Volume 6, Issue 8 , Pages e31-e32, August 2008
Appendiceal Orifice Inflammation and Ulcerative Proctitis
Article Outline
A 14-year-old girl has a 2-month history of vague abdominal discomfort at the time of cheerleading and track activities. She reports hematochezia with the passage of formed to firm stools. Hemogram, erythrocyte sedimentation rate, and chemistry panel were normal. Physical examination was normal including the absence of perianal disease. Digital rectal examination yielded no palpable stool. Endoscopy revealed normal esophagus, stomach, duodenum, terminal ileum (TI), and cecum, but the appendiceal orifice was everted, edematous, and grossly inflamed with mucopus (Figure A). The rectum had asymmetric edema, erythema, and friability. The intervening colon was normal. Because of a concern for appendicitis, a computerized tomography was performed immediately after the endoscopy, which documented the partially everted appendix without appendicitis (Figure B). Biopsy specimens eventually showed no abnormalities in the TI, but biopsy specimens of the appendiceal orifice revealed acute cryptitis and moderate acute and chronic inflammation without granulomas. The rectum showed mild inflammation with cryptitis. These biopsy specimens were compatible with inflammatory bowel disease, specifically, ulcerative colitis (UC). A small-bowel contrast radiograph was normal. The inflammatory bowel disease panel did not reveal increased anti-saccharomyces cerevisiae antibody, anti-outer membrane protein core antibody, or perinuclear antineutrophil cytoplasmic antibody levels.
The patient's symptoms resolved upon treatment with oral mesalamine and rectal mesalamine suppository. Colonoscopy was performed 4 months later and no gross mucosal abnormalities were seen. Biopsy specimens of the appendiceal orifice revealed only focal, minimal acute cryptitis. TI, cecum, ascending colon, and rectal biopsy specimens were normal. The patient continues to do well on maintenance oral mesalamine during 2 years of follow-up evaluation, necessitating only 2 brief courses of rectal mesalamine for minor proctitis exacerbation.
Discussion
Isolated appendiceal orifice inflammation (AOI) in patients with UC is uncommon but is becoming increasingly recognized in adults with distal UC. AOI in UC histologically resembles the colonic disease rather than acute appendicitis.1 Discontinuous appendiceal involvement in the presence of histologically normal cecum was found in 12% of patients who underwent proctocolectomy for UC.2 The clinical significance of AOI in the setting of distal UC is unclear. An 8-year follow-up evaluation of 15 patients with left-sided UC and patchy cecal/appendiceal orifice inflammation showed that these patients experienced a more aggressive disease course with more frequent relapses compared with patients without AOI.3
A more important dilemma is the possibility of Crohn's colitis in the presence of discontinuous inflammation noted at the time of colonoscopy. The distribution of gross and microscopic inflammation in UC is diffuse and symmetric in contrast to Crohn's disease (CD) in which the inflammation may vary within the same segment of the colon. In distal UC with AOI, the severity of the inflammation in the affected distal colon showed a gradient of decreasing severity from distal toward more proximal segments, and the ileum was normal.3 Hence, the clinician can conclude that if patchy inflammatory changes are observed in a patient with chronic colitis without involvement of the cecum, the diagnosis more likely points to CD. The gastroenterologist, especially the pediatric subspecialist, should be aware of the finding of isolated AOI in conjunction with distal UC. Recognition of this association allays the suspicion for CD and is essential for initiation of proper anti-inflammatory therapy while avoiding appendectomy.
The author's sincere appreciation goes to Dr Jean Molleston for her tireless mentoring. The author is grateful to Dr Kevin Kernek and Dr Kimberly Applegate for providing the histologic and radiographic illustrations. Finally, the author is indebted to Ms Vicki Haviland-Wilhite for her help in the preparation of this article.
References
- Appendiceal inflammation in ulcerative colitis. Histopathology. 1998;33:168–173
- Discontinuous appendiceal involvement in ulcerative colitis: pathology and clinical correlation. J Gastrointest Surg. 1999;3:141–144
- Patchy cecal inflammation associated with distal ulcerative colitis: a prospective endoscopic study. Am J Gastroenterol. 1997;92:1275–1279
PII: S1542-3565(08)00431-X
doi:10.1016/j.cgh.2008.04.029
© 2008 AGA Institute. Published by Elsevier Inc. All rights reserved.
Volume 6, Issue 8 , Pages e31-e32, August 2008
