DEPARTMENT
Case Study—Acute and Specialty Care
Crohn’s Disease Masquerading as an Acute Abdomen Jessica Judge, PA-C, Beverly P. Giordano, MS, RN, CPNP, PMHS, & Joan English, PA-C
KEY WORDS Crohn’s disease, abdominal pain
Section Editors Karin Reuter-Rice, PhD, CPNP-AC, FCCM Corresponding Editor Duke University Durham, North Carolina Terea Giannetta, DNP, RN, CPNP California State University Children’s Hospital Central California Fresno, California Maureen A. Madden, MSN, RN, CPNP-AC, CCRN, FCCM Rutgers Robert Wood Johnson Medical School New Brunswick, New Jersey Bristol Myers Squibb Children’s Hospital New Brunswick, New Jersey Jessica Judge, Physician Assistant, Crestview Health Center, Crestview, FL. At the time this article was prepared, she was Physician Assistant Student, College of Medicine, University of Florida, Gainesville, FL. Beverly P. Giordano, Pediatric Nurse Practitioner, Division of General Pediatrics, University of Florida, Gainesville, FL. Joan English, Physician Assistant, Division of Pediatric Gastroenterology, University of Florida, Gainesville, FL. Conflicts of interest: None to report. Correspondence: Beverly P. Giordano, MS, RN, CPNP, PMHS, 1701 SW 16th Ave, Gainesville, FL 32608; e-mail: bgiordano@ peds.ufl.edu. J Pediatr Health Care. (2014) -, ---. 0891-5245/$36.00 Copyright Q 2014 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pedhc.2014.03.003
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CASE PRESENTATION A 14-year-old African American boy presented to a primary care clinic with diffuse abdominal pain. He was in no acute distress and was conversational, but he was a poor historian. He reported a 2-week history of abdominal cramping, particularly at night. The pain was described as ‘‘all over,’’ increasing in severity over time, not associated with particular foods, and not alleviated with bowel movements. During the first week, the pain was intermittent and mild (3 to 4 out of 10), but it became constant and worsened to 6 to 7 out of 10 in severity during the 4 days before he presented. He denied having fever, constipation, hematochezia, or change in urine color. He denied recent travel. Although the patient denied having any life stressors, the pain had caused him to miss several days of school and basketball practice. MEDICAL HISTORY The patient’s growth and weight gain had been normal since birth; however, he had lost 10 lb in the previous 3 months. His medical history was positive for eczema, recurrent otitis externa, and joint pain thought to be sports related. His immunizations were up to date. FAMILY/SOCIAL/DEVELOPMENTAL HISTORY The patient’s mother had been treated for Grave’s disease, cholelithiasis, and tuberculosis. The family history was negative for other gastrointestinal disease or autoimmune processes. The patient lived with both parents and attended the ninth grade at a local high school. He participated in several sports. He was -/- 2014
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sexually active with females and reported occasional marijuana use. REVIEW OF SYSTEMS In addition to the abdominal pain, the patient reported ‘‘occasional’’ diarrhea. He also admitted to nausea, vomiting, and a recent decrease in appetite. Results of a pertinent review of other systems, except as noted, were otherwise negative. PHYSICAL EXAMINATION The patient was quiet but pleasant, ambulatory, and conversant. He did not appear to be in distress and had age-appropriate vital signs. His height was 182.9 cm (72 inches, 95th percentile) and his weight was 59.2 kg (130.5 lb, 65th percentile), with a body mass index of 17.70 kg/m2. His skin was warm and dry with no lesions. Results of his respiratory and cardiac examination were unremarkable. His abdomen was soft and nondistended with hypoactive bowel sounds present throughout. No organomegaly was noted, and the abdomen was tympanic to percussion. The patient had mild periumbilical tenderness with deep palpation, but no rebound tenderness. Obturator, psoas, and Murphy’s signs were negative. A rectal examination was not performed. The patient had no apparent neurologic deficits. DIAGNOSTIC TESTING AND CLINICAL COURSE At the initial clinic visit, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), complete blood cell count (CBC), complete metabolic profile (CMP), and stool Giardia and Helicobacter pylori antigen were ordered. Five days elapsed before test results were obtained. The results were significant for a CRP of 45.4 mg/L (normal, < 4.9 mg/L) and an ESR of 25 (normal, 0 to 20 mm/h). Results of the CBC and CMP were normal, and the stool tests were negative. The day after the blood tests were obtained, the patient presented to the clinic with worsening abdominal pain, which had localized to the right lower quadrant (RLQ). He had rebound tenderness in both lower quadrants. He was sent from the clinic to the emergency department (ED), and the pediatric surgery service was consulted because of a concern for possible acute appendicitis. The CRP on that day was further elevated (79.6 mg/L). A pelvic computed tomography (CT) scan demonstrated a dilated appendix measuring 12 mm with surrounding edema and inflammatory stranding in the RLQ and a large amount of free fluid in the pelvis. The radiologist’s impression was of appendicitis with no evidence of perforation or abscess formation despite the presence of fluid in the pelvis. The pediatric surgeon thought that a perforation was present and admitted the patient for a trial of nonoperative management with 1 week of ceftriaxone and metronidazole. This approach was consistent with guidelines for 2
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nonoperative management (i.e., patients with small appendiceal abscesses may be treated with intravenous [IV] antibiotics and then undergo an interval appendectomy 4 to 6 weeks later; Blakely et al., 2011). The patient improved clinically and was discharged on a regular diet. The day after discharge, the patient presented to the ED with recurrent RLQ pain and multiple episodes of nonbilious, nonbloody emesis. A CT of the abdomen and pelvis showed residual phlegmon (i.e., spreading diffuse inflammatory process with formation of suppurative/purulent exudate or pus) without a drainable abscess. His white blood cell count and CRP were within normal limits. Findings of an ultrasound of the right upper quadrant were unremarkable. Abdominal CT demonstrated cholelithiasis (later disproved) and increased distention of the distal small bowel with focal edema and thickening of the distal terminal ileum. The patient had a moderate volume of intra-abdominal free fluid but no intra-abdominal free air. He was admitted for 7 days of treatment with IV piperacillin/tazobactam. He improved clinically and had a CRP of 5.8 mg/L on the day of discharge. Twenty-four hours after being discharged, he presented to the ED with recurrent abdominal pain and vomiting. He was readmitted, and the pediatric gastroenterology service was consulted. An abdominal and pelvic CT scan demonstrated increasing inflammation in the terminal ileum. He underwent a colonoscopy and an upper endoscopy to evaluate for possible Crohn’s disease, assess the disease location, and possibly obtain tissue for pathologic examination. The bowel preparation procedure was unsatisfactory with poor cleanout. The findings of the colonoscopy and upper endoscopy were reported as normal, although the gastroenterologist who performed the procedure noted the presence of an abnormal perianal skin tag. Because the endoscopy procedures did not elucidate a diagnosis, the patient underwent a planned exploratory laparoscopy. The appendix was normal. However, the distal ileum was dilated and thickened, which was consistent with chronic obstruction and possible inflammatory bowel disease (IBD). The ileocecal valve appeared to contain a mass, which was concerning for IBD or malignancy. At this point, the procedure was converted to a laparotomy with resection of the distal ileum and half of the right colon. The resected terminal ileum and right colon demonstrated severe active chronic ileitis with fissures, ulceration, inflammation, and poorly formed granulomas, consistent with Crohn’s disease. The appendix had fibrous obliteration of the tip. Seven reactive lymph nodes were found with scattered granulomas. Gram and acid-fast bacteria stains were negative. The small bowel resection margin demonstrated mild active chronic ileitis with villitis, submucosal edema, and serositis, consistent with severe active IBD. The patient Journal of Pediatric Health Care
