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Shivani Priyadarshni Gillian Lieberman, MD 05/26/2015 Atlas of Signs and Findings in Crohn’s Disease Shivani Priyadarshni, Kasturba Medical College, India 4th Year Medical Student Gillian Lieberman, MD. Shivani Priyadarshni Gillian Lieberman, MD Outline 1. Our Patient’s Clinical Features: History and Physical Exam 2. Differential Diagnosis 3. Investigations of our patient 4. Crohn’s Disease i. Clinical Manifestations ii. Extraintestinal Manifestations iii. Diagnostic Tests iv. Radiological Findings v. Crohn’s vs Ulcerative colitis 5. Conclusion 6. Summary 2 Shivani Priyadarshni Gillian Lieberman, MD History A 55 yo F with • 2-3 loose-formed nonbloody bowel movements a day • Fleeting cramps prior to bowel movements • Came for follow up • No fever, chills, nausea, vomiting • No loss of appetite or weight change 3 Shivani Priyadarshni Gillian Lieberman, MD Past History • PMH: – Crohn’s Disease – GERD • PSH and Family History - Not significant for GI problems • Social History: Smoked 2 PPD for 30 years, stopped 10 years ago 4 Shivani Priyadarshni Gillian Lieberman, MD Physical Exam • General: Well-developed, well-nourished female in no apparent distress • Vital Signs WNL • HEENT: Unremarkable • Neck: Supple, no lymphadenopathy • Abdomen: Soft, mild tender below umbilicus without guarding or rebound 5 Shivani Priyadarshni Gillian Lieberman, MD Differential Diagnosis • • • • • • Crohn’s Disease Ulcerative Colitis Irritable Bowel Syndrome Yersinia Ileitis Ileocaecal Tuberculosis Mesenteric Adenitis 6 Shivani Priyadarshni Gillian Lieberman, MD Investigations of Our Patient • CT scan done outside, 5 years ago, showed inflammation in the proximal transverse colon, with focal microperforations and some abnormal thickening of the terminal ileum • A repeat CT scan showed an ileocecal fistula • Colonoscopy, 5 years back, showed a single aphthous erosion in the terminal ileum and an area in the transverse colon that looked like a probable fistula site 7 Shivani Priyadarshni Gillian Lieberman, MD Our Patient Past Coronal C+ T2 Weighted MR Enterography Image Terminal ileum showing mural thickening and BIDMC: PACS 8 mucosal enhancement Shivani Priyadarshni Gillian Lieberman, MD Let us view another image of the same study. 9 Shivani Priyadarshni Gillian Lieberman, MD Our Patient Past Coronal C+ T2 Weighted MR Enterography Image Abnormal inflammatory process between the terminal ileum and BIDMC: PACS proximal transverse colon 10 Shivani Priyadarshni Gillian Lieberman, MD Let us move on to the report of the same study. 11 Shivani Priyadarshni Gillian Lieberman, MD Our Patient Past C+ MR Enterography • Abnormal wall thickening, mucosal enhancement and surrounding mesenteric inflammatory changes of the terminal ileum, consistent with terminal ileitis • Abnormal inflammatory process between the terminal ileum and proximal transverse colon without fluid collection or discrete tract, suggest early changes of fistulization or may represent changes related to recent perforation • No lymphadenopathy, no evidence of abscess or 12 ascites Shivani Priyadarshni Gillian Lieberman, MD Labs on Present Admission • Hb - 13.8 • ESR -14 • CRP - 1.2 Hb, ESR and CRP are normal suggesting that there is no active disease. 13 Shivani Priyadarshni Gillian Lieberman, MD Coronal C+ CT Enterography in Our Patient Focal tethering of terminal ileum against proximal transverse colon Mild active inflammation and ulcerations in terminal ileum BIDMC: PACS 14 Shivani Priyadarshni Gillian Lieberman, MD Let us view another image of the same study. 15 Shivani Priyadarshni Gillian Lieberman, MD Coronal C+ CT Enterography in Our Patient Skip lesion proximal to the previous segment BIDMC: PACS 16 Shivani Priyadarshni Gillian Lieberman, MD Let us move on to the report of the same study. 