Case Presentation An Unusual Case Presentation An Unusual Cause of - - PowerPoint PPT Presentation

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 pain of acute onset

RLQ abdominal pain of acute onset

• Accompanied by nausea, without vomiting

PE+Lab PE+Lab

• Hemodynamically stable
• Marked RLQ tenderness with signs of

i l i i i d i i b d peritoneal irritation and positive rebound test

• Leukocytosis (15.6) with neutrophilia (79.4%)

Abdominal US Abdominal US

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

Pathology Findings Pathology Findings

• Gross examination of the surgical specimen
• Gross examination of the surgical specimen

revealed a thickened cecal wall and ulceration f l

• f cecal mucosa

Possible Diagnosis? Possible Diagnosis?

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

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

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

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

• rganized thrombosis of these veins
• rganized thrombosis of these veins
• Arteries are not involved in the inflammatory

process process

ELP Histologic DD ELP – Histologic DD

• Hypersensitivity reaction
• Henoch‐Schonlein Purpura
• SLE

SLE

• Behcet’s disease

ELP Histologic DD ELP – 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

ELP Epidemiology ELP – Epidemiology

• Wide range of ages (16‐78), although most

frequently it presents at age >50 years q y p g y F l d i

• Female predominance

ELP Clinical Features ELP – Clinical Features

• ELP can present as:
• Acute abdomen (may mimic acute

di i i ) appendicitis)

• Tumor‐like mass
• GI bleeding

GI bleeding

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

ELP of Cecum At Colonoscopy ELP of Cecum – At Colonoscopy

ELP Treatment and Prognosis ELP – 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

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

S l h l h b ff f

• Several months later he began to suffer from

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

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

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 P i d ll i

• Patient reported excellent symptomatic

response

• Steroids were discontinued

C ti t l l i d t

• Continues to use oral mesalamine and to

function well

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

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%) I f i f IBD i h di i

• In fewer specimens of IBD without diversion

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
• r diversion colitis

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

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

Summary Summary

• ELP is a very rare disease
• Characteristic histologic appearance of a

d i l l h i i fil i d predominantly lymphocytic infiltration and thrombosis of intramural mesenteric venules, without arterial involvement

• Can present as a tumor‐like mass, GI bleeding
• r acute abdomen

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

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