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Differentiating ulcerative colitis from Crohn disease in children
and young adults: report of a working group of the North American
Society for Pediatric Gastroenterology, Hepatology, and Nutrition
and the Crohn's and Colitis Foundation of America.J
Pediatr Gastroenterol Nutr. 2007 May;44(5):653-74.
BACKGROUND: Studies of pediatric inflammatory
bowel disease (IBD) have varied in the criteria used to classify
patients as having Crohn disease (CD), ulcerative colitis (UC), or
indeterminate colitis (IC). Patients undergoing an initial
evaluation for IBD will often undergo a series of diagnostic tests,
including barium upper gastrointestinal series with small bowel
follow-through, abdominal CT, upper endoscopy, and colonoscopy with
biopsies. Other tests performed less frequently include magnetic
resonance imaging scans, serological testing, and capsule endoscopy.
The large amount of clinical information obtained may make a
physician uncertain as to whether to label a patient as having CD or
UC. Nevertheless, to facilitate the conduct of epidemiological
studies in children, to allow the entry of children into clinical
trials, and to allow physicians to more clearly discuss diagnosis
with their patients, it is important that clinicians be able to
differentiate between CD and UC. METHODS: A consensus conference
regarding the diagnosis and classification of pediatric IBD was
organized by the Crohn's and Colitis Foundation of America. The
meeting included 10 pediatric gastroenterologists and 4 pediatric
pathologists. The primary aim was to determine the utility of
endoscopy and histology in establishing the diagnosis of CD and UC.
Each member of the group was assigned a topic for review. Topics
evaluated included differentiating inflammatory bowel disease from
acute self-limited colitis, endoscopic and histological features
that allow differentiation between CD and UC, upper endoscopic
features seen in both CD and UC, ileal inflammation and "backwash
ileitis" in UC, patchiness and rectal sparing in pediatric IBD,
periappendiceal inflammation in CD and UC, and definitions of IC.
RESULTS: Patients with UC may have histological features such as
microscopic inflammation of the ileum, histological gastritis,
periappendiceal inflammation, patchiness, and relative rectal
sparing at the time of diagnosis. These findings should not prompt
the clinician to change the diagnosis from UC to CD. Other
endoscopic findings, such as macroscopic cobblestoning, segmental
colitis, ileal stenosis and ulceration, perianal disease, and
multiple granulomas in the small bowel or colon more strongly
suggest a diagnosis of CD. An algorithm is provided to enable the
clinician to differentiate more reliably between these 2 entities.
CONCLUSIONS: The recommendations and algorithm presented here aim to
assist the clinician in differentiating childhood UC from CD. We
hope the recommendations in this report will reduce variability
among practitioners in how they use the terms "ulcerative colitis,"
"Crohn disease," and "indeterminate colitis." The authors hope that
progress being made in genetic, serological, and imaging studies
leads to more reliable phenotyping.
Diagnostic problems and advances in
inflammatory bowel disease.Mod
Pathol. 2003 Apr;16(4):347-58.
This review summarizes current diagnostic
problems and advances with regard to patterns of inflammation and
dysplasia in ulcerative colitis and Crohn's disease. Ulcerative
colitis and Crohn's disease have a variety of characteristic but
non-specific pathologic features. In approximately 5% of
inflammatory bowel disease cases, a definite diagnosis of ulcerative
colitis or Crohn's disease cannot be established, in which case the
term "indeterminate" colitis is used. Most cases of indeterminate
colitis are related to fulminant colitis, a condition in which the
classic features of ulcerative colitis or Crohn's disease may be
obscured by severe ulceration with early superficial fissuring
ulceration, transmural lymphoid aggregates, and relative rectal
sparing. Approximately 20% of patients with indeterminate colitis
develop severe pouch complications, which is intermediate in
frequency between ulcerative colitis (8-10%) and Crohn's disease
(30-40%). In order to establish a diagnosis of ulcerative colitis or
Crohn's disease, it is important to evaluate pathologic material in
conjunction with clinical, laboratory, radiologic, and endoscopic
features and to recognize the variety of changes that may be seen in
fulminant ulcerative colitis. There are a number of exceptions to
the classic principles of inflammatory bowel disease pathology that
may lead to diagnostic confusion. For instance, apparent skip
lesions on biopsy analysis may occur in patients with ulcerative
colitis in the following settings; long term oral or topical
therapy, focal ascending colon, cecum and/or appendiceal involvement
in patients with left sided ulcerative colitis, upper
gastrointestinal involvement in patients with ulcerative colitis,
and at initial presentation of ulcerative colitis in pediatric
patients. In all of these circumstances, the finding of patchy
disease and/or rectal sparing should not be misinterpreted as either
evidence against a diagnosis of ulcerative colitis, or as
representing skip areas characteristic of Crohn's disease. Patients
with ulcerative colitis and Crohn's disease are at increased risk
for the development of dysplasia and carcinoma. Recent studies
suggest that given a similar duration and extent of disease,
patients with Crohn's disease have a similar risk of dysplasia and
cancer as patients with ulcerative colitis. Dysplasia in ulcerative
colitis may be classified as flat or elevated (dysplasia associated
lesion or mass [DALM]). Patients with flat high grade dysplasia are
generally treated with colectomy. However, there is recent evidence
to suggest that patients with flat low grade dysplasia, particularly
if detected at the time of initial endoscopic exam, or if its
multifocal or synchronous, should also be treated with colectomy.
Elevated lesions in ulcerative colitis (DALM) are subdivided into
"adenoma-like" and "non-adenoma-like" lesions based on their
endoscopic appearance. Recent data suggests that adenoma-like
lesions, regardless of the grade of dysplasia, or the location of
the lesion (i.e., inside or outside areas of established colitis)
may be treated adequately by polypectomy if there are no other areas
of flat dysplasia in the patient. Although there are some histologic
and molecular features that can help differentiate sporadic adenomas
from adenoma-like polypoid dysplastic lesions related to ulcerative
colitis, none of these adjunctive techniques can help distinguish
these lesions definitively in any single patient. Patients with a
non-adenoma-like DALM, (irregular, broad based, or strictured
lesion) should be treated with colectomy because of the high
probability of adenocarcinoma. The surveillance and treatment
options for patients with flat and elevated dysplasia in ulcerative
colitis are reviewed in detail. |