was discharged on a regular diet after this 14-day admission and was scheduled to follow up with the pediatric gastroenterology clinic. Two months after surgery, after having missed three appointments, the family finally met with the pediatric gastroenterologist. At that visit, the physician provided extensive education on IBD and the importance of medication compliance, answered the family’s questions, and measured the patient’s thiopurine methyltransferase (TPMT) activity, because the plan was to prescribe 6-mercaptopurine (6-MP; low TPMT activity increases the risk of drug-induced bone marrow suppression as a result of the accumulation of drug metabolites). The patient’s pretreatment TPMT activity was normal, and thus he was cleared to begin 6-MP at 1.5 mg/kg (100 mg by mouth daily), which is consistent with dosing guidelines (Ghazi, 2013). Follow-up TPMT monitoring was ordered for 2 weeks after beginning 6-MP therapy and again at 4 weeks, and he was to have 6-MP metabolite levels measured at 6 weeks after initiating therapy to monitor compliance. He was scheduled to return to the pediatric gastroenterology clinic in 6 weeks. The patient failed to undergo the follow-up TPMT monitoring. When the pediatric gastroenterologist contacted the family, the patient’s father reported that his son was not taking the prescribed 6-MP. The father stated, ‘‘I’m not sure why he needs this medication. I think his positive attitude and mind can control the disease.’’ The gastroenterologist reiterated that the bowel
inflammation would return without treatment, which would result in disease progression. One month later, the patient was seen again in the pediatric gastroenterology clinic. He and his parents expressed reluctance to accept the diagnosis of Crohn’s disease and were uncomfortable with making a long-term commitment to medication therapy. They requested a second opinion before beginning 6-MP therapy. However, when they returned 2 weeks later to meet with a different gastroenterologist, the patient reported that he was taking 6-MP, and he had gained 0.8 kg. Laboratory tests obtained at that visit included CRP, folate, vitamin B12, CMP, CBC, ESR, and vitamin D, 25-OH. All the laboratory tests were normal except for a low vitamin D, 25-OH level (18.7 ng/mL; normal, > 29 ng/mL). An over-the-counter vitamin D supplement was prescribed, and he was directed to continue taking the 6-MP. At an IBD support group meeting 2 months later, the patient reported that he had discontinued all medications. Eleven months after surgery, he is in a surgically induced clinical and biochemical remission. Although the pediatric gastroenterologist recommends that the patient take medication to prevent inflammation in other parts of his bowel, the patient’s family prefers to continue to monitor him while he remains medication-free. The patient’s most recent laboratory tests revealed normal serum inflammatory markers and no signs of anemia. He continues to gain weight and thrive.
CASE STUDY QUESTIONS 1. What are common presenting symptoms of Crohn’s disease? 2. How is Crohn’s disease managed? 3. What are sequelae of untreated Crohn’s disease? CASE STUDY ANSWERS Presenting Symptoms 1. What are common presenting symptoms of Crohn’s disease? Crohn’s disease is characterized by chronic intestinal inflammation in the absence of a recognized cause. Immune system malfunction, ethnicity (an Crohn’s disease is increased incidence in being discovered Ashkenazi Jews and a lower incidence in Afwith increasing rican Americans), famfrequency in ily history of Crohn’s children of all ages. disease, and environment (with a higher risk for urban dwellers) are risk factors (Sauer & Kugathasan, 2009). Crohn’s disease is being discovered with increasing frequency in children of all ages. Recent
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studies indicate that 20% to 30% of all patients with Crohn’s disease present before age 20 years (Grossman & Mamula, 2012). Disease location and severity determine the clinical presentation. The terminal ileum is involved in 50% to 70% of affected children (Grossman & Mamula, 2012). Children commonly present with a several-week history of abdominal pain, diarrhea, and weight loss (Day, 2012). The patient in this case study underreported his symptoms and withheld vital information, which delayed the diagnosis of Crohn’s disease. Crohn’s disease affecting the upper gastrointestinal tract typically causes nausea, vomiting, and abdominal pain, whereas disease in the small intestine causes signs of malabsorption (e.g., diarrhea, abdominal pain, growth deceleration, anorexia, and weight loss). Colonic Crohn’s disease may be difficult to distinguish
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from ulcerative colitis, because the symptoms (i.e., bloody diarrhea, cramping abdominal pain, and urgency to defecate) are similar (Grossman & Mamula, 2012). Perianal Crohn’s disease may cause skin tags, fissures, abscesses, fistulae, painful defecation, perirectal pain, bright red rectal bleeding, perirectal erythema, and rectal discharge. Abdominal examination findings can vary from normal to those seen with an acute abdomen. Diffuse abdominal pain often is present, along with a discrete mass found on palpation. Typically, the mass represents a palpable thickened loop of bowel (Day, 2012), as was the case in this patient. Extraintestinal manifestations are present in 25% to 35% of patients with Crohn’s disease (Grossman & Mamula, 2012). Dermatologic manifestations include erythema nodosum on the anterior aspects of the lower extremities and pyoderma gangrenosum on the dorsal surfaces of the feet and legs, ears, around stomas, and on the face. Anemic patients may have pallor. Peripheral asymmetric, polyarticular arthritis develops in 15% to 20% of patients and worsens with disease exacerbations. Approximately 10% of patients with Crohn’s disease experience ankylosing spondylitis, which presents as morning stiffness, diffuse low-back pain, and buttock pain (Friedman & Blumberg, 2011). Long-standing Crohn’s disease affects linear growth, and thus decreased growth velocity can be a sign of Crohn’s disease. The patient in this case study had achieved his final adult height before his diagnosis. Management 2. How is Crohn’s disease managed? The treatment goals are to reduce or eliminate symptoms; optimize nutritional status; promote normal growth and development; prevent complications; minimize psychological effects of chronic illness; achieve optimal clinical, laboratory, and histologic control of the inflammatory disease with the least adverse medication effects; and permit patients to function as normally as possible (e.g., school participation; Grossman & Mamula, 2012). Medication, lifestyle and dietary changes, stress reduction, and moderate activity and exercise are the mainstays of treatment. Treatment strategies depend on the severity and location of the disease. Monitoring When patients with Crohn’s disease have symptom exacerbation episodes, laboratory studies (e.g., CBC, CRP, ESR, fecal calprotectin, stool culture, stool Giardia, and testing for Clostridium difficile) help determine the presence of infections. The immunocompromised state of patients who take biologic therapies or immunomodulators increases the risk for invasive infections.
