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.


Bongi said...

i once saw a patient with polycytemia vera who infarcted more than half her small bowel. she survived the operation and was discharged. she developed dvt of her arm on warfarin!!! she was pretty serious about clotting. but hers wasn't a vasculitis. hope the rheumatology boys can help.

rlbates said...

Tough case. I don't have anything to add. Like, Bongi, I hope the Rheumatology guys can find something.

Bongi said...

operated a query appendicitis that turned out to be a tiflitis. did a right hemi. turned out to be crohns. actually the whole thing still sits badly with me because the whole family went crazy in the end as if i caused the crohns with my omnipotence.

Anonymous said...

I have Crohns, been in remission for a long while... I just turned 22, I have to watch myself because around older folk when they start talking about colonoscopies I start telling stories, then they look at me like "WTF?"

I will tell you, though, that a strict diet (mainly watching out for a severe gluten allergy), along with high doses of Omega-3 fatty acids ( 10G/day ) has let my GI Doc (Great guy, btw...first one was terrible) give me the all clear to stop all meds

*God how I hated Prednisone*

Well, I look out for a few foods that throw me.

Chocolate/Egg Whites/A lot of Coffee/to much dairy.

^^^That's actually a pretty common list

I'd like to help in any way I can if it is Crohns, it took me a while to figure out how to live with it, especially when my first flare up was in 9th grade, simple tricks that I picked up along the way.

Most are way to embarrassing to post here.

rlbates said...

Any update on what the cause turned out to be?

Jeffrey Parks MD FACS said...

Looks like polyarteritis nodosa (PAN)

Unknown said...

I am a pathologist at a small community hospital in Evanston IL and I have a case exactly like this on my desk right! The patient is an otherwise healthy non-drug abusing 25 year old male who presented exactly the same way and has a mesenteric vasculitis with segmental infarction of the cecum. It is not inflammatory bowel disease. We are having a rheumatologist see the patient now. Any chance I could get a look at the path slides on your case? I'd be interested.