Progressive Primary Appendiceal Crohn’s Disease in a 21-Year-old Female

Crohn’s disease is an inflammatory bowel disorder that can affect any portion of the gastrointestinal tract, most commonly the terminal ileum near the ileocecal valve. Crohn’s disease can be characterized by transmural inflammation and deep fissuring ulcers that predispose to fistula formation and “skip” lesions separated by normal segments of bowel. While often affecting the terminal ileum near the ileocecal valve, Crohn’s disease presenting primarily in the appendix is a rare entity. In part due to its low prevalence, cases of appendiceal Crohn’s disease can be confused for acute, non-Crohn’s-related appendicitis on initial presentation. Although there are published cases of primary appendiceal Crohn’s disease in the medical literature, in most cases the diagnosis is made retrospectively following appendectomy for presumed appendicitis. We report on a case of Crohn’s disease that was diagnosed pre-operatively, primarily involved the appendix, and which progressed radiographically despite medical therapy and resolution of clinical symptoms. Unique management issues related to this case include the appropriateness of systemic therapy for disease isolated to the appendix, an inability to endoscopically obtain tissue for a definitive diagnosis, and the decision to proceed with surgery in an asymptomatic patient with progressive disease on imaging. Intraoperatively, the appendix was severely inflamed and densely adherent to the left pelvic side wall and adjacent to the left ovary and fallopian tube. A laparoscopic appendectomy was performed. Pathology demonstrated acute appendicitis as well as marked mural chronic inflammation and epithelioid granulomas, consistent with Crohn’s disease. Surgical resection may be the most appropriate treatment for Crohn’s disease primarily involving the appendix, obviating the need for systemic therapy and minimizing the risk for appendiceal perforation and fistula formation.