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Medications Corticosteroids, immunomodulators, and antitumor necrosis factor alpha (TNFa) agents are the mainstays of Crohn’s disease management, but they have inherent risks as well as benefits. Corticosteroids decrease the disease signs and symptoms but do not heal mucosa, and they impair growth and can lead to corticosteroid dependency. Immunomodulators (e.g., azathioprine and 6-MP) are effective maintenance therapies and offer corticosteroid-sparing effects. TNFa agents (e.g., infliximab, adalimumab, certolizumab, and natalizumab) bind to tumor-necrosing factor to block inflammatory response. They are one kind of biologic therapy approved as maintenance therapy for patients with moderate to severe Crohn’s disease who do not respond to standard therapies and for treatment of open, draining fistulas (National Digestive Diseases Information Clearinghouse [NDDIC], 2011). Traditionally, corticosteroids and anti-inflammatory medications containing mesalamine (sulfasalazine, 5aminosalicylic acid) were the medications used in the initial treatment of Crohn’s disease. However, they frequently do not control the disease adequately. Adverse effects include nausea, vomiting, heartburn, diarrhea, temporary hair loss, and headaches. Immunomodulators are standard therapy after initial treatment with corticosteroids. Regular laboratory tests (e.g., 6-MP/azathioprine metabolite testing) are required to monitor medication compliance. Women must take precautions to avoid pregnancy, because these medications are rated category D for pregnancy. A recent study compared the effectiveness of early treatment with an anti-TNFa agent versus an immunomodulator in attaining clinical remission and facilitating growth of pediatric patients. The investigators concluded that in newly diagnosed children with comparably severe Crohn’s disease, early (# 3 months after diagnosis) monotherapy with an anti-TNFa agent produced better overall clinical and growth outcomes at 1 year than did early monotherapy with an immunomodulator (Walters & Kim, 2014). A retrospective analysis of long-term patient outcomes demonstrated efficacy and safety of anti-TNFa treatment for pediatric IBD (Assa et al., 2013). Adjunctive use of immunomodulators and anti-TNFa agents increases TNFa drug levels, reduces the risk of antidrug antibodies, and improves response (Absah & Stephens, 2013). These results are tempered by reports of the development of hepatosplenic T-cell lymphoma in younger patients while taking thiopurine immunomodulators with and without concomitant anti-TNFa medications (Absah & Stephens, 2013; Hyams, 2009). Information available at www.ccfa.org/assets/pdfs/biologic102011.pdf provides a brief explanation of the biologic source of TNF blockers and their role in targeting the inflammation process.
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Antibiotics (e.g., metronidazole and ciprofloxacin) are used for patients with colonic or perianal involvement and also to treat abscesses accompanying Crohn’s disease. Rifaximin and oral vancomycin are nonabsorbable antibiotics that are effective in treating C. difficile infections, which can be difficult to manage in patients with Crohn’s disease. Oral iron supplements or IV iron sucrose may be needed to treat anemia caused by blood loss and poor intestinal absorption. Psyllium powder or methylcellulose may relieve diarrhea by adding bulk to the stool. Loperamide can relieve severe diarrhea. Laxatives, along with low-residue diets, may be indicated if inflammation narrows the intestinal lumen, leading to constipation. Acetaminophen is usually preferred for pain relief, because aspirin and nonsteroidal antiinflammatory medications may worsen symptoms. Vitamin B12 injections may be required if terminal ileum inflammation or surgical removal interferes with vitamin absorption. Calcium and vitamin D supplements may be prescribed to counteract the increased risk for osteoporosis associated with corticosteroid therapy. Other unproven but currently hotly debated therapies for Crohn’s disease include fecal transplant (i.e., adding to the microbiota present in bowel), helminth therapy, and the use of certain probiotics for management of pouchitis. Lifestyle changes Dietary adjustments, proper hydration, and smoking cessation are essential. Regular exercise, a healthy diet, and sufficient sleep can counteract fatigue. Nutritional management may include enteral or parental nutrition to improve overall nutrition and allow bowel rest. Enteral nutrition can induce remission, but relapse is common after primary nutritional therapy is discontinued, mandating concomitant pharmacologic therapy (Grossman & Mamula, 2012). Foods with high fiber content or gluten and dairy products can trigger symptoms. Patients with small intestine disease may have diarrhea with rapid intestinal transit of fatty foods. Many patients tolerate five or six small meals per day rather than two or three larger ones. Carbonated drinks produce gas, and caffeinated drinks stimulate the intestines and can worsen diarrhea. Primary care providers should ensure that patients who take biologic therapy agents have annual tests for tuberculosis. Sunscreen use must be emphasized at every encounter, and annual influenza vaccines should be administered. Additionally, referrals for annual eye examinations are part of care coordination. When patients are initially diagnosed with Crohn’s disease, it is important to ensure that immunizations are up to date, because no live vaccines can be administered to patients who take biologic agents.
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Surgery Approximately two thirds of patients with Crohn’s disease require surgery at some point in their lives (Lichtenstein, Hanauer, Sandborn, & the Practice Parameters Committee, 2009). Surgical intervention can range from resection of only the diseased portion to proctocolectomy (removal of the rectum and part of or the entire colon). Surgery may be indicated for obstructions, fistulas, and abscesses or if the disease is unresponsive to medication. However, surgery does not cure Crohn’s disease, and postoperative complications are common. After bowel resection, scar tissue can cause strictures, leading to bowel obstruction. Patients who have duodenal obstructions caused by strictures may be managed with strictureplasty or resection of the affected portion of the bowel (Viola et al., 2013). Growth impairment and delayed pubertal development are common in children with Crohn’s disease. Growth rate is a marker of the disease status. Close monitoring of growth Growth impairment velocity and periodic and delayed determination of skeletal maturation are pubertal essential components development are of the treatment plan common in children (Gupta, Lustig, Kohn, & Vittinghoff, 2013). with Crohn’s Coping with Crohn’s disease. disease is challenging. Long-term remission management requires most patients to take one or more oral medications daily in the absence of symptoms. Adverse effects of medication (e.g., nausea, vomiting, headaches, weight gain, facial changes, excessive facial hair, decreased resistance to infection, seizures, increased cancer risk, tendon rupture, and numbness and tingling in the extremities) interfere with treatment adherence (LeLeiko et al., 2013). Periodic imaging studies (e.g., upper GI series with small-bowel follow-through and magnetic resonance enterography) may be indicated to look for small bowel disease, disease activity, structuring, and fistulae. Magnetic resonance enterography has the advantage of no radiation exposure but has limited use because it requires that the patient drink large amounts of a contrast solution. Crohn’s disease outcomes have not improved with advances in technology and medications. Mortality, cancer, disease recurrence, extraintestinal manifestations, recurrent hospitalizations, and surgery continue to complicate the lives of patients with Crohn’s disease. A study of 246 pediatric patients with Crohn’s disease who were followed up at 48 practice sites demonstrated a wide variation in diagnostic and therapeutic interventions and gaps between recommended and actual care (Colletti et al., 2009). The investigators recommended
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use of quality improvement methods to evaluate the effectiveness of treatment guidelines, reduce treatment variation, and improve clinical outcomes of children and adolescents with Crohn’s disease. The diagnosis of Crohn’s disease is a major insult to adolescent self-esteem. The disease symptoms are embarrassing and can lead to social isolation. Diagnostic procedures are invasive, and surgical intervention reduces all-important social and academic activities. Dietary recommendations are inconsistent with the average adolescent’s eating habits. Support groups and organizations (see http://www.ccfa.org, http:// www.crohnsandme.com, and http://www.pedsibd. org/teens) are available to help patients and families cope with Crohn’s disease (D’Auria & Kelly, 2013). Students with Crohn’s disease should have 504 plans (Section 504 of the Rehabilitation Act of 1973) that grant ad lib bathroom access at school (http://www.ccfa.org/ resources/template-section-504-plan.html). Stress can trigger Crohn’s symptoms and must be managed along with nutrition and medication. Sequelae of Untreated Disease 3. What are the sequelae of untreated Crohn’s disease? Abscesses, fistulae, sinus tract formation, strictures, and bowel adhesions may occur because of the transmural nature of Crohn’s disease. Perianal and perirectal fistula formation are the most common, although enterovesical and enterocutaneous fistulas also occur (Irani, Ramsanahie, & Bleday, 2012). Surgical resection of the terminal ileum may cause spillage of bile salts into the colon, which, combined with absence of bile acid sequestrants, can cause chronic diarrhea. If more than 100 cm of ileum is resected, fat cannot be digested, and steatorrhea may develop (Irani et al., 2012). Small bowel overgrowth frequently occurs, especially in patients who have undergone ileocecal valve resection or those with stricture disease. Deficiencies in fatsoluble vitamins (A, D, E, K), iron, folate, and vitamin B12 are common, leading to osteoporosis and anemia. Bile salt reabsorption may be compromised by the disease’s effect on the ileum. The most feared long-term complication of Crohn’s disease is colorectal cancer (CRC). Patients with Crohn’s disease and those with ulcerative colitis have equal risks for developing CRC (Freeman, 2008). Risk factors include duration and extent of disease, severity of inflammation, family history of CRC, and early age at Crohn’s diagnosis. Unlike typical CRC, which presents with adenomatous polyps, the CRC associated with Crohn’s disease typically presents as dysplastic epithelium that may be difficult to recognize. Thus the standard of care is to begin colonoscopy screening 8 to 10 years after Crohn’s disease diagnosis and continue this screening every 2 years with random four-quadrant
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biopsies every 10 cm throughout the colon (U.S. Preventive Services Task Force, 2008). The patient in this case study underwent resection of 10.8 cm of terminal ileum continuous with the cecum and ascending colon. This surgery increases his risk for bile salt–induced diarrhea and vitamin B12 deficiency. His young age at diagnosis and the extent of disease at diagnosis put him in the high-risk .clinicians must category for develinclude Crohn’s oping CRC later in life.
