Complications of Perianal Crohn s Disease - Adenocarcinoma & Extensive Fistulization

Transcription

1 Complications of Perianal Crohn s Disease - Adenocarcinoma & Extensive Fistulization Poster No.: C-0711 Congress: ECR 2013 Type: Educational Exhibit Authors: P. Faria João 1, D. Penha 2, P. Cabral 1, E. Rosado 1, A. M. D. Keywords: DOI: Costa 3 ; 1 Amadora/PT, 2 Lisboa/PT, 3 Amora, Se/PT Fistula, Cancer, Acute, Education, Contrast agent-intravenous, MR, CT, Gastrointestinal tract, Abdomen /ecr2013/C-0711 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 10

2 Learning objectives To understand the potential for Perianal Crohn s disease to have the serious and potentially fatal complications of extensive fistulous disease and adenocarcinoma. To recognize Magnetic Ressonance as the best imaging modality in the management of this disease and be familiar with its findings. Background Perianal Crohn s disease (PCD) refers to the involvement of the perianal and perineal regions in the inflammatory process of Crohn s disease (CD). It is very frequent, with reported incidences of up to 80% in patients with CD. It is almost universal in the presence of rectal CD and in a significant proportion of cases there will be disease manifestations in the ileum. The transmural inflamation process will often evolve with formation of fistulous tracts which can originate from the inflamed anal glands or result from penetration of ulcers from the rectum or the anal canal. Perianal fistulization may be the initial manifestation of CD, preceeding the diagnosis by several years. A smaller group of patients with CD may have perianal fistulization as the sole manifestation of the disease. The tracts are often complex with secondary extensions and frequently lead to abbcesses formation (with high recurrence rate), making its medical and surgical management extremely challenging. Adenocarcinoma arising along a fistulous tract is a rare, but well-known and very serious complication of long-standing (> 15 years) PCD, with an estimated incidence of less than 1%. There is no consensus regarding the causes of development of fistula-related carcinoma. The lesions usually show slow growth and features of local aggressivness. Tumor spread is usually lymphatic. An early diagnosis may allow curative treatment (chemotherapy and surgery), therefore a high-degree of suspicion is required so that this diagnosis is not missed. The management of PCD is controversial. It is however widely recognized that an accurate identification of the fistula s anatomy is absolutely essential, both for characterization of the real extension of the disease (often underestimated at clinical examination) and to provide the surgeon with a pre-operative road map (to minimize surgical trauma to the anal sphincters). Imaging plays a key role in this process and Magnetic Ressonance (MR) has been shown to be the method of choice for the evaluation of perianal fistulas (diagnosis, classification and monitoring). Among its recognized atributes one can mention its high soft-tissue contrast resolution, multiplanar capability, Page 2 of 10

3 wide field of view and the lack of ionizing radiation. The latter is an important aspect as many of these patients will require repeated studies in the course of the disease. Imaging findings OR Procedure details MR is best imaging method to evaluate PCD. Conventional fistulography is very limited to characterize complex fistulas or abbcess and gives no information about its relation to the sphincter complex or elevator plate. Anal Endosonography is operator-dependent, has a more restrictive field of view and is often badly tolerated when inflammation is present. Computed Tomography lacks contrast resolution to differentiate subtle fistulas from the sphincter complex and exposes the patient to ionizing radiation. MR allows multiplanar assessment of the sphincter complex, which is essential for surgical planning. Therefore imaging planes must be correctly aligned to the longest axis of the anal canal (i.e the axial oblique plane should parallel and the coronal oblique perpendicular). The whole perineum should be imaged. Protocols vary among institutions, but should include T1-WS and a fluid-sensitive sequence (e.g STIR or FS T2-WI). Gadolinium administration is not consensual. Images for this section: Page 3 of 10

4 Fig. 1: Coronal oblique STIR (Single Time Inversion Recovery)image 46-year-old female with difficult to control CD. MR showed large complex fistulous tracts with involvment of the rectovaginal septum, rectum and urogenital diaphragm and external. Some fistulous tracts endend at the perianal cutaneous surface and external genitals. Page 4 of 10

5 Fig. 2: Sagittal STIR image of the same patient as in Fig. 1 Page 5 of 10

6 Fig. 3: Axial oblique STIR image of the same patient as in Fig. 1 Page 6 of 10

7 Fig. 4: Sagittal STIR image of a 48-year-old female with agressive PCD with complex fistula to the rectovaginal septum and a large perianal mass with heterogeneous highsignal at STIR sequence. At biopsy mucinous adenocarcinoma was found. Page 7 of 10

8 Fig. 5: Axial oblique STIR image of the same patient as in Fig. 4. Page 8 of 10

9 Conclusion Perianal involvment in CD is a predictive factor for the development of severe CD and is often associated with extensive fistulization. This condition can be highly-debilitating and lead to recurrent hospitalization and multiple surgeries. Adenocarcinoma is a rare but potentially fatal complication of long-standing PCD, and should be considered when evaluating such patients. The management of this entity requires a multidisciplinary approach in order to obtain the best results. The Radiologist has a key role and MR is the best imaging modality for diagnosis, classification and follow-up of PCD. References - Cohen, RD; "Inflamatory Bowel Disease - Diagnostics and Therapeutics", Humana Press (2011) - Tersigni, R, Prantera C; "Crohn s Disease - A Multidisciplinary Approach", Springer (2011) - Schäfer, AO, Langer, M "MRI of Rectal Cancer", Springer (2010) - Ruffolo, C, Citton, M, Scarpa, M, Angriman, I, Massani,M, Caratozzolo, E. "Perianal Crohn's disease: Is there something new?" World J Gastroenterol 2011 April 21; 17(15): Platell C, Mackay J, Collopy B, et al. Anal pathology in patients with Crohn's disease. Aust N Z J Surg 1996; 66:5. - Miguel Criado J, del Salto L, Rivas P, del Hoyo L, Velasco L, de las Vacas M, Marco Sanz A, Paradela M, Moreno E.MR imaging evaluation of perianal fistulas: spectrum of imaging features" Radiographics 2012 Jan-Feb;32(1): Page 9 of 10

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