November 2013 Case 1
A 73 year-old female with changing bowel habits
Jessica Johnson, MS4, Robert Strauch, M.D., Jeffrey Stead, M.D.
A 73 year-old Caucasian female with a history of changes in bowel habits varying between constipation and diarrhea was scheduled for sigmoid resection after abdominal CT with contrast revealed a 4 cm apple-core lesion suspicious for a cancerous mass (figure 1). The patient’s medical history was positive for coronary artery disease with past myocardial infarction treated with stent placement and a bypass graft along with a more recent history of atrial fibrillation. Other medical conditions included hypertension, hypercholesterolemia, and gastroesophageal reflux disease. Current medicines were Pradaxa, Atenolol, Aspirin, Prilosec, Nitroglycerin, and prophylactic Calcium. All chronic conditions were being managed appropriately and the patient was deemed a fair surgical candidate.
Preoperative physical exam was unremarkable with the patient’s abdomen noted to be soft, non-tender, and not distended. There was well healed lower midline scar from a previous hysterectomy and multiple healed laparoscopic incisional scars from a previous cholecystectomy. Cardiac exam revealed an irregularly regular heart rate consistent with atrial fibrillation. Recent laboratory results revealed normal CMP, TSH, and cardiac enzymes. CBC showed a mild anemia with hemoglobin and hematocrit of 12.9 and 37.2. The patient vitals were also normal.
Laparotomy revealed diverticulosis with evidence of previous perforation and narrowing of the sigmoid colon. No evidence of metastatic disease was found. The narrowed segment was excised and sent for pathological examination. The patient tolerated the procedure well and was taken to the recovery room hemodynamically stable. No post-operative complications were noted.
A segment of bowel 14.0 cm long and 2.5 cm in average diameter was received which was grossly unremarkable, aside from a few shallow diverticula. Specifically, the serosa was smooth and tan-pink, the bowel wall soft, the haustral folds neither edematous nor congested, and the moderate amount of attached fatty tissue soft and yellow. Sections of the specimen stained with Hematoxylin and Eosin (H&E) were examined. A striking finding was the presence of moderate to severe phlebitis involving many small and medium sized veins in the submucosa (figure 2). The inflammatory infiltrate consisted almost entirely of lymphocytes, with occasional plasma cells and eosinophils (figure3). Arteries were not involved by the inflammatory process (figure 4). Some veins in mesenteric fat and a few within the muscle wall of the bowel were also involved, but the inflammation was mostly less severe.
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Enterocolic lymphocytic phlebitis (ELP) is an extremely rare vasculitis of unknown etiology. For most vasculitides involving the gastrointestinal tract symptoms vary considerably without allowing classification of the underlying disease. ELP may present with a wide range of complaints including abdominal pain, nausea, vomiting, diarrhea, constipation, and/or rectal bleeding. Most commonly, ELP presents acutely with hematochezia. Often noted is a prodromal period of non-specific abdominal pain weeks to months before an acute event of lower gastrointestinal blood loss. However, patients have also presented with chronic symptoms with work-up revealing a concerning mass, like the case presented.
Most vasculitides that affect the gastrointestinal tract have multiple systemic manifestations. For instance, polyarteritis nodosa most commonly presents with glomerulonephritis and mononeuritis multiplex, along with possible gastrointestinal vasculitis. ELP, is unique in that its vasculitis has been limited to the gastrointestinal tract most often veins in the colon. Cases of ELP with isolated involvement of small intestine, appendix, gallbladder, mesentery, and stomach have also been reported.
Epidemiology of ELP is also variable. Most patients are diagnosed with ELP in their fifties and sixties but cases of patients in their twenties have been reported. No reported patient has had systemic vasculitis. Like the patient presented, most with ELP do have a history of cardiovascular disease and hypertension. Other associated medical conditions include malignancies and renal failure. No casual role has been found between ELP and these conditions. Flutamide has been hypothesized as a possible risk factor since a few patients with ELP had a history of its use. Our patient, however, did not report using flutamide.
In all reported cases, ELP was not the suspected diagnosis. Along with its vague abdominal symptom, ELP has no specific endoscopic or imaging findings. Multiple reports have shown CT findings of thickened and edematous bowel walls or masses of the GI tract that are nonspecific. For these reasons, pathological examination is required to diagnose ELP.
ELP is characterized by lymphocytic infiltration of the mesenteric veins and venules with sparing of arteries and arterioles. This is unique when compared to most vasculitides that have no predominant histopathological patterns and commonly affect both arteries and veins. Indeed, involvement of the gastrointestinal tract by other vasculitides is manifested mainly in arteries. Vasculitis in Churg-Strauss can preferentially involve veins. However, Churg-Strauss shows an eosinophilic infiltration around the vessels on pathological review along with systemic allergic symptoms not seen in ELP.
The lymphocytic infiltration that occurs in ELP results in edema and inflammation that causes venous outflow obstruction. This obstruction is probably responsible for the mass formation that is found in some patients. Inflammation contributes to another feature associated with ELP of fresh and/or organized venous thrombi. Obstruction and thrombi both cause ischemia that leads to associated symptoms of abdominal pain and bloody bowel movements. This mesenteric ischemia is the cause of a few deaths that ELP has been associated with.
ELP involves vessels mostly in the submucosa of the bowel wall. More significant changes occur among the smaller sized venules than the larger veins. The predominant type of inflammatory response has been shown to be lymphocytic phlebitis that is composed of perivenular infiltrative cuffs of lymphocytes. These “cuffs” are composed mostly of T cells that are CD3+ and/or CD8+ with rare B cells. These T cells are most likely responsible for causing vascular changes and cytotoxic effects. In addition to lymphocytes, the inflammatory infiltrate of ELP may include small numbers of plasma cells, eosinophils, epithelioid histiocytes, and multinucleated giant cells. Areas of necrotizing phlebitis and granulomatous phlebitis are sometimes present. Endothelial and myointimal hyperplasia with vascular narrowing is seen in some cases.
No reported cases of ELP have been shown to respond to medical management. Chronic presentations often lead patients to be treated for presumed inflammatory bowel disease until development of an acute abdomen prompts surgical exploration. Regardless, resection has shown to be necessary for pathological diagnosis and treatment. This is unlike other vasculitides in which surgical intervention is often non-curative. For instance, when Behҫet syndrome involves the arteries of the intestinal tract, often multiple surgical interventions are required to treat reoccurring mesenteric aneurysms. Gastrointestinal involvement by Behҫet syndrome most often affects the distal ileum and cecum with ulcerations that can mimic Crohn’s disease. There may be a predominantly lymphocytic venulitis, however, the vasculitis typically involves both arteries and veins and includes both lymphocytes and neutrophils. Diagnosis of Behҫet syndrome is made on the basis of extra-intestinal findings of oral and/or genital ulcers and iritis.
This patient’s presentation with changes in bowel habits and an apple-core lesion on CT scan is consistent with many case reports of Enterocolic Lymphocytic Phlebitis (ELP). These non-specific findings would not have prompted most clinicians to consider the extremely rare diagnosis of ELP. For this patient, resection with pathological examination allowed pathological diagnosis, and will likely prove curative. Since sigmoid resection, the patient has been asymptomatic. One year post-op, follow-up colonoscopy was conducted that showed no polyps, masses, or bleeding and was positive for only a few scattered diverticuli.
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