Pediatric Crohn Disease Follow-up

  • Author: Andrew B Grossman, MD; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Apr 27, 2011
 

Further Inpatient Care

  • Most patients with presumed Crohn disease (CD), or Crohn's disease, can undergo outpatient diagnostic evaluation.
  • Patients with an exacerbation of Crohn disease can be treated on an outpatient basis; however, if concern a serious complication of Crohn disease (eg, obstruction, perforation, abscess, hemorrhage) is a concern or if the patient fails outpatient treatment, intravenous [IV] therapy (eg, corticosteroids, antibiotics, total parenteral nutrition) may be required and hospitalization is warranted.
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Further Outpatient Care

  • Patients should be examined on a regular basis. The frequency depends on the severity and activity of their disease.
  • Follow-up laboratory workup should be performed regularly to monitor the safety and success of therapy.
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Complications

  • The major intestinal complications of Crohn disease are due to the transmural nature of the disease. This leads to the formation of abscesses, fistulae, sinus tracts (incomplete fistulae ending in a "cul de sac"), strictures, and adhesions, which may also contribute to obstruction.
    • Frank perforation is one of the most serious complications of Crohn disease. Perforation typically occurs into other segments of bowel, leading to fistulae, or to areas such as the retroperitoneum, resulting in abscess formation. The presenting features of frank perforation are those of classic peritonitis, although high-dose corticosteroid therapy may mask these features.
    • Fistula and abscess formation is common in Crohn disease and is due to transmural bowel perforation. Perianal and perirectal fistulization are most common. Proper evaluation of perianal disease requires a combination of 2 of the following: pelvic MRI, examination under anesthesia, or endoscopic ultrasonography. Other complications of fistulizing disease include enterovesical and enterocutaneous fistulas.
  • Colonic malignancy is a clinically significant complication of Crohn disease in patients with pancolitis beginning in childhood.
    • Although the risk of malignancy in Crohn disease is not as high as that in ulcerative colitis (UC), the risk of adenocarcinoma of the colon in Crohn colitis is 4-20 times that of the general population. Small intestinal carcinoma is 50-100 times more likely to develop in patients with small intestinal Crohn disease but is still rare. The risk for children with an onset of disease in the first decade is unknown, but children who develop colitis when younger than 10 years should undergo colonoscopic screening during adolescence.
    • Epithelial dysplasia generally precedes carcinoma; therefore, yearly surveillance colonoscopy is recommended for patients with this condition, who are at high risk.
  • Approximately 25-35% of patients with Crohn disease have at least one extraintestinal manifestation, which may be diagnosed before, when, or after Crohn disease is diagnosed. Extraintestinal manifestations may carry prognostic importance and include the following:
    • Dermatologic manifestations
      • Erythema nodosum
      • Pyoderma gangrenosum
      • Orofacial granulomatosis
      • Angular and aphthous stomatitis
      • Acrodermatitis enteropathica
      • Alopecia
      • Metastatic Crohn disease
      • Crohn disease of the vulva and penis
    • Ophthalmologic manifestations
      • Episcleritis
      • Uveitis, iritis
      • Conjunctivitis
    • Musculoskeletal manifestations
      • Arthralgia
      • Arthritis
      • Ankylosing spondylitis
      • Sacroiliitis
    • Bone metabolic disorders
    • Hematologic manifestations
      • Iron deficiency anemia
      • Vitamin B12 deficiency anemia
      • Folate deficiency anemia
      • Anemia of chronic disease
      • Autoimmune hemolytic anemia
      • Thrombocytosis
      • Anemia due to GI bleed
      • Thrombosis
    • Hepatobiliary manifestations
      • Primary sclerosing cholangitis
      • Autoimmune hepatitis
      • Granulomatous hepatitis
      • Cholelithiasis
      • Portal vein thrombosis
    • Genitourinary manifestations
      • Nephrolithiasis
      • Obstructive uropathy
      • Fistulas (enterovesical)
      • Glomerulonephritis
      • Amyloidosis
    • Pancreatic manifestations - Pancreatitis
    • Pulmonary manifestations
      • Granulomatous lung disease
      • Fibrosing alveolitis
      • Pulmonary vasculitis
    • Cardiovascular manifestations
      • Pericarditis
      • Myocarditis
      • Vasculitis
  • Growth failure and delayed sexual development are common in adolescents and children with Crohn disease. From studies of the growth of children with Crohn disease, impairment of linear growth was common before diagnosis and in subsequent years. Decrease in height velocity before the onset of intestinal symptoms can be observed in as many as 46% of patients with Tanner stage 1 or 2. Height at maturity is often compromised. The etiology of growth failure is multifactorial, with nutritional, hormonal, and disease-related factors all contributing.
  • The most common extraintestinal manifestation in children and adolescents is arthritis (7-25% of pediatric patients). The arthritis is usually transient, nondeforming, asymmetric in distribution, and involves the large joints of the lower extremities. In adults, the arthritis occurs when the disease is active, but in children, the arthritis may occur years before any GI symptoms develop.
  • Skin manifestations include the following:
    • The most common skin manifestation of Crohn disease is erythema nodosum. Erythema nodosum is more common in Crohn disease than in UC and usually follows the course of the disease. Erythema nodosum affects 3% of pediatric patients with Crohn disease, less frequent than in adults. Approximately 75% of patients with erythema nodosum ultimately develop arthritis. The lesions of erythema nodosum are raised, red, tender nodules that appear primarily on the anterior surfaces of the lower leg.
    • Pyoderma gangrenosum is another skin manifestation, although it is uncommon in Crohn disease. Pyoderma gangrenosum is often an indolent chronic ulcer, which may occur even when the disease is in remission. Therefore, medical therapy for the underlying bowel disease is not always successful.
  • Aphthous ulceration in the mouth is the most common oral manifestation of Crohn disease. This ulceration is commonly associated with skin and joint lesions. Oral lesions appear to parallel intestinal disease in most cases, but they may also occur before any GI symptoms occur.
  • In Crohn disease, ophthalmologic manifestations most frequently occur when the disease is active. The rate is 4% in the adult population but is lower in children and adolescents. The most common ocular findings are episcleritis and anterior uveitis. The uveitis is usually symptomatic, causing pain or decreased visual acuity. Increased intraocular pressure and cataracts may be observed in children who receive corticosteroid therapy. All patients with Crohn disease require ophthalmologic examination at regular intervals.
  • Urologic manifestations of Crohn disease include nephrolithiasis, hydronephrosis, and enterovesical fistulae. Nephrolithiasis occurs in less than 5% of children with Crohn disease. Nephrolithiasis is usually the result of fat malabsorption that occurs with small bowel Crohn disease. Dietary calcium binds to malabsorbed fatty acids in the colonic lumen; therefore, free oxalate is absorbed. The absorption of free oxalate results in hyperoxaluria and oxalate stones. In patients with an ileostomy, increased fluid and electrolyte losses may lead to concentrated acidic urine and the formation of uric acid stones. External compression of the ureter by an inflammatory mass or abscess may lead to hydronephrosis. Enterovesical fistulae may present with recurrent urinary tract infections or pneumaturia.
  • Hepatobiliary disease is one of the most common extraintestinal manifestations of Crohn disease and its therapies.
    • Abnormal serum aminotransferases are common during the course of Crohn disease in children. Most aminotransferase elevations are transient and appear to relate to medications or disease activity. Persistent aminotransferase elevations (>6 mo) should be investigated because the likelihood of serious liver disease is increased.
    • Both intrahepatic and extrahepatic manifestations of liver disease occur in children with Crohn disease. Intrahepatic manifestations include chronic active hepatitis, granulomatous hepatitis, amyloidosis, fatty liver, and pericholangitis. Extrahepatic manifestations include cholelithiasis and obstruction.
    • Chronic active hepatitis and sclerosing cholangitis develops in fewer than 1% of children with Crohn disease.
  • Thromboembolic disease is considered the result of a hypercoagulable state that parallels disease activity and is manifested by thrombocytosis, elevated plasma fibrinogen, factor V, factor VIII, and decreased plasma antithrombin III. This may lead to deep vein thrombosis, pulmonary emboli, and neurovascular disease.
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Prognosis