17 Shivani Priyadarshni Gillian Lieberman, MD C+ CT Enterography in Our Patient • Terminal ileum demonstrating mild active inflammation and ulcerations, with focal tethering against the adjacent proximal transverse colon without patent fistula, similar in configuration to the past MR enterography. Tiny skip lesion just proximal to this segment appears new • No new fistula or fluid collection • No obstruction • No lymphadenopathy or ascites 18 Shivani Priyadarshni Gillian Lieberman, MD Crohn’s Disease • Type of inflammatory bowel disease (IBD) • Etiology: Unknown, Possible environmental, genetic and autoimmune factors • Involvement: any segment from mouth to perianal region • Distal ileum - most common 19 Shivani Priyadarshni Gillian Lieberman, MD Clinical Manifestations LOCATION SYMPTOMS COMMON DIAGNOSTIC TESTING Ileum and colon Diarrhea, cramping, abdominal Colonoscopy with ileoscopy, CT enterography, pain, weight loss biopsy Diarrhea, rectal bleeding, Colonoscopy with ileoscopy, CT enterography, perirectal abscess, fistula, biopsy Colon only perirectal ulcer Small bowel only Diarrhea, cramping, abdominal Colonoscopy with ileoscopy, CT enterography, pain, weight loss capsule endoscopy, small bowel follow-through, enteroscopy, biopsy, MR enterography Gastroduodenal Anorexia, weight loss, nausea, Esophagogastroduodenoscopy, small bowel region vomiting follow-through, enteroscopy Wilkins T, Jarvis K, Patel J.(2011). American Family Physician.84(12). 20 Shivani Priyadarshni Gillian Lieberman, MD Extraintestinal Manifestations • Dermatological • Rheumatological - Migratory polyarthritis, Ankylosing spondylitis • Ocular - Conjunctivitis, Anterior uveitis, Episcleritis • Urological - Nephrolithiasis • Hepatobiliary - Cholelithiasis, Hepatic steatosis, Primary sclerosing cholangitis • Metabolic bone disorder - Osteoporosis, osteonecrosis, pathological fracture • Venous and arterial thrombosis 21 Shivani Priyadarshni Gillian Lieberman, MD Let us view some images of the same. 22 Shivani Priyadarshni Gillian Lieberman, MD Extraintestinal Manifestations Erythema nodosum Schwartz and Nervi. Am Fam Physician. 2007. Superficial erosion of tongue Sanderson, et al. Inflamm Bowel Dis. 2005. Pyoderma gangrenosum Brooklyn, et al. BMJ. 2006. Anterior uveitis Mintz, et al. Inflammatory bowel diseases. 2004. 23 Shivani Priyadarshni Gillian Lieberman, MD Various Diagnostic Tests for Crohn's Disease TEST COMMENT Small bowel follow- Visualization of lumen using contrast medium (barium) through/ enteroclysis/ enema Computed tomography enterography Magnetic resonance enterography radiation exposure, no wall and extraluminal visualization Permits visualization of the bowel wall and lumen;extraluminal sequelae exposes patient to ionizing radiation. Similar to CT, no ionizing radiation expensive Endoscopy Direct visualization of mucosa - inflammation, fistula, or stricture of terminal ileum and colon; ability to obtain biopsies. extraluminal not seen. Ultrasonography Detects increase in vascular flow, abscess, sinus tracts, and lymphadenopathy operator dependant, obesity Wilkins T, Jarvis K, Patel J.(2011). American Family Physician.84(12). 24 Shivani Priyadarshni Gillian Lieberman, MD Barium Follow Through Image of Companion Patient #1 Long narrow ileum with mucosal irregularity Koh, D. M., et al. (2001). American Journal of Roentgenology. 177(6) . 25 Shivani Priyadarshni Gillian Lieberman, MD Barium Enema Image of Companion Patient #2 String sign - severe narrowing of terminal ileum with dilated proximal bowel Wells, C.(1952). Annals of the Royal College of Surgeons of England .11(2). 26 Shivani Priyadarshni Gillian Lieberman, MD Axial C+ CT Abdomen of Companion Patient #3 Target sign Periintestinal fat showing marked inflammatory change Gore, R.M., et al.(1996). American journal of roentgenology. 167(1). 27 Shivani Priyadarshni Gillian Lieberman, MD Target Sign Seen on contrast enhanced CT and MRI. It consists of 3 concentric circles of bowel wall: • Outer Layer: Inflamed muscularis propria (high attenuation) • Middle Layer: Intermediate edema/fat (low attenuation) • Inner Layer: Inflamed mucosa (high attenuation) Best seen during late arterial, early venous phase. 28 Shivani Priyadarshni Gillian Lieberman, MD Axial C+ CT Abdomen of Companion Patient #4 A D Luminal narrowing and mural thickening of distal ileum Dilatation of fluid-filled small bowel proximally due to obstruction. Collapsed ascending colon (A) and descending colon (D) Gore, R.M., et al.(1996). American journal of roentgenology. 167(1). 