disease in the CONCLUSION differential Crohn’s disease does diagnosis of not typically present as an acute abdomen. patients with However, clinicians abdominal pain. must include Crohn’s disease in the differential diagnosis of patients with abdominal pain. This recommendation is especially relevant to patients with a long history of vague abdominal complaints and those with unexplained weight loss or growth velocity deceleration. We thank Dr. Regino Gonzalez-Peralta, pediatric gastroenterologist at the University of Florida, for content review. REFERENCES Absah, I., & Stephens, M. (2013). Adjunctive treatment to antitumor necrosis factor in pediatric patients with refractory Crohn’s disease. Current Opinion in Pediatrics, 25(5), 624-628. Assa, A., Hartman, C., Weiss, B., Broide, E., Rosenbach, Y., Zevit, N., . Shamir, R. (2013). Long-term outcome of tumor necrosis factor alpha antagonist’s treatment in pediatric Crohn’s disease. Journal of Crohn’s and Colitis, 7(5), 369-376. Blakely, M. L., Williams, R., Dassinger, M. S., Eubanks, J. W., Fischer, P., Huang, E. U., . Langham, M. R. (2011). Early vs interval appendectomy for children with perforated appendicitis. Archives of Surgery, 146(6), 600-665. Colletti, R. B., Baldassano, R. N., Milov, D. E., Margolis, P. A., Bousavaros, A., Crandall, W. V., . Qureshi, M. A. (2009). Variation in care in pediatric Crohn disease. Journal of Pediatric Gastroenterology and Nutrition, 49, 297-303. Day, A. S. (2012). Crohn’s and colitis in children and adolescents. World Journal of Gastroenterology, 41(18), 5862-5869. D’Auria, J. P., & Kelly, M. (2013). Inflammatory bowel disease: Top resources for children, adolescents, and their families. Journal of Pediatric Health Care, 27(2), e25-e28. Freeman, H. J. (2008). Colorectal cancer risk in Crohn’s disease. World Journal of Gastroenterology, 14(12), 1810-1811. Friedman, S., & Blumberg, R. S. (2011). Disorders of the alimentary tract: Inflammatory bowel disease. In D. Longo, A. Fauci, D. Kasper, S. Hause, J. Jameson & J. Loscalzo (Eds.), Harrison’s principles of internal medicine (18th ed., pp. 24772496). New York, NY: McGraw. Ghazi, L. J. (2013). Crohn disease medication. Retrieved from http:// emedicine.medscape.com/article/172940-medication Grossman, A. B., & Mamula, P. (2012). Pediatric Crohn disease. Retrieved from http://emedicine.medscape.com/article/928288
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Gupta, N., Lustig, R. H., Kohn, M. A., & Vittinghoff, E. (2013). Determination of bone age in pediatric patients with Crohn’s disease should become part of routine care. Inflammatory Bowel Disease, 19(1), 61-65. Hyams, J. S. (2009). Risk/benefit strategies must be employed in the management of pediatric Crohn’s disease. Digestive Diseases, 27(3), 291-296. Irani, J. L., Ramsanahie, A., & Bleday, R. (2012). Inflammatory bowel disease: surgical considerations. Crohn Disease. In N. Greenberger, R. Blumberg & R. Burakoff (Eds.), Current diagnosis and treatment—gastroenterology, hepatology & endoscopy (2nd ed.). New York, NY: McGraw Medical. LeLeiko, N. S., Lobato, D., Hagin, S., McQuaid, E., Seifer, R., Kopel, S. J., . Bancroft, B. (2013). Rates and predictors of oral medication adherence in pediatric patients with IBD. Inflammatory Bowel Disease, 19(4), 832-839. Lichtenstein, G. R., Hanauer, S. B., Sandborn, W. J. & the Practice Parameters Committee of The American College of Gastroenterology. (2009). Management of Crohn’s disease in adults. American Journal of Gastroenterology, 104(2), 465-483.
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National Digestive Diseases Information Clearinghouse. (2011). Crohn’s disease. Retrieved from http://digestive.niddk.nih. gov/ddiseases/pubs/crohns Sauer, C. G., & Kugathasan, S. (2009). Pediatric inflammatory bowel disease: Highlighting pediatric differences in IBD. Gastroenterology Clinics of North America, 38(4), 611-628. U.S. Preventive Services Task Force. Screening for colorectal cancer: U.S. Preventive Services Task Force recommendation statement (AHRQ Publication No. 08-05124-EF-3, October 2008). Retrieved from http://www.uspreventiveservicestaskforce. org/uspstf08/colocancer/colors.htm Viola, A. M., D’Arcangelo, G., DiNardo, G., Civitelli, F., Casciani, E., Oliva, S., . Cucchiara, S. (2013). Disease course and efficacy of medical therapy in structuring paediatric Crohn’s disease. Digestive and Liver Disease, 45(6), 464-468. Walters, T. D., Kim, M., Denson, L. A., Griffiths, A. M., Dubinsky, M., Markowitz, J., . Hyams, J. S. (2014). Increased effectiveness of early therapy with anti-tumor necrosis factor-a vs an immunomodulator in children with Crohn’s disease. Gastroenterology, 146(2), 383-391.
