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Case Presentation An Unusual Case Presentation An Unusual Cause of RLQ Abdominal Pain Q Itai Ghersin Itai Ghersin Rambam Health Care Campus Medical History Medical History 37 year old male Previously healthy RLQ abdominal


  1. Case Presentation – An Unusual Case Presentation – An Unusual Cause of RLQ Abdominal Pain Q Itai Ghersin Itai Ghersin Rambam Health Care Campus

  2. Medical History Medical History • 37 year old male • Previously healthy • RLQ abdominal pain of acute onset RLQ abdominal pain of acute onset • Accompanied by nausea, without vomiting

  3. PE+Lab PE+Lab • Hemodynamically stable • Marked RLQ tenderness with signs of peritoneal irritation and positive rebound test i l i i i d i i b d • Leukocytosis (15.6) with neutrophilia (79.4%)

  4. Abdominal US Abdominal US

  5. At Surgery At Surgery • It was decided to proceed immediately to exploratory laparotomy • At surgery a cecal mass was found near the ileocecal valve • The appendix was normal • Due to high clinical suspicion of malignancy a • Due to high clinical suspicion of malignancy a right hemicolectomy was performed

  6. Pathology Findings Pathology Findings • Gross examination of the surgical specimen • Gross examination of the surgical specimen revealed a thickened cecal wall and ulceration of cecal mucosa f l

  7. Possible Diagnosis? Possible Diagnosis?

  8. Case # 40747/09 H&E (x 10) (Cecum, surgical specimen) Lymphocytic phlebitis in the p muscularis propria

  9. Enterocolic Lymphocytic Phlebitis Enterocolic Lymphocytic Phlebitis • ELP is a very rare disease involving venules of the large intestine and ,less frequently, of the g , q y, small bowel, gallbladder and omentum • The term was coined by Saraga and Costa in • The term was coined by Saraga and Costa in 1989 • Similar cases have been reported earlier as mesenteric inflammatory veno ‐ occlusive mesenteric inflammatory veno occlusive disease

  10. Enterocolic Lymphocytic Phlebitis Enterocolic Lymphocytic Phlebitis • Saraga and Costa initially suspected an association between the disease and the drug g Rutoside • This association, however, has not been found in other reported cases, leaving disease etiology largely unknown etiology largely unknown

  11. ELP Histology ELP ‐ Histology • Typical histologic features include a predominantly lymphocytic infiltration of p y y p y intramural mesenteric veins and fresh and/or organized thrombosis of these veins organized thrombosis of these veins • Arteries are not involved in the inflammatory process process

  12. ELP ELP – Histologic DD Histologic DD • Hypersensitivity reaction • Henoch ‐ Schonlein Purpura • SLE SLE • Behcet’s disease

  13. ELP ELP – Histologic DD Histologic DD • These entities can be ruled out by: • Lack of arterial involvement • Lack of neutrophils and/or eosinophils in the Lack of neutrophils and/or eosinophils in the vascular lesion • Absence of clinical or lab evidence of systemic Absence of clinical or lab evidence of systemic vasculitis

  14. ELP ELP – Epidemiology Epidemiology • Wide range of ages (16 ‐ 78), although most frequently it presents at age >50 years q y p g y • Female predominance F l d i

  15. ELP ELP – Clinical Features Clinical Features • ELP can present as: • Acute abdomen (may mimic acute appendicitis) di i i ) • Tumor ‐ like mass • GI bleeding GI bleeding

  16. ELP Diagnosis ELP ‐ Diagnosis • The diagnosis is established after surgical resection and histologic examination of the g surgical specimen • Endoscopic biopsies are inadequate for diagnosis, since the pathological hallmarks of ELP are usually undetectable because of their ELP are usually undetectable because of their deep intramural location

  17. ELP of Cecum ELP of Cecum – At Colonoscopy At Colonoscopy

  18. ELP ELP – Treatment and Prognosis Treatment and Prognosis • Treatment is local excision of affected bowel • Data on conservative treatment have not been published • The disease seems to be self limited after • The disease seems to be self ‐ limited after resection • Almost all cases described up to now had an uneventful course following surgical therapy, even in cases with positive surgical margins • Only one case of recurrent ELP, necessitating a Only one case of recurrent ELP, necessitating a second surgical resection, has been reported

  19. Back to our patient Back to our patient • In contrast to most reported cases, our patient had a very eventful post ‐ surgical course y p g • Initially asymptomatic after surgery • Several months later he began to suffer from S l h l h b ff f abdominal pain, which persisted on an almost daily basis for two years • Multiple episodes of diarrhea and rectal • Multiple episodes of diarrhea and rectal bleeding

  20. Back to our patient Back to our patient • Patient was admitted several times to our Patient was admitted several times to our institution • Has undergone several GI endoscopies which have not yielded a clear ‐ cut diagnosis • Most recently admitted for evaluation of rectal bleeding rectal bleeding • Colonoscopy revealed moderate to severe continuous proctitis up to 10 cm from anus • Histology ‐ severe chronic active IBD type Histology severe chronic active IBD type colitis

  21. Back to Our Patient Back to Our Patient • A diagnosis of ulcerative colitis was made • Daily local therapy with mesalamine and Daily local therapy with mesalamine and hydrocortisone was started • Patient reported excellent symptomatic P i d ll i response • Steroids were discontinued • Continues to use oral mesalamine and to C ti t l l i d t function well

  22. ELP and IBD ELP and IBD • A recent study examined the occurrence of ELP as an incidental finding in surgical g g specimens from patients with diversion colitis and IBD and IBD

  23. ELP and IBD ELP and IBD • ELP was present: • In the majority of IBD cases with diversion In the majority of IBD cases with diversion colitis (21/29=72%) • In fewer specimens of IBD without diversion I f i f IBD i h di i colitis (8/63=12%) and of diversion colitis without IBD (1/6=16%) • Only in 1 of 100 colectomy cases without IBD • Only in 1 of 100 colectomy cases without IBD or diversion colitis

  24. ELP and IBD ELP and IBD • The authors have hypothesized that the combination of altered bowel flora (due to ( diversion colitis) and immune dysregulation (as in IBD) may have a synergistic or additive (as in IBD) may have a synergistic or additive effect on the development of ELP

  25. ELP and IBD ELP and IBD • To the best of our knowledge a case in which IBD developed following surgical resection of p g g symptomatic ELP has not been described until now in English medical literature now in English medical literature

  26. Summary Summary • ELP is a very rare disease • Characteristic histologic appearance of a predominantly lymphocytic infiltration and d i l l h i i fil i d thrombosis of intramural mesenteric venules, without arterial involvement • Can present as a tumor ‐ like mass, GI bleeding or acute abdomen

  27. Summary Summary • Treatment is local excision of the affected bowel • Almost all cases described up to now had an uneventful course following surgical therapy uneventful course following surgical therapy • An association between ELP and IBD, as demonstrated in our case, has been suggested in a study of surgical specimens from IBD in a study of surgical specimens from IBD patients, but was not noted in clinical practice until now until now

  28. Summary Summary • As the etiology of IBD remains largely unknown, this association between IBD and , ELP might provide some insight into the possible role of vascular lesions in the possible role of vascular lesions in the development of IBD • More research and clinical experience is More research and clinical experience is needed in order to determine the exact relationship between these two entities relationship between these two entities

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