World Journal of Colorectal Surgery

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1 World Journal of Colorectal Surgery Volume 6, Issue Article 4 Isolated Jejunal Crohn s Disease, A Diagnostic Dilemma Elise Biesboer BS Lacey Stelle MD Michelle M. Olson MD, MACM Paul Tender MD University of Illinois College of Medicine, Urbana, Illinois, The United States Of America, ebiesbo2@uic.edu Carle Foundation Hospital, Urbana, Illinois, The United States Of America, lacey.stelle@carle.com Carle Foundation Hospital, Urbana, Illinois, The United States Of America, michelle.olson@carle.com Carle Foundation Hospital, Urbana, Illinois, The United States Of America, paul.tender@carle.com Copyright c 2017 The Berkeley Electronic Press. All rights reserved.

2 Isolated Jejunal Crohn s Disease, A Diagnostic Dilemma Elise Biesboer BS; Lacey Stelle MD; Michelle M. Olson MD, MACM; and Paul Tender MD Abstract A 50-year-old male was referred for double-balloon enteroscopy following a several month history of abdominal pain, bloating, weight loss, and post-prandial nausea with vomiting. The enteroscopy revealed erythematous, ulcerated, and friable mucosa with evidence of bleeding and mucosal necrosis in the distal duodenum and proximal jejunum. On colonoscopy, the colon and terminal ileum appeared grossly normal. The patient underwent jejunal resection with duodenojejunostomy and two stricturoplasties of the proximal jejunum. Post-operatively, the patient s symptoms resolved. The pathology, as well as serology, was consistent with Crohn s disease. The inflammatory effects of Crohn s disease are most commonly found in the ileocecal and colonic regions of the bowel. The prevalence of jejunal involvement in Crohn s disease has been estimated at 4-10%, and isolated jejunal Crohn s is even more rare at less than 1% of all cases. Jejunal involvement has been seen to be associated with an increased risk for stricturing behavior, relapse, and multiple abdominal surgeries. This case is an example of a rare presentation of isolated jejunal Crohn s disease. KEYWORDS: inflammatory bowel disease, Crohn s Disease, jejunum, small bowel

3 Biesboer et al.: Isolated Jejunal Crohn's Disease, A Diagnostic Dilemma 1 Case Report A 50-year-old male was referred to our institution for double-balloon enteroscopy following a four-month history of epigastric abdominal pain, bloating, pound weight loss, and post-prandial nausea with vomiting. Notably, the patient had normal bowel movements. The patient s physical exam was unremarkable, with stable vital signs, a soft, flat, non-tender abdomen, and no organomegaly. Laboratory findings were also mostly unremarkable, with a normal complete blood count, albumin 2.6 g/dl, potassium 5.8 meq/l, and a C-reactive protein of 3.6 mg/l. At the discretion of the patient s primary care provider, he had recently undergone an abdominal ultrasound, two CT scans of the abdomen/pelvis, and an MRI enterography. Possible jejunal intussusception was identified on these images. Double-balloon push enteroscopy revealed erythematous, ulcerated, friable mucosa with active bleeding in the proximal jejunum. There was a stenotic segment of the proximal jejunum that was unable to be traversed by the endoscope. Pathology from jejunal biopsy indicated necrotic and inflamed granulation tissue of the mucosa. The stomach was normal despite mild esophagitis without active bleeding. Perianal and digital rectal examinations were normal. The colon and terminal ileum were grossly normal. The patient underwent elective surgery the following week including jejunal resection with duodenojejunostomy and multiple small bowel stricturoplasties. The patient had a segment of small bowel extending 40 cm distal to the ligament of Treitz with multiple high-grade strictures, inflammatory thickening, and fat wrapping. The gross appearance was consistent with Crohn s disease. Proximal to this segment, the distal duodenum was significantly dilated, but otherwise appeared grossly normal. The following 30 cm distal to the grossly abnormal segment contained three short-segment strictures. Two of these strictured areas had a luminal diameter less than 2 cm, and the third allowed passage of a Foley balloon, which was measured to be greater than 2 cm in diameter. Each of these three strictures was approximately 4-6 cm in length. The remaining small bowel distal to these strictures was normal. There was no evidence of ileitis, and the colon, as well as the upper abdominal contents, were also normal. Pathology results of the jejunal resection included ulceration, acute inflammation, focal organizing thrombus in a submucosal blood vessel, transmural lymphoid aggregates, muscular hypertrophy, and serosal adhesions. The acute inflammation and focal ulceration extended to the mucosal margin. The cytomegalo virus (CMV) immunohistochemical stain was negative. No fungal organisms were found with methenamine silver stain. There were 23 lymph nodes with reactive lymphoid hyperplasia. The pathological results were consistent with Crohn s disease. Post-operatively, the patient received nasogastric tube decompression and total parenteral nutrition in anticipation of prolonged ileus. There was return of normal bowel function, and the patient was eventually advanced to a regular diet. He was discharged to home on post-operative day number eight. Currently, the patient is Produced by The Berkeley Electronic Press, 2016