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Case Study—Acute and Specialty Care
Crohn’s Disease Masquerading as an Acute Abdomen Jessica Judge, PA-C, Beverly P. Giordano, MS, RN, CPNP, PMHS, & Joan English, PA-C
KEY WORDS Crohn’s disease, abdominal pain
Section Editors Karin Reuter-Rice, PhD, CPNP-AC, FCCM Corresponding Editor Duke University Durham, North Carolina Terea Giannetta, DNP, RN, CPNP California State University Children’s Hospital Central California Fresno, California Maureen A. Madden, MSN, RN, CPNP-AC, CCRN, FCCM Rutgers Robert Wood Johnson Medical School New Brunswick, New Jersey Bristol Myers Squibb Children’s Hospital New Brunswick, New Jersey Jessica Judge, Physician Assistant, Crestview Health Center, Crestview, FL. At the time this article was prepared, she was Physician Assistant Student, College of Medicine, University of Florida, Gainesville, FL. Beverly P. Giordano, Pediatric Nurse Practitioner, Division of General Pediatrics, University of Florida, Gainesville, FL. Joan English, Physician Assistant, Division of Pediatric Gastroenterology, University of Florida, Gainesville, FL. Conflicts of interest: None to report. Correspondence: Beverly P. Giordano, MS, RN, CPNP, PMHS, 1701 SW 16th Ave, Gainesville, FL 32608; e-mail: bgiordano@ peds.ufl.edu. J Pediatr Health Care. (2014) -, ---. 0891-5245/$36.00 Copyright Q 2014 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pedhc.2014.03.003
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CASE PRESENTATION A 14-year-old African American boy presented to a primary care clinic with diffuse abdominal pain. He was in no acute distress and was conversational, but he was a poor historian. He reported a 2-week history of abdominal cramping, particularly at night. The pain was described as ‘‘all over,’’ increasing in severity over time, not associated with particular foods, and not alleviated with bowel movements. During the first week, the pain was intermittent and mild (3 to 4 out of 10), but it became constant and worsened to 6 to 7 out of 10 in severity during the 4 days before he presented. He denied having fever, constipation, hematochezia, or change in urine color. He denied recent travel. Although the patient denied having any life stressors, the pain had caused him to miss several days of school and basketball practice. MEDICAL HISTORY The patient’s growth and weight gain had been normal since birth; however, he had lost 10 lb in the previous 3 months. His medical history was positive for eczema, recurrent otitis externa, and joint pain thought to be sports related. His immunizations were up to date. FAMILY/SOCIAL/DEVELOPMENTAL HISTORY The patient’s mother had been treated for Grave’s disease, cholelithiasis, and tuberculosis. The family history was negative for other gastrointestinal disease or autoimmune processes. The patient lived with both parents and attended the ninth grade at a local high school. He participated in several sports. He was -/- 2014
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sexually active with females and reported occasional marijuana use. REVIEW OF SYSTEMS In addition to the abdominal pain, the patient reported ‘‘occasional’’ diarrhea. He also admitted to nausea, vomiting, and a recent decrease in appetite. Results of a pertinent review of other systems, except as noted, were otherwise negative. PHYSICAL EXAMINATION The patient was quiet but pleasant, ambulatory, and conversant. He did not appear to be in distress and had age-appropriate vital signs. His height was 182.9 cm (72 inches, 95th percentile) and his weight was 59.2 kg (130.5 lb, 65th percentile), with a body mass index of 17.70 kg/m2. His skin was warm and dry with no lesions. Results of his respiratory and cardiac examination were unremarkable. His abdomen was soft and nondistended with hypoactive bowel sounds present throughout. No organomegaly was noted, and the abdomen was tympanic to percussion. The patient had mild periumbilical tenderness with deep palpation, but no rebound tenderness. Obturator, psoas, and Murphy’s signs were negative. A rectal examination was not performed. The patient had no apparent neurologic deficits. DIAGNOSTIC TESTING AND CLINICAL COURSE At the initial clinic visit, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), complete blood cell count (CBC), complete metabolic profile (CMP), and stool Giardia and Helicobacter pylori antigen were ordered. Five days elapsed before test results were obtained. The results were significant for a CRP of 45.4 mg/L (normal, < 4.9 mg/L) and an ESR of 25 (normal, 0 to 20 mm/h). Results of the CBC and CMP were normal, and the stool tests were negative. The day after the blood tests were obtained, the patient presented to the clinic with worsening abdominal pain, which had localized to the right lower quadrant (RLQ). He had rebound tenderness in both lower quadrants. He was sent from the clinic to the emergency department (ED), and the pediatric surgery service was consulted because of a concern for possible acute appendicitis. The CRP on that day was further elevated (79.6 mg/L). A pelvic computed tomography (CT) scan demonstrated a dilated appendix measuring 12 mm with surrounding edema and inflammatory stranding in the RLQ and a large amount of free fluid in the pelvis. The radiologist’s impression was of appendicitis with no evidence of perforation or abscess formation despite the presence of fluid in the pelvis. The pediatric surgeon thought that a perforation was present and admitted the patient for a trial of nonoperative management with 1 week of ceftriaxone and metronidazole. This approach was consistent with guidelines for 2
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nonoperative management (i.e., patients with small appendiceal abscesses may be treated with intravenous [IV] antibiotics and then undergo an interval appendectomy 4 to 6 weeks later; Blakely et al., 2011). The patient improved clinically and was discharged on a regular diet. The day after discharge, the patient presented to the ED with recurrent RLQ pain and multiple episodes of nonbilious, nonbloody emesis. A CT of the abdomen and pelvis showed residual phlegmon (i.e., spreading diffuse inflammatory process with formation of suppurative/purulent exudate or pus) without a drainable abscess. His white blood cell count and CRP were within normal limits. Findings of an ultrasound of the right upper quadrant were unremarkable. Abdominal CT demonstrated cholelithiasis (later disproved) and increased distention of the distal small bowel with focal edema and thickening of the distal terminal ileum. The patient had a moderate volume of intra-abdominal free fluid but no intra-abdominal free air. He was admitted for 7 days of treatment with IV piperacillin/tazobactam. He improved clinically and had a CRP of 5.8 mg/L on the day of discharge. Twenty-four hours after being discharged, he presented to the ED with recurrent abdominal pain and vomiting. He was readmitted, and the pediatric gastroenterology service was consulted. An abdominal and pelvic CT scan demonstrated increasing inflammation in the terminal ileum. He underwent a colonoscopy and an upper endoscopy to evaluate for possible Crohn’s disease, assess the disease location, and possibly obtain tissue for pathologic examination. The bowel preparation procedure was unsatisfactory with poor cleanout. The findings of the colonoscopy and upper endoscopy were reported as normal, although the gastroenterologist who performed the procedure noted the presence of an abnormal perianal skin tag. Because the endoscopy procedures did not elucidate a diagnosis, the patient underwent a planned exploratory laparoscopy. The appendix was normal. However, the distal ileum was dilated and thickened, which was consistent with chronic obstruction and possible inflammatory bowel disease (IBD). The ileocecal valve appeared to contain a mass, which was concerning for IBD or malignancy. At this point, the procedure was converted to a laparotomy with resection of the distal ileum and half of the right colon. The resected terminal ileum and right colon demonstrated severe active chronic ileitis with fissures, ulceration, inflammation, and poorly formed granulomas, consistent with Crohn’s disease. The appendix had fibrous obliteration of the tip. Seven reactive lymph nodes were found with scattered granulomas. Gram and acid-fast bacteria stains were negative. The small bowel resection margin demonstrated mild active chronic ileitis with villitis, submucosal edema, and serositis, consistent with severe active IBD. The patient Journal of Pediatric Health Care