29 Shivani Priyadarshni Gillian Lieberman, MD Axial C+ CT Abdomen of Companion Patient #5 * * Creeping fat of mesentery Homogeneously thickened walls of ileum and ascending colon Separation of normal small-bowel loop from these diseased segments caused by abnormal mesenteric fat Gore, R.M., et al.(1996). American journal of roentgenology. 167(1). 30 Shivani Priyadarshni Gillian Lieberman, MD Creeping Fat of Mesentery Fibrofatty proliferation with hypertrophied mesenteric fat between inflamed intestinal segments. 31 Shivani Priyadarshni Gillian Lieberman, MD Axial C- CT Pelvis of Companion Patient #6 and Patient #7 R * Perirectal abscesses with sinus tract extending into right buttock. * * Presacral abscess attributable to fistula from rectum (R). Gore, R.M., et al.(1996). American journal of roentgenology. 167(1). 32 Shivani Priyadarshni Gillian Lieberman, MD Coronal C+ MRI of Companion Patient #8 Mucosal enhancement with narrowing of lumen of terminal ileum Dilation of proximal bowel Albert, J.G., et al.(2005). Gut. 54(12). 33 Shivani Priyadarshni Gillian Lieberman, MD Coronal C+MRE(a) and Conventional Enteroclysis(b) in Companion Patient #9 Terminal ileum shows Two aphthous ulcers with Wall thickening and cobblestoning. Gourtsoyiannis, et al. (2006). European radiology.16(9). 34 Shivani Priyadarshni Gillian Lieberman, MD Coronal T1 Fat Suppressed C+ MRI of Companion Patient #10 Comb sign J. Panés, et al. (2011). Aliment Pharmacol Ther. 34(2). 35 Shivani Priyadarshni Gillian Lieberman, MD Comb Sign Shaft of comb - Mural hyper enhancement and thickening in the distal ileum Bristles of comb - Prominent engorged vasa recta in the mesentery 36 Shivani Priyadarshni Gillian Lieberman, MD Axial 3D FLASH C+ MRI of Companion Patient #11 Mesenteric lymphadenopathy Gourtsoyiannis, et al. (2006). European radiology. 16(9). 37 Shivani Priyadarshni Gillian Lieberman, MD Mesenteric Lymphadenopathy Mesenteric lymphadenopathy <1 cm may be seen in Crohn’s disease. If >1 cm, then rule out other causes, especially lymphoma. 38 Shivani Priyadarshni Gillian Lieberman, MD Crohn’s vs Ulcerative Features CD UC Mucosal Granularity + ++ Aphthoid ulcers ++ - Deep ulceration ++ - Discontinuous ulceration/ Skip lesion ++ - Rectal sparing + - Colonic shortening + ++ Halligan and Robinson.(2003). In Sutton, D. (Ed.), A Textbook of Radiology and Imaging, Volume 1. 39 7th edition. Shivani Priyadarshni Gillian Lieberman, MD Let us see some more features that differentiate the two diseases. 40 Shivani Priyadarshni Gillian Lieberman, MD Crohn’s vs Ulcerative CD UC Haustral obliteration + ++ Pseudodiverticula ++ - Enteric fistulae ++ - Abscess ++ - Small bowel disease/ Anal disease ++ - Toxic megacolon + ++ Halligan and Robinson.(2003). In Sutton, D. (Ed.), A Textbook of Radiology and Imaging, Volume 1. 7th edition. 41 Shivani Priyadarshni Gillian Lieberman, MD Conclusion Based on the clinical features and radiological findings of our patient, a diagnosis of Crohn’s disease was arrived upon. Patient counselled and does not want to take medications, but is willing for follow up. Plan of follow up: - ESR, CRP - Repeat CT Enterography 42 Shivani Priyadarshni Gillian Lieberman, MD Summary • Crohn’s disease is a type of IBD which can affect any part of GIT, most commonly ileum • Clinical features include abdominal pain, diarrhea, weight loss, abscess, fistula, etc • Extraintestinal manifestations may also be present as already mentioned • Various investigations for evaluation include small bowel follow through, enteroclysis, enema with barium contrast, CT enterography, MR enterography, endoscopy and ultrasonography 43 Shivani Priyadarshni Gillian Lieberman, MD Summary Radiological findings include: ● Aphthous ulcers, eccentric bowel wall thickening ● Skip lesions ● String sign, creeping fat of mesentery ● Target sign ● Comb sign ● Abscesses and Fistulae 44 Shivani Priyadarshni Gillian Lieberman, MD Summary Treatment: ● Symptomatic OR to induce remission ● Medical ○ Steroids ○ 5-ASA derivatives ○ Immunomodulators ● Surgical 45 Shivani Priyadarshni Gillian Lieberman, MD References 1. Lichtenstein, G.R., Hanauer, S.B., Sandborn, W.J.