CT angiography in the diagnosis and post-treatment followup of midaortic syndrome: Presentation of 4 cases.

Transcription

1 CT angiography in the diagnosis and post-treatment followup of midaortic syndrome: Presentation of 4 cases. Poster No.: C-0698 Congress: ECR 2014 Type: Educational Exhibit Authors: L. Valoyes Guerrero, E. Montaño Claure, C. Sancho Calsina ; L'Hospitalet de Llobregat/ES, L' Hospitalet de Llobregat/ES Keywords: Arteries / Aorta, Interventional vascular, Computer applications, CT-Angiography, Computer Applications-Detection, diagnosis, Computer Applications-3D, Arteriovenous malformations, Obstruction / Occlusion, Grafts DOI: /ecr2014/C-0698 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 22

2 Learning objectives Describe the radiologic findings of four adult patients with midaortic syndrome diagnosed at our institution by CT angiography. Page 2 of 22

3 Images for this section: Fig. 1: Case years old man. Abdominal CT angiography with sagital 3D reconstruction and axial images.severe calcified atheromatosis located at the start of the abdominal aorta and visceral vessels (a) with severe distal stenosis (b) and pre-stenotic dilatation with intraluminal coarse calcifications (c). Page 3 of 22

4 Fig. 2: The same patient Fig 1. with axial image and coronal 3D reconstruction.occlusion of the celiac trunk ostium(a) and superior mesenteric artery( SMA) with distal repermeabilization (b) by the pancreaticoduodenal (blue arrow) and Riolano arcades(white arrow) and hypertrophied inferior mesenteric artery. Page 4 of 22

5 Fig. 3: Axial image and 3D reconstruction.a and b. Caudal to the stenosis and coinciding with the ostium of the left renal artery saccular eccentric calcified and partially thrombosed aneurysm is observed. Page 5 of 22

6 Fig. 4: Axial and coronal 3D reconstructions. The right renal artery is occluded in its proximal third (a), and distal patency by collaterals is observed in image b (blue arrow). Page 6 of 22

7 Fig. 5: Abdominal CT angiography post-intervention in axial (a) and 3D reconstruction (b) shows Stent patency of the left renal artery. Page 7 of 22

8 Background The midaortic syndrome is a rare entity, characterized by coarctation or stenosis of the aorta in its abdominal or distal descending thoracic course with progressive involvement of visceral branches: renals(63%), mesenteric(33%) arteries, or both, in various degrees. The main clinical manifestation is hypertension and weakness or absence of the femoral pulses. Although the etiology is unknown, some authors have postulated a congenital origin and acquired as Takayasu arteritis, retroperitoneal fibrosis, Williams syndrome, fibromuscular dysplasia, neurofibromatosis and mucopolysaccharidosis. Historically arteriography has been the gold standard technique to study aortic stenosis, however nowadays multidetector computed tomography ( CT ) has high resolution and the ability to perform imaging postprocesing and has proven to be a useful tool for the diagnosis of this disease, besides providing information on its extent, degree of stenosis, pre or post-stenotic dilatation, collateral circulation and impact on visceral branches. Both surgical and endovascular therapy (stent) may improve long-term hypertension and its effects on health. CT angiography also allows us to determine appropriate treatment options in each case as well as being a treatment follow-up tool. In the present study we reviewed 4 cases of midaortic syndrome diagnosed in our hospital, their diagnostic features and post-treatment follow-up for CT angiography. The scans were performed with patients in supine position and image acquisition were performed with the patient in apnea. The equipment used was a 64-detector scanner, General Electric Light Speed VCT, with the following parameters: a radiation dose of mGY and mas, a gantry rotation time of 0.6 seconds and FOV of 50cm. 80cc intravenous iodinated contrast is administered with a rate of 5-6cc per second. Later images were reviewed on a workstation with images of 2.5 mm thickness reconstructed at 0.6mm intervals and multiplanar reconstructions, and 2D - 3D volume rendering were performed with vessels analysis program. Page 8 of 22

9 Images for this section: Fig. 6: Case years old woman. Abdominal CT angiography with images in coronal.a and b. Permeable abdominal aorta, with severe stenosis of 9mm maximum diameter is located at the outlet portion of visceral trunks. Page 9 of 22

10 Fig. 7: Image with a fusiform poli-lobed eccentric aneurysm above a 21mm diameter and 30mm long stenosis. Page 10 of 22

11 Fig. 8: Coronal and oblique 3