was discharged on a regular diet after this 14-day admission and was scheduled to follow up with the pediatric gastroenterology clinic. Two months after surgery, after having missed three appointments, the family finally met with the pediatric gastroenterologist. At that visit, the physician provided extensive education on IBD and the importance of medication compliance, answered the family’s questions, and measured the patient’s thiopurine methyltransferase (TPMT) activity, because the plan was to prescribe 6-mercaptopurine (6-MP; low TPMT activity increases the risk of drug-induced bone marrow suppression as a result of the accumulation of drug metabolites). The patient’s pretreatment TPMT activity was normal, and thus he was cleared to begin 6-MP at 1.5 mg/kg (100 mg by mouth daily), which is consistent with dosing guidelines (Ghazi, 2013). Follow-up TPMT monitoring was ordered for 2 weeks after beginning 6-MP therapy and again at 4 weeks, and he was to have 6-MP metabolite levels measured at 6 weeks after initiating therapy to monitor compliance. He was scheduled to return to the pediatric gastroenterology clinic in 6 weeks. The patient failed to undergo the follow-up TPMT monitoring. When the pediatric gastroenterologist contacted the family, the patient’s father reported that his son was not taking the prescribed 6-MP. The father stated, ‘‘I’m not sure why he needs this medication. I think his positive attitude and mind can control the disease.’’ The gastroenterologist reiterated that the bowel
inflammation would return without treatment, which would result in disease progression. One month later, the patient was seen again in the pediatric gastroenterology clinic. He and his parents expressed reluctance to accept the diagnosis of Crohn’s disease and were uncomfortable with making a long-term commitment to medication therapy. They requested a second opinion before beginning 6-MP therapy. However, when they returned 2 weeks later to meet with a different gastroenterologist, the patient reported that he was taking 6-MP, and he had gained 0.8 kg. Laboratory tests obtained at that visit included CRP, folate, vitamin B12, CMP, CBC, ESR, and vitamin D, 25-OH. All the laboratory tests were normal except for a low vitamin D, 25-OH level (18.7 ng/mL; normal, > 29 ng/mL). An over-the-counter vitamin D supplement was prescribed, and he was directed to continue taking the 6-MP. At an IBD support group meeting 2 months later, the patient reported that he had discontinued all medications. Eleven months after surgery, he is in a surgically induced clinical and biochemical remission. Although the pediatric gastroenterologist recommends that the patient take medication to prevent inflammation in other parts of his bowel, the patient’s family prefers to continue to monitor him while he remains medication-free. The patient’s most recent laboratory tests revealed normal serum inflammatory markers and no signs of anemia. He continues to gain weight and thrive.
CASE STUDY QUESTIONS 1. What are common presenting symptoms of Crohn’s disease? 2. How is Crohn’s disease managed? 3. What are sequelae of untreated Crohn’s disease? CASE STUDY ANSWERS Presenting Symptoms 1. What are common presenting symptoms of Crohn’s disease? Crohn’s disease is characterized by chronic intestinal inflammation in the absence of a recognized cause. Immune system malfunction, ethnicity (an Crohn’s disease is increased incidence in being discovered Ashkenazi Jews and a lower incidence in Afwith increasing rican Americans), famfrequency in ily history of Crohn’s children of all ages. disease, and environment (with a higher risk for urban dwellers) are risk factors (Sauer & Kugathasan, 2009). Crohn’s disease is being discovered with increasing frequency in children of all ages. Recent
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studies indicate that 20% to 30% of all patients with Crohn’s disease present before age 20 years (Grossman & Mamula, 2012). Disease location and severity determine the clinical presentation. The terminal ileum is involved in 50% to 70% of affected children (Grossman & Mamula, 2012). Children commonly present with a several-week history of abdominal pain, diarrhea, and weight loss (Day, 2012). The patient in this case study underreported his symptoms and withheld vital information, which delayed the diagnosis of Crohn’s disease. Crohn’s disease affecting the upper gastrointestinal tract typically causes nausea, vomiting, and abdominal pain, whereas disease in the small intestine causes signs of malabsorption (e.g., diarrhea, abdominal pain, growth deceleration, anorexia, and weight loss). Colonic Crohn’s disease may be difficult to distinguish
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from ulcerative colitis, because the symptoms (i.e., bloody diarrhea, cramping abdominal pain, and urgency to defecate) are similar (Grossman & Mamula, 2012). Perianal Crohn’s disease may cause skin tags, fissures, abscesses, fistulae, painful defecation, perirectal pain, bright red rectal bleeding, perirectal erythema, and rectal discharge. Abdominal examination findings can vary from normal to those seen with an acute abdomen. Diffuse abdominal pain often is present, along with a discrete mass found on palpation. Typically, the mass represents a palpable thickened loop of bowel (Day, 2012), as was the case in this patient. Extraintestinal manifestations are present in 25% to 35% of patients with Crohn’s disease (Grossman & Mamula, 2012). Dermatologic manifestations include erythema nodosum on the anterior aspects of the lower extremities and pyoderma gangrenosum on the dorsal surfaces of the feet and legs, ears, around stomas, and on the face. Anemic patients may have pallor. Peripheral asymmetric, polyarticular arthritis develops in 15% to 20% of patients and worsens with disease exacerbations. Approximately 10% of patients with Crohn’s disease experience ankylosing spondylitis, which presents as morning stiffness, diffuse low-back pain, and buttock pain (Friedman & Blumberg, 2011). Long-standing Crohn’s disease affects linear growth, and thus decreased growth velocity can be a sign of Crohn’s disease. The patient in this case study had achieved his final adult height before his diagnosis. Management 2. How is Crohn’s disease managed? The treatment goals are to reduce or eliminate symptoms; optimize nutritional status; promote normal growth and development; prevent complications; minimize psychological effects of chronic illness; achieve optimal clinical, laboratory, and histologic control of the inflammatory disease with the least adverse medication effects; and permit patients to function as normally as possible (e.g., school participation; Grossman & Mamula, 2012). Medication, lifestyle and dietary changes, stress reduction, and moderate activity and exercise are the mainstays of treatment. Treatment strategies depend on the severity and location of the disease. Monitoring When patients with Crohn’s disease have symptom exacerbation episodes, laboratory studies (e.g., CBC, CRP, ESR, fecal calprotectin, stool culture, stool Giardia, and testing for Clostridium difficile) help determine the presence of infections. The immunocompromised state of patients who take biologic therapies or immunomodulators increases the risk for invasive infections.