(2009). Management of Crohn's disease in adults. Am J Gastroenterol.104(2): 465-83. Available from: http://www.ncbi.nlm.nih.gov/pubmed/19174807. [Accessed: 22 May 2015]. 2. Friedman, S., Blumberg, R.S. (2012). Chapter 295: Inflammatory Bowel Disease. In Longo, D.L. et al(eds.). Harrison’s Principles Of Internal Medicine, Volume 2. 18th edition. New York: McGraw-Hill Medical, 2477-2495. 3. Stange, E. F., et al.(2008). European evidence-based consensus on the diagnosis and management of ulcerative colitis: definitions and diagnosis. Journal of Crohn's and Colitis. 2(1): 1-23. Available from: http://www.sciencedirect.com/science/article/pii/S187399460700075X. [Accessed: 22 May 2015]. 4. Wilkins T, Jarvis K, Patel J.(2011). Diagnosis and management of Crohn's disease. American Family Physician. 84(12): 1365-75. Available from: http://www.aafp.org/afp/2011/1215/p1365.html. [Accessed: 22 May 2015]. 5. Gourtsoyiannis, N. C. et al.(2006). Imaging of small intestinal Crohn’s disease: comparison between MR enteroclysis and conventional enteroclysis. European radiology. 16(9): 1915-1925. Available from: http://www.researchgate.net/profile/Ioannis_Koutroubakis/publication/7107800_Imaging_of_small_intesti nal_Crohn's_disease_comparison_between_MR_enteroclysis_and_conventional_enteroclysis/links/00b 7d5233301d4490c000000.pdf. [Accessed: 23 May 2015]. 6. Panés, J., et al. (2011). Systematic Review: The Use of Ultrasonography, Computed Tomography and Magnetic Resonance Imaging for the Diagnosis, Assessment of Activity and Abdominal Complications of Crohn's Disease. Alimentary Pharmacology & Therapeutics. 34(2):125-145. Available from: http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2036.2011.04710.x/full. [Accessed: 23 May 2015]. 7. Schwartz, R.A. and Nervi, S.J.(2007). Erythema nodosum: a sign of systemic disease. American Family Physician. 75(5): 695-700. Available from: http://europepmc.org/abstract/med/17375516. [Accessed: 22 May 2015]. 8. Halligan, S., Robinson, P.A.J.(2003). Chapter 20: The small bowel and peritoneal cavity and Chapter 21: The large bowel. In D Sutton (Ed.), A Textbook of Radiology and Imaging, Volume 1. 7th edition. New 46 York: Churchill Livingstone, 615-662. Shivani Priyadarshni Gillian Lieberman, MD 9. 10. 11. 12. 13. 14. 15. References Brooklyn, T., Dunnill, G., Probert, C.(2006). Diagnosis and treatment of pyoderma gangrenosum. BMJ. 333 :181-4. Available from: http://www.ncbi.nlm.nih.gov/pubmed/16858047. [Accessed: 23 May 2015]. Sanderson, J., et al.(2005). Oro-facial granulomatosis: Crohn's disease or a new inflammatory bowel disease?. Inflammatory Bowel Diseases.11(9):840-846. Available from: http://onlinelibrary.wiley.com/doi/10.1097/01.MIB.0000178261.88356.67/full. [Accessed: 23 May 2015]. Mintz, R., et al.(2004). Ocular manifestations of inflammatory bowel disease. Inflammatory Bowel Diseases. 10(2):135-139. Available from: http://onlinelibrary.wiley.com/doi/10.1097/00054725-200403000-00012/full. [Accessed: 23 May 2015]. Koh, D. M., et al.(2001). MR imaging evaluation of the activity of Crohn's disease. American Journal of Roentgenology. 177(6): 1325-1332. Available from: http://www.ajronline.org/doi/full/10.2214/ajr.177.6.1771325. [Accessed: 22 May 2015] Albert, J.G., et al.(2005). Diagnosis of small bowel Crohn’s disease: a prospective comparison of capsule endoscopy with magnetic resonance imaging and fluoroscopic enteroclysis. Gut. 54(12): 1721-1727. Available from: http://gut.bmj.com/content/54/12/1721.full. [Accessed: 23 May 2015]. Gore, R.M., et al.(1996). CT features of ulcerative colitis and Crohn's disease. American journal of roentgenology. 167(1) : 3-15. Available from: http://www.ajronline.org/doi/pdf/10.2214/ajr.167.1.8659415. [Accessed: 23 May 2015]. Wells, C.(1952). Ulcerative Colitis and Crohn's Disease: Lecture delivered at the Royal College of Surgeons of England on 5th October, 1952. Annals of the Royal College of Surgeons of England. 11(2) : 105-120. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2377529/pdf/annrcse00267-0041.pdf. [Accessed: 22 May 2015]. 47 Shivani Priyadarshni Gillian Lieberman, MD Acknowledgements Dr. G. Lieberman, MD Dr. Jonathan Kim 48 Shivani Priyadarshni Gillian Lieberman, MD Thank You 49