  • Crohn disease may largely affect the life of a child or adolescent.
  • With appropriate treatment and support, the prognosis is good, and the risk of a fatal outcome is extremely low.
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Patient Education

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Contributor Information and Disclosures
Author

Andrew B Grossman, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine

Andrew B Grossman, MD is a member of the following medical societies: American Gastroenterological Association, Crohns and Colitis Foundation of America, and North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Coauthor(s)

Petar Mamula, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine

Petar Mamula, MD, is a member of the following medical societies: American Academy of Pediatrics, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Specialty Editor Board

Robert Baldassano, MD  Director, Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Nutrition, Associate Professor, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania

Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Stefano Guandalini, MD  Director, University of Chicago Celiac Disease Program, Section Chief of Gastroenterology, Hepatology and Nutrition; Professor, Department of Pediatrics, University of Chicago Comer Children's Hospital

Stefano Guandalini, MD is a member of the following medical societies: American Gastroenterological Association, European Society for Paediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research

Disclosure: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

Chief Editor

Carmen Cuffari, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

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Colonoscopic image of a large ulcer and inflammation of the descending colon in a 12-year-old boy with Crohn disease.
Histologic features of chronic colitis with crypt atrophy and branching, and lymphocytic infiltrate. Hematoxylin-eosin staining. Courtesy of Dr E. Ruchelli.
Colonic granuloma in a patient with Crohn disease. Hematoxylin-eosin staining. Courtesy of Dr E. Ruchelli.
Image obtained during upper GI series with a small-bowel follow-through shows narrowing and irregularity in the distal ileum in a 16-year-old male adolescent with Crohn disease.
MRI of an inflamed terminal ileum in a 10-year-old girl with Crohn disease.
MRI of a small abscess on the right side of the anal sphincter in a 9-year-old boy with Crohn disease.
Table. Characteristics Differentiating Crohn Disease and Ulcerative Colitis
Characteristic
Crohn diseaseUlcerative Colitis
DistributionEntire GI tractColon only, although gastritis recognized
Skip lesionsContinuous involvement proximally from rectum
PathologyFull thicknessMucosa only
Granulomas (30%)No granulomas
RadiologyEntire GI tractColon only
Skip lesionsContinuous involvement proximally from rectum
Fistulas, abscesses, fibrotic stricturesMucosal disease only
Cancer riskIncreasedEstimated 1% per year starting 10 years after diagnosis
Presentation
Crohn DiseaseUlcerative Colitis
BleedingCommonVery common
ObstructionCommonUncommon
FistulaCommonNone
Weight lossCommonUncommon
Perianal diseaseCommonRare
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