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Medications Corticosteroids, immunomodulators, and antitumor necrosis factor alpha (TNFa) agents are the mainstays of Crohn’s disease management, but they have inherent risks as well as benefits. Corticosteroids decrease the disease signs and symptoms but do not heal mucosa, and they impair growth and can lead to corticosteroid dependency. Immunomodulators (e.g., azathioprine and 6-MP) are effective maintenance therapies and offer corticosteroid-sparing effects. TNFa agents (e.g., infliximab, adalimumab, certolizumab, and natalizumab) bind to tumor-necrosing factor to block inflammatory response. They are one kind of biologic therapy approved as maintenance therapy for patients with moderate to severe Crohn’s disease who do not respond to standard therapies and for treatment of open, draining fistulas (National Digestive Diseases Information Clearinghouse [NDDIC], 2011). Traditionally, corticosteroids and anti-inflammatory medications containing mesalamine (sulfasalazine, 5aminosalicylic acid) were the medications used in the initial treatment of Crohn’s disease. However, they frequently do not control the disease adequately. Adverse effects include nausea, vomiting, heartburn, diarrhea, temporary hair loss, and headaches. Immunomodulators are standard therapy after initial treatment with corticosteroids. Regular laboratory tests (e.g., 6-MP/azathioprine metabolite testing) are required to monitor medication compliance. Women must take precautions to avoid pregnancy, because these medications are rated category D for pregnancy. A recent study compared the effectiveness of early treatment with an anti-TNFa agent versus an immunomodulator in attaining clinical remission and facilitating growth of pediatric patients. The investigators concluded that in newly diagnosed children with comparably severe Crohn’s disease, early (# 3 months after diagnosis) monotherapy with an anti-TNFa agent produced better overall clinical and growth outcomes at 1 year than did early monotherapy with an immunomodulator (Walters & Kim, 2014). A retrospective analysis of long-term patient outcomes demonstrated efficacy and safety of anti-TNFa treatment for pediatric IBD (Assa et al., 2013). Adjunctive use of immunomodulators and anti-TNFa agents increases TNFa drug levels, reduces the risk of antidrug antibodies, and improves response (Absah & Stephens, 2013). These results are tempered by reports of the development of hepatosplenic T-cell lymphoma in younger patients while taking thiopurine immunomodulators with and without concomitant anti-TNFa medications (Absah & Stephens, 2013; Hyams, 2009). Information available at www.ccfa.org/assets/pdfs/biologic102011.pdf provides a brief explanation of the biologic source of TNF blockers and their role in targeting the inflammation process.
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Antibiotics (e.g., metronidazole and ciprofloxacin) are used for patients with colonic or perianal involvement and also to treat abscesses accompanying Crohn’s disease. Rifaximin and oral vancomycin are nonabsorbable antibiotics that are effective in treating C. difficile infections, which can be difficult to manage in patients with Crohn’s disease. Oral iron supplements or IV iron sucrose may be needed to treat anemia caused by blood loss and poor intestinal absorption. Psyllium powder or methylcellulose may relieve diarrhea by adding bulk to the stool. Loperamide can relieve severe diarrhea. Laxatives, along with low-residue diets, may be indicated if inflammation narrows the intestinal lumen, leading to constipation. Acetaminophen is usually preferred for pain relief, because aspirin and nonsteroidal antiinflammatory medications may worsen symptoms. Vitamin B12 injections may be required if terminal ileum inflammation or surgical removal interferes with vitamin absorption. Calcium and vitamin D supplements may be prescribed to counteract the increased risk for osteoporosis associated with corticosteroid therapy. Other unproven but currently hotly debated therapies for Crohn’s disease include fecal transplant (i.e., adding to the microbiota present in bowel), helminth therapy, and the use of certain probiotics for management of pouchitis. Lifestyle changes Dietary adjustments, proper hydration, and smoking cessation are essential. Regular exercise, a healthy diet, and sufficient sleep can counteract fatigue. Nutritional management may include enteral or parental nutrition to improve overall nutrition and allow bowel rest. Enteral nutrition can induce remission, but relapse is common after primary nutritional therapy is discontinued, mandating concomitant pharmacologic therapy (Grossman & Mamula, 2012). Foods with high fiber content or gluten and dairy products can trigger symptoms. Patients with small intestine disease may have diarrhea with rapid intestinal transit of fatty foods. Many patients tolerate five or six small meals per day rather than two or three larger ones. Carbonated drinks produce gas, and caffeinated drinks stimulate the intestines and can worsen diarrhea. Primary care providers should ensure that patients who take biologic therapy agents have annual tests for tuberculosis. Sunscreen use must be emphasized at every encounter, and annual influenza vaccines should be administered. Additionally, referrals for annual eye examinations are part of care coordination. When patients are initially diagnosed with Crohn’s disease, it is important to ensure that immunizations are up to date, because no live vaccines can be administered to patients who take biologic agents.
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Surgery Approximately two thirds of patients with Crohn’s disease require surgery at some point in their lives (Lichtenstein, Hanauer, Sandborn, & the Practice Parameters Committee, 2009). Surgical intervention can range from resection of only the diseased portion to proctocolectomy (removal of the rectum and part of or the entire colon). Surgery may be indicated for obstructions, fistulas, and abscesses or if the disease is unresponsive to medication. However, surgery does not cure Crohn’s disease, and postoperative complications are common. After bowel resection, scar tissue can cause strictures, leading to bowel obstruction. Patients who have duodenal obstructions caused by strictures may be managed with strictureplasty or resection of the affected portion of the bowel (Viola et al., 2013). Growth impairment and delayed pubertal development are common in children with Crohn’s disease. Growth rate is a marker of the disease status. Close monitoring of growth Growth impairment velocity and periodic and delayed determination of skeletal maturation are pubertal essential components development are of the treatment plan common in children (Gupta, Lustig, Kohn, & Vittinghoff, 2013). with Crohn’s Coping with Crohn’s disease. disease is challenging. Long-term remission management requires most patients to take one or more oral medications daily in the absence of symptoms. Adverse effects of medication (e.g., nausea, vomiting, headaches, weight gain, facial changes, excessive facial hair, decreased resistance to infection, seizures, increased cancer risk, tendon rupture, and numbness and tingling in the extremities) interfere with treatment adherence (LeLeiko et al., 2013). Periodic imaging studies (e.g., upper GI series with small-bowel follow-through and magnetic resonance enterography) may be indicated to look for small bowel disease, disease activity, structuring, and fistulae. Magnetic resonance enterography has the advantage of no radiation exposure but has limited use because it requires that the patient drink large amounts of a contrast solution. Crohn’s disease outcomes have not improved with advances in technology and medications. Mortality, cancer, disease recurrence, extraintestinal manifestations, recurrent hospitalizations, and surgery continue to complicate the lives of patients with Crohn’s disease. A study of 246 pediatric patients with Crohn’s disease who were followed up at 48 practice sites demonstrated a wide variation in diagnostic and therapeutic interventions and gaps between recommended and actual care (Colletti et al., 2009). The investigators recommended
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use of quality improvement methods to evaluate the effectiveness of treatment guidelines, reduce treatment variation, and improve clinical outcomes of children and adolescents with Crohn’s disease. The diagnosis of Crohn’s disease is a major insult to adolescent self-esteem. The disease symptoms are embarrassing and can lead to social isolation. Diagnostic procedures are invasive, and surgical intervention reduces all-important social and academic activities. Dietary recommendations are inconsistent with the average adolescent’s eating habits. Support groups and organizations (see http://www.ccfa.org, http:// www.crohnsandme.com, and http://www.pedsibd. org/teens) are available to help patients and families cope with Crohn’s disease (D’Auria & Kelly, 2013). Students with Crohn’s disease should have 504 plans (Section 504 of the Rehabilitation Act of 1973) that grant ad lib bathroom access at school (http://www.ccfa.org/ resources/template-section-504-plan.html). Stress can trigger Crohn’s symptoms and must be managed along with nutrition and medication. Sequelae of Untreated Disease 3. What are the sequelae of untreated Crohn’s disease? Abscesses, fistulae, sinus tract formation, strictures, and bowel adhesions may occur because of the transmural nature of Crohn’s disease. Perianal and perirectal fistula formation are the most common, although enterovesical and enterocutaneous fistulas also occur (Irani, Ramsanahie, & Bleday, 2012). Surgical resection of the terminal ileum may cause spillage of bile salts into the colon, which, combined with absence of bile acid sequestrants, can cause chronic diarrhea. If more than 100 cm of ileum is resected, fat cannot be digested, and steatorrhea may develop (Irani et al., 2012). Small bowel overgrowth frequently occurs, especially in patients who have undergone ileocecal valve resection or those with stricture disease. Deficiencies in fatsoluble vitamins (A, D, E, K), iron, folate, and vitamin B12 are common, leading to osteoporosis and anemia. Bile salt reabsorption may be compromised by the disease’s effect on the ileum. The most feared long-term complication of Crohn’s disease is colorectal cancer (CRC). Patients with Crohn’s disease and those with ulcerative colitis have equal risks for developing CRC (Freeman, 2008). Risk factors include duration and extent of disease, severity of inflammation, family history of CRC, and early age at Crohn’s diagnosis. Unlike typical CRC, which presents with adenomatous polyps, the CRC associated with Crohn’s disease typically presents as dysplastic epithelium that may be difficult to recognize. Thus the standard of care is to begin colonoscopy screening 8 to 10 years after Crohn’s disease diagnosis and continue this screening every 2 years with random four-quadrant
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biopsies every 10 cm throughout the colon (U.S. Preventive Services Task Force, 2008). The patient in this case study underwent resection of 10.8 cm of terminal ileum continuous with the cecum and ascending colon. This surgery increases his risk for bile salt–induced diarrhea and vitamin B12 deficiency. His young age at diagnosis and the extent of disease at diagnosis put him in the high-risk .clinicians must category for develinclude Crohn’s oping CRC later in life.
