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Atypical rectosigmoid histology in children with newly diagnosed ulcerative colitis
J Markowitz1, E Kahn, K Grancher
1Department of Pediatrics, North Shore University Hospital-Cornell University Medical College, Manhasset, New York.
Insights
Rectal sparing in children with inflammatory colitis may indicate atypical ulcerative colitis (UC), not Crohn's disease (CD). This finding is crucial for accurate diagnosis and treatment planning in pediatric colitis cases.
Area of Science:
- Pediatric Gastroenterology
- Colorectal Pathology
- Inflammatory Bowel Disease
Background:
- Rectal sparing or patchy rectal inflammation in untreated inflammatory colitis is typically associated with Crohn's disease (CD).
- Distinguishing between ulcerative colitis (UC) and CD is critical for appropriate patient management.
Purpose of the Study:
- To investigate the presence of atypical histological findings in rectal biopsies of children with ulcerative colitis (UC) at disease onset.
- To determine if rectal sparing or patchy inflammation can occur in untreated pediatric UC.
Main Methods:
- Retrospective review of endoscopic rectosigmoid mucosal biopsies from 12 treatment-naive children with UC requiring surgery.
- Biopsies were blindly classified as typical UC (diffuse inflammation, severe crypt destruction) or CD (patchy inflammation, mild crypt changes).
- Biopsies classified as normal or CD were considered evidence of atypical UC with rectal sparing, as all subjects were confirmed to have UC.
Main Results:
- Five of 12 subjects (38%) exhibited atypical histology, including mild patchy inflammation or normal rectal biopsies.
- Atypical findings were observed in both endoscopically normal and inflamed rectosigmoid segments.
- No clinical features differentiated subjects with atypical versus typical UC histology.
Conclusions:
- Patchy or absent rectal inflammation can be a feature of untreated pediatric ulcerative colitis at disease onset.
- Misdiagnosis as Crohn's disease is possible, necessitating careful consideration of these findings in treatment and research protocols for pediatric colitis.
Background:
In the untreated patient with inflammatory colitis, rectal sparing or patchy rectal inflammation is generally considered a sign of Crohn's disease (CD), rather than ulcerative colitis (UC).
Methods:
The initial endoscopic rectosigmoid mucosal biopsies obtained at disease onset from 12 untreated children with UC who ultimately required surgery were blindly reviewed (randomly mixed with another 62 specimens obtained from children with CD or treated UC). Biopsies were classified as typical UC if there was diffuse, active inflammation and severe crypt destruction or distortion. Those with patchy, active inflammation and only mild crypt changes were classified as CD. Because all 12 subjects had ultimately been proven to have UC by examination of a subtotal colectomy specimen, for the purposes of this report biopsies read as either normal or CD were both considered evidence of atypical UC with rectal sparing.
Results:
Five of 12 subjects (seven biopsies) had atypical histology. Mild, patchy inflammation was seen in six rectal or sigmoid biopsies, whereas one rectal biopsy was normal. The remaining seven subjects (10 biopsies) had diffuse inflammation. Two of five subjects with atypical biopsies had an endoscopically normal rectosigmoid, one had patchy inflammation, and the remaining two had diffuse endoscopic changes. All seven subjects with typical UC histology had diffuse endoscopic changes. Subjects with atypical findings could not be differentiated by age, duration, or types of symptoms at presentation, years of disease at colectomy, or indications for colectomy.
Conclusions:
Patchy or absent inflammation of the rectum and sigmoid can be present in untreated children with UC at disease onset. Because such children may be mistakenly diagnosed as having CD, these data must be considered when treatments or clinical research protocols are designed to include children with colitis.