Case Presentation of amyloidosis of the gastrointestinal tract with CT enterography

Transcription

1 Case Presentation of amyloidosis of the gastrointestinal tract with CT enterography Poster No.: C-1406 Congress: ECR 2015 Type: Educational Exhibit Authors: Y. C. Ho, O. S. H. Lo, W. L. Law, W. W. M. Lam; Hong Kong/CN Keywords: Pathology, Diagnostic procedure, CT, Small bowel DOI: /ecr2015/C-1406 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 12

2 Learning objectives Describe the imaging findings of gastrointestinal amyloidosis in CT enterography. Background Amyloidosis is an uncommon heterogeneous group of diseases caused by extracellular deposition of the amyloid protein. The amyloid deposits demonstrate apple-green birefringence under a polarized light microscope after staining with Congo red, and presence of rigid and non-branching fibrils of nm diameters on electron microscopy. Amyloidosis can be classified into systemic or localized forms. The gastrointestinal tract (GIT) is the commonest site of involvement (98%) of systemic amyloidosis in both primary and secondary forms. Of which, small bowel has the highest prevalence of involvement. Amyloid deposition in the muscularis, Auerbach plexus and arterioles contributes to the dysmotility and ischemia of GIT. The clinical symptoms are non-specific and causing challenge the physician's diagnostic capability. Imaging hence plays a key role in investigation and establishing the correct diagnosis. Radiological findings of amyloidosis in barium studies have been well described. These include symmetrical fold thickenings, jejunalization of ileum, small polypoid protrusions, fine granular densities and amyloid tumor. CT enterography is a new non-invasive imaging technique which provides superior small bowel visualization compared with standard abdomino-pelvic CT. The simultaneous assessment of extramucosal areas is clearly advantageous to conventional barium studies. It also provides complementary diagnostic information to capsule endoscopy. Last but not the least; CT enterography is well tolerated by patients. Therefore, it is emerging as the modality of choice in imaging small bowel. Reports of CT enterographic findings on amyloidosis are however scarce. Findings and procedure details Page 2 of 12

3 We reported a 56-year-old lady presented with poor appetite and weight loss. Upper endoscopy revealed gastritis and erosions and colonoscopy revealed colonic diverticula. PET-CT revealed small amount of ascites and no other significant findings. She was latter presented for abdominal pain and distension. Intestinal obstruction was diagnosed and laparotomy was performed with intraoperative finding of adhesive intestinal obstruction. In view of persistent hypoalbuminemia, nephrologist was consulted for suspected nephrotic syndrome. Subsequently she was found to have paraproteinuria and light chain myeloma with amyloidosis was confirmed. Rectal biopsy confirmed amyloid deposition by the increase in submucosal extracellular eosinophilic material and positive Congo Red staining. CT enterography was requested for assessment. The study was performed according to departmental protocol. After fasting for 6 hours, the patient was asked to drink 1.5L of PEG 45 minutes before the scan in 6 divided doses. 20 mg of hyoscine butylbromide was administered intravenously immediately prior to scanning. Post-contrast scans of the abdomen and pelvis were obtained at 38s after injection of 120 ml of Omnipaque 300 at -1 4 ml s via an 18-guage cannula. The scanner used being 320-MDCT scanner (Aquilion One Toshiba Systems). The slice thickness was 2mm, with reconstruction interval of 0.5mm for reformatted images. On reviewing the CT enterography, diffuse dilated small bowel loops with symmetrical and smoothly thickened walls were noted. (Fig. 1, 2) Suboptimal mucosal enhancement with no mural stratification was present. (Fig. 3) Such finding was useful to distinguish it from inflammatory enteritis. There was also evidence of ileus and impaired motility as suggested by the sedimentation and retention of the bowel contents. (Fig. 4) Marked diffuse mesenteric infiltration was also present, which was a feature not commonly seen at inflammatory enteritis. (Fig. 5) Prominent mesenteric lymph nodes of reactive morphology were also noted. (Fig.6) No focal mass or pseudoaneurysmal dilatation was seen associated with the small bowel loops. No intussusception or perforation was noted. Additional finding of mural thickening of descending colon with thickening most apparent at the submucosal layer with halo appearance was also noted. (Fig 7) Findings were compatible with intestinal amyloidosis. Images for this section: Page 3 of 12

4 Page 4 of 12

5 Fig. 1: Coronal reformatted CT enterography image showing diffuse small bowel dilatation and mural thickening (arrow). Fig. 2: Coronal reformatted CT enterography image showing symmetrical and smoothly thickened mucosal folds (arrow). Page 5 of 12

6 Fig. 3: Axial CT enterography image showing suboptimal mucosal enhancement with no mural stratification (arrow). Page 6 of 12

7 Fig. 4: Axial CT enterography image showing sedimentation of small bowel contents (arrow). Page 7 of 12

8 Fig. 5: Axial CT enterography image showing marked and diffuse mesenteric infiltration (arrow). Page 8 of 12

9 Fig. 6: Coronal reformatted CT enterography image showing multiple prominent mesenteric lymph nodes (arrow). Page 9 of 12

10 Fig. 7: Axial CT enterography image showing thickened wall of descending colon with halo appearance (arrow). Page 10 of 12

11 Conclusion Although no pathognomonic imaging feature of gastrointestinal amyloidosis from CT enterography was detected, the advantage of assessment of mucosal and extramucosal disease and consociation of findings allow radiologists to make the correct diagnosis in proper clinical settings. Personal information 1. Yuen Chi Ho, Department of Radiology, Queen Mary Hospital, Hong Kong 2. Oswens Siu Hung Lo, Department of Surgery, The University of Hong Kong, Hong Kong 3. Wai Lun Law, Department of Surgery, The University of Hong Kong, Hong Kong 4. Wendy Wai Man Lam, Department of Radiology, Queen Mary Hospital, Hong Kong References 1. Merlini G, Bellotti V: Molecular mechanisms of amyloidosis. NEngl JMed 349: , Loizos S, Chrysie T S, Christos G S. Amyloidosis: Review and Imaging Findings. Semin Ultrasound CT MR 2014 Jun; 35(3): Kim S H, Han J K, Lee K H, Won HJ, Kim K W, Kim J S, Park C H, Choi B I. Abdominal Amyloidosis: Spectrum of Radiological Findings. Clinical Radiology : Page 11 of 12

12 4. Kala Z, V alek V, Kysela P. Amyloidosis of the small intestine. European Journal of Radiology 63 (2007) Mainenti P P, Segreto S, Mancini M, Rispo A, Cozzolino I, Masone S, Rinaldi CR, Nardone G, Salvatore M. Intestinal amyloidosis: Two cases with different patterns of clinical and imaging presentation. World J Gastroenterol 2010 May 28; 16(20): Cowan A J, Skinner M, Seldin D C, Berk J L, Lichtenstein D R, O'Hara C J, Doros G, Sanchorawala V. Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience.haematologica 2013; 98(1) Page 12 of 12