disease in the CONCLUSION differential Crohn’s disease does diagnosis of not typically present as an acute abdomen. patients with However, clinicians abdominal pain. must include Crohn’s disease in the differential diagnosis of patients with abdominal pain. This recommendation is especially relevant to patients with a long history of vague abdominal complaints and those with unexplained weight loss or growth velocity deceleration. We thank Dr. Regino Gonzalez-Peralta, pediatric gastroenterologist at the University of Florida, for content review. REFERENCES Absah, I., & Stephens, M. (2013). Adjunctive treatment to antitumor necrosis factor in pediatric patients with refractory Crohn’s disease. Current Opinion in Pediatrics, 25(5), 624-628. Assa, A., Hartman, C., Weiss, B., Broide, E., Rosenbach, Y., Zevit, N., . Shamir, R. (2013). Long-term outcome of tumor necrosis factor alpha antagonist’s treatment in pediatric Crohn’s disease. Journal of Crohn’s and Colitis, 7(5), 369-376. Blakely, M. L., Williams, R., Dassinger, M. S., Eubanks, J. W., Fischer, P., Huang, E. U., . Langham, M. R. (2011). Early vs interval appendectomy for children with perforated appendicitis. Archives of Surgery, 146(6), 600-665. Colletti, R. B., Baldassano, R. N., Milov, D. E., Margolis, P. A., Bousavaros, A., Crandall, W. V., . Qureshi, M. A. (2009). Variation in care in pediatric Crohn disease. Journal of Pediatric Gastroenterology and Nutrition, 49, 297-303. Day, A. S. (2012). Crohn’s and colitis in children and adolescents. World Journal of Gastroenterology, 41(18), 5862-5869. D’Auria, J. P., & Kelly, M. (2013). Inflammatory bowel disease: Top resources for children, adolescents, and their families. Journal of Pediatric Health Care, 27(2), e25-e28. Freeman, H. J. (2008). Colorectal cancer risk in Crohn’s disease. World Journal of Gastroenterology, 14(12), 1810-1811. Friedman, S., & Blumberg, R. S. (2011). Disorders of the alimentary tract: Inflammatory bowel disease. In D. Longo, A. Fauci, D. Kasper, S. Hause, J. Jameson & J. Loscalzo (Eds.), Harrison’s principles of internal medicine (18th ed., pp. 24772496). New York, NY: McGraw. Ghazi, L. J. (2013). Crohn disease medication. Retrieved from http:// emedicine.medscape.com/article/172940-medication Grossman, A. B., & Mamula, P. (2012). Pediatric Crohn disease. Retrieved from http://emedicine.medscape.com/article/928288
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Gupta, N., Lustig, R. H., Kohn, M. A., & Vittinghoff, E. (2013). Determination of bone age in pediatric patients with Crohn’s disease should become part of routine care. Inflammatory Bowel Disease, 19(1), 61-65. Hyams, J. S. (2009). Risk/benefit strategies must be employed in the management of pediatric Crohn’s disease. Digestive Diseases, 27(3), 291-296. Irani, J. L., Ramsanahie, A., & Bleday, R. (2012). Inflammatory bowel disease: surgical considerations. Crohn Disease. In N. Greenberger, R. Blumberg & R. Burakoff (Eds.), Current diagnosis and treatment—gastroenterology, hepatology & endoscopy (2nd ed.). New York, NY: McGraw Medical. LeLeiko, N. S., Lobato, D., Hagin, S., McQuaid, E., Seifer, R., Kopel, S. J., . Bancroft, B. (2013). Rates and predictors of oral medication adherence in pediatric patients with IBD. Inflammatory Bowel Disease, 19(4), 832-839. Lichtenstein, G. R., Hanauer, S. B., Sandborn, W. J. & the Practice Parameters Committee of The American College of Gastroenterology. (2009). Management of Crohn’s disease in adults. American Journal of Gastroenterology, 104(2), 465-483.
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National Digestive Diseases Information Clearinghouse. (2011). Crohn’s disease. Retrieved from http://digestive.niddk.nih. gov/ddiseases/pubs/crohns Sauer, C. G., & Kugathasan, S. (2009). Pediatric inflammatory bowel disease: Highlighting pediatric differences in IBD. Gastroenterology Clinics of North America, 38(4), 611-628. U.S. Preventive Services Task Force. Screening for colorectal cancer: U.S. Preventive Services Task Force recommendation statement (AHRQ Publication No. 08-05124-EF-3, October 2008). Retrieved from http://www.uspreventiveservicestaskforce. org/uspstf08/colocancer/colors.htm Viola, A. M., D’Arcangelo, G., DiNardo, G., Civitelli, F., Casciani, E., Oliva, S., . Cucchiara, S. (2013). Disease course and efficacy of medical therapy in structuring paediatric Crohn’s disease. Digestive and Liver Disease, 45(6), 464-468. Walters, T. D., Kim, M., Denson, L. A., Griffiths, A. M., Dubinsky, M., Markowitz, J., . Hyams, J. S. (2014). Increased effectiveness of early therapy with anti-tumor necrosis factor-a vs an immunomodulator in children with Crohn’s disease. Gastroenterology, 146(2), 383-391.
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