toxic Megacolon
  18. References For Inflammatory Bowel Disease

Abstract

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.

Case Presentations

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...

Introduction For Pediactric Inflammatory Bowel Disease

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.

Critical Appraisal Of The Literature For Imflammatory Bowel Disease

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.

Risk Management Pitfalls For Inflammatory Bowel Disease

  1. “You can’t diagnose IBD when patients don’t have any GI symptoms.” Although IBD typically manifests with abdominal pain/distention and diarrhea that is often bloody, it can also present with only extraintestinal manifestations. These are particularly prominent in the pediatric population (especially arthralgias, delayed puberty, and delayed growth).
  2. “I am worried about an intra-abdominal abscess, but I decided not to order CT of the abdomen in this pediatric patient because the radiation risk is too high.” There are various imaging modalities that could aid in the diagnosis of IBD or in the detection of IBD-related complications. Such imaging modalities include x-ray, CT, MRI, ultrasound, and endoscopy. The choice of imaging modality largely depends on the patient's clinical presentation, consideration of risks versus benefits, and availability of the chosen modality. If the patient appears toxic and the clinical examination is concerning for possible bowel perforation and/or toxic megacolon, then abdominal CT may provide the necessary information in the shortest period of time. Computed tomography with oral contrast can evaluate the bowel wall and lumen, and it can identify perforation, obstruction, and abscesses. If using oral contrast and there is a concern for perforation, use a water soluble contrast medium.
  3. “If all laboratory testing results are normal (including inflammatory markers), then this patient does not have IBD.” Normal laboratory test results do not exclude the diagnosis of IBD, but laboratory testing should be done when IBD is suspected. Common abnormalities seen in laboratory testing include anemia (usually normocytic or microcytic), thrombocytosis, elevated ESR and CRP, mild elevation of AST and ALT, and hypoalbuminemia. In addition, studies have shown that red blood cell distribution width is markedly increased in active IBD and may be useful in monitoring disease progression.87
  4. “I saw a pediatric patient with painful oral ulcers. She has a history of IBD and is taking multiple immunosuppressants. She appeared well otherwise, so I sent her home with ibuprofen only.” In addition to pain medication, such oral lesions may be treated with topical prednisolone syrup (5 mg/5 mL) or dexamethasone (0.5 mg/5 mL), either applied directly to the lesions or by the swish-and-spit method twice daily. If the lesions are localized to the lips, triamcinolone 0.1% may be used 2 to 4 times per day.
  5. “Patients with IBD always need antibiotics when they present with abdominal pain.” Antibiotics are indicated when there is suspicion of infectious colitis, toxic megacolon, or intestinal perforations. Blood and stool cultures should be sent prior to initiating antibiotics.
  6. “I can’t tell if the patient is having an acute flare or having complications secondary to Crohn disease, so I was unsure of the best way to treat the patient.” It is often difficult to distinguish between acute flares or IBD complications solely based on physical examination and laboratory testing, as findings can be very similar in both circumstances. Imaging can aid in differentiating these 2 etiologies; however, it may not be needed because management is similar for both conditions. Patients often require intravenous steroids, intravenous fluid hydration, and broadspectrum intravenous antibiotics (if underlying infection is suspected).
  7. “This parent is asking for a flu shot for her child, but the boy is taking immunomodulators for IBD.” Patients taking immunomodulators have suppressed immune systems and are more prone to developing infections. Sepsis is one of the leading causes of mortality in patients with IBD. Thus, it is important to vaccinate patients. Immunization with inactivated vaccines (particularly influenza, pneumococcus, and meningococcus) should be maintained and updated. Depending on the level of immunosupression, live attenuated vaccines (Measles, Mumps, and Rubella; Varicella; intranasal influenza) may be contraindicated. Decisions regarding whether specific vaccinations are appropriate for patients on immunomodulatory medications for IBD should be made by the prescribing clinician. Risk Management Pitfalls For Inflammatory Bowel Disease (Continued from page 12)
  8. “X-ray findings of toxic megacolon are the same in adults as in children.” Findings of toxic megacolon in both adults and children include colonic dilatation with an abnormal mucosal contour, which is typically most pronounced in the transverse colon. Acute dilatation of the transverse colon to > 5 to 6 cm with loss of haustral folds during a severe exacerbation of colitis is diagnostic of toxic megacolon in older children and adults. In children aged < 10 years, transverse colonic diameter> 4 cm is suggestive of toxic megacolon.
  9. “This patient is having a flare of IBD, but I wasn't sure if all outpatient medications should be continued.” During a flare, patients typically require high dose intravenous steroids. The decision to continue other classes of outpatient medication (such as biologic agents or immunomodulators) should be made in conjunction with gastrointestinal specialists. It is important to note that 5-ASA agents (sulfasalazine, mesalamine, olsalazine) are ineffective during an acute exacerbation and should be discontinued to prevent worsening of colitis symptoms.
  10. “This patient looks like he is having an acute flare, but I was concerned about further immunosuppression, so no steroid was given.” While many patients with IBD are relatively immunocompromised due to immunomodulator therapy, intravenous steroids are indicated as one of the essential treatments during an acute flare. High-dose steroids (methylprednisolone 1 mg/kg/dose every 12 hours to a maximum of 30 mg every 12 hours) are most commonly used.

Tables and Figures

Table 1. Differential Diagnosis For Inflammatory Bowel Disease

References For Inflammatory Bowel Disease

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.

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Publication Information
Authors

Margaret Huang, MD; Emily Rose, MD, FAAP, FAAEM, FACEP

Publication Date

July 1, 2014

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