4 2 World Journal of Colorectal Surgery Vol. 6, Iss. 5 [2016], Art. 4 seen regularly by a gastrointestinal physician, is considered to be in remission, and is on adalimumab for maintenance. Discussion The inflammatory effects of Crohn s disease are most often found in the ileocecal region of the bowel, and the jejunum may be involved in less than 10% of cases 1. Isolated jejunal involvement, as seen in this patient, is exceedingly rare. In adults with proximal small bowel Crohn s, sparing of the terminal ileum is uncommon 2. Similarly, Lazarev et al. found that 92% of their patients with jejunal disease had concurrent ileal disease 3. Two studies have demonstrated that isolated jejunal Crohn s disease could be found in as low as 1% of cases 4,5. The limited case reports describing isolated jejunal disease have shown that patients present with symptoms similar to this patient, including small bowel obstruction, fever, epigastric pain, weight loss, and general malaise 1, 5-7. As demonstrated with this patient, a lack of diarrhea may confuse the diagnosis 5. This is in contrast to the usual presentation of Crohn s disease, which includes diarrhea, lower abdominal pain, fatigue, and weight loss. Additionally, the patient s diagnosis was unable to be identified with imaging modalities alone, which is consistent with current literature demonstrating that jejunal lesions are not usually able to be seen on CT scan or MRI 2. Indeed, the previous case reports required endoscopy or laparotomy for diagnosis 6-8. Patients who present with jejunal lesions have also been shown to be significantly younger compared to those with lesions in other locations 8. Zalev and Prokipchuk found that in 81% of their patients with jejunal involvement, and in 100% of their patients with jejunal lesions and spared terminal ileum, Crohn s disease had developed before age Likewise, Farmer et al. found that only 22% of patients with duodenal or jejunal involvement were diagnosed after 50 years 5. Jejunal involvement has been associated with increased risk for stricture 1-3, 9. Occurrence of stricturing behavior is seen with both jejunal and ileal involvement, and an absence of colonic or anoperineal involvement 9. Approximately 70% of patients with proximal Crohn s disease had strictures 1,2, and 63% of these patients required proximal jejunal resection 1. Generally, patients who underwent resection for proximal Crohn s disease (defined as mouth through jejunum) required removal of longer segments of bowel compared to distal Crohn s disease 2. There has been shown to be a higher rate of relapse, as well as increased requirement for surgical intervention seen with jejunal involvement of the disease 3,10,11. In one study, the 3, 5, and 10-year re-operation rate for jejunal Crohn s was 43, 50, and 61% respectively, compared to 22, 30, and 51% for ileocecal Crohn s 11. Although the time from diagnosis to first operation does not seem to be significantly shorter 3, early re-operation rates for jejunal Crohn s are much higher. Cosnes et al. also indicated that the presence of jejunal lesions predisposes to early

5 Biesboer et al.: Isolated Jejunal Crohn's Disease, A Diagnostic Dilemma 3 complications, particularly of stricturing nature 9. This may suggest that jejunal disease has a more aggressive early clinical pattern. Conclusion Our patient represents an incredibly unique scenario of Crohn s disease isolated to the jejunum. Jejunal involvement in Crohn s disease is rarely seen, and isolated jejunal Crohn s disease is even more uncommon. Although he presented with symptoms similar to those in previous case reports, these symptoms do not coincide with the standard presentation of Crohn s disease. Additionally, the unusual age distribution, lack of useful imaging, and requirement for invasive testing makes isolated jejunal Crohn s disease difficult to diagnose. As seen with this patient, operative intervention is frequently required and the disease may be more aggressive than Crohn s disease in other locations. References: 1. Wagtmans MJ, Verspaget HW, Lamers CBHW, van Hozegand RA. Clinical aspects of Crohn s disease of the upper gastrointestinal tract: a comparison with distal Crohn s disease. Am J Gastroenterol. 1997; 92(9): Zalev AH, Prokipchuk EJ. Crohn s disease of the proximal small intestine: radiologic findings in 55 patients. Can Assoc Radiol J. 1992; 43(3): Lazarev M, Huang C, Bitton A, et al. Relationship between proximal Crohn s disease location and disease behavior and surgery: a cross-sectional study of the IBD Genetics Consortium. Am J Gastroenterol. 2013; 108(1): Cottone M, Brignola C, Rosselli M, et al. Relationship between site of disease and familial occurrence in Crohn s disease. Dig Dis Sci. 1997; 42(1): Farmer RG, Hawk WA, Turnbull RB Jr. Clinical patterns in Crohn s disease: a statistical study of 615 cases. Gastroenterology. 1975; 68(4): Lonardo A, Tondelli E, Selmi I, Bagni A, Della Casa G, Grisendi A. Isolated jejunal Crohn s disease in a young adult presenting as fever of unknown origin. Am J Gastroenterol. 1998; 93(11): Hapke RJ, Corless C, Katon RM. Isolated jejunal Crohn s disease in a young woman presenting with malabsorption syndrome and abdominal pain. Am J Gastroenterol. 1997; 92(10): Raju BB, Bavanandam S, Thiagarajan A, Ganapathy N. Isolated jejunal Crohn s disease in an adolescent girl. Indian J Pediatr. 2015; 83(1): Cosnes J, Cattan S, Blain A, et al. Long-term evolution of disease behavior of Crohn s disease. Inflamm Bowel Dis. 2002; 8(4): Flamant M, Trang C, Maillard O, et al. The prevalence and outcome of jejunal lesions visualized by small bowel capsule endoscopy in Crohn s disease. Inflamm Bowel Dis.2013; 19(7): Produced by The Berkeley Electronic Press, 2016

6 4 World Journal of Colorectal Surgery Vol. 6, Iss. 5 [2016], Art Keh C, Shatari T, Yamamoto T, Menon A, Clark MA, Keighley MR. Jejunal Crohn s disease is associated with a higher postoperative recurrence rate than ileocaecal Crohns disease. Colorectal Dis. 2005; 7(4):