Monday, September 29, 2008
Vasculitis and Acute Cecal Infarct
A 27 year old female presented with 24 hours of severe, acute right lower quadrant abdominal pain. She had a white count, focal RLQ peritoneal signs and she simply looked ill. The CT scan was read: "peri-cecal inflammatory changes and thickened appendix, all consistent with acute appendicitis." Again, she was 27 and had no known other medical conditions. It seemed like yet another clear-cut case of appendicitis. So we booked her for a presumed laparoscopic appendectomy.
Once inside, it immediately became obvious that the intial diagnosis was incorrect. The appendix was normal; pale-pink and supple and striated with healthy blood vessels. Her cecum, however, appeared to have sustained some sort of hemorrhagic infarct; bright red, edematous, almost like a smashed tomato. So I switched gears and performed the ileocecectomy laparoscopically. At the top of my list was Crohn's disease or typhlitis (cecal inflammation usually seen in immunocompromised patients). The final pathology surprised me; severe mesenteric vasculitis with segmental colonic infarcts.
Vasculitis is a general term to describe a multitude of disorders characterized by an autoimmune-mediated inflammation of blood vessels. Basically, the body attacks the proteins in our vascular system and causes destruction, thrombosis, and ultimately tissue death of the supplied organ. Here's a good link that reviews systemic vasculitides.
Examples of systemic vasculitides include lupus, Wegener's granulomatosis, polyarteritis nodosa, and Takayasu's arteritis. Rarely, there can be gastrointestinal manifestations. In this case, the patient did well after the resection. She resumed a regular diet and went home by post op day #3. But she still has an elevated CRP and ESR and there was evidence of vasculitis at the resection margins. Rheumatology is involved and and the autoimmune work-up is pending. My concern is that the segmental, systemic nature of these diseases puts her at risk for future events.
If anyone has any ideas/experience with this, please let me know.