Inflammatory bowel disease (IBD) is a spectrum involving Crohn disease (CD) and ulcerative colitis (UC).
CD was first written about by Dr Crohn in 1932 describing terminal ileitis.
UC was described first in 1859 by Wilks and Moxon.
Initially fecal diversion and end ileostomy were recommended earlier in the 1900s, but in 1944 Strauss and Strauss introduced proctocolectomy and ileostomy for severe UC.1
EPIDEMIOLOGY AND ETIOLOGY
The annual incidence of UC varies between 0 and 19.2 per 100 000 in North America.
CD has a similar incidence of 0 to 20.2 per 100 000 in North America.3
There has been a general increase in incidence globally since World War II.2
Approximately 25% of IBD cases present in childhood.
Pediatric CD is slightly more dominant in males (1.5:1 ratio), but UC is equal.
There is also a significantly higher ratio of CD to UC in children compared with adult populations.4
Etiology of CD is unknown, but it is likely induced by multiple environmental and genetic factors.
Mutations of NOD2 and CARD15 on chromosome 16 have been noted in 30% to 43% of CD patients.
Also gene IBD5 on chromosome 5q31 has been associated with perianal disease.
A gene variant of ATG16L1 has been recently noted to be related to increased abnormal inflammatory response.2
UC is also not fully understood but is a chronic immune-mediated inflammatory condition.
Recent genetic research has noted increased risk with presence of HLA-B27 and some association with NOD2 and IL23R genes.
TABLE 27.1 Comparison of Crohn Disease and Ulcerative Colitis
Crohn Disease
Ulcerative Colitis
Epidemiology
More common in whites vs blacks
More common in Jews vs non-Jews
More common in women
Affects young adults
More common in whites vs blacks
No sex predilection
Affects young adults
Extent
Transmural
Mucosal and submucosal
Location
Terminal ileum alone (30%)
Ileum and colon (50%)
Colon alone (20%)
Involves other areas of GI tract (mouth to anus)
Mainly the rectum
May extend into descending colon
May involve entire colon
Does not involve other areas of GI tract
Gross features
Thick bowel wall and narrow lumen (leads to obstruction)
Aphthous ulcers (early sign)
Skip lesions, strictures, and fistulas
Deep linear ulcers with cobblestone pattern
Fat creeping around the serosa
Inflammatory pseudopolyps
Areas of friable, bloody residual mucosa
Ulceration and hemorrhage
Microscopic findings
Noncaseating granulomas
Lymphoid aggregates
Dysplasia or cancer less likely
Ulcers and intestinal gland abscesses with neutrophils
Dysplasia of cancer may be present
Clinical findings
Recurrent right lower quadrant colicky pain with diarrhea
Bleeding occurs with colon or anal involvement
Recurrent left-sided abdominal cramping with bloody diarrhea and mucus
Radiography
“String” sign in terminal ileum due to luminal narrowing
“Lead pipe” appearance chronic state
Complications
Fistulas, obstruction
Calcium oxalate renal calculi
Malabsorption due to bile deficiency
Macrocytic anemia due to vitamin B12 deficiency
Toxic megacolon
Primary sclerosing cholangitis
Adenocarcinoma
Reprinted with permission from Dudek RW, Louis TM. High-Yield Gross Anatomy. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2015.
CLINICAL PRESENTATION
Pediatric CD presents with colonic or ileocolonic involvement in 80% to 90% cases.
They generally present with inflammatory disease and then progress into structuring and penetrating disease.4
CD patients usually present with crampy abdominal pain usually in right lower abdomen, diarrhea, loss of appetite, weight loss, and failure to thrive (50%).
10% will have perianal fistulas or other perianal diseases such as fissures and abscesses.
UC also presents mainly as pancolitis in children (80%-90%).4
Initial symptoms usually are persistent diarrhea with blood, mucus, and pus, along with abdominal cramping, tenesmus, and anemia.
Anorexia, weight loss, and growth retardation occur in up to 38% of UC patients.
About 3% of patients develop colorectal cancer in the first 10 years of disease, which increased to 10% to 15% afterward.
Extracolonic manifestations of IBD are arthralgias, skin lesions (erythema nodosum, pyoderma gangrenosum), osteoporosis, primary sclerosing cholangitis, nephrolithiasis, uveitis, and stomatitis.1,2
DIAGNOSIS
During a thorough history and physical examination, rectal examination is important especially if there is any concern for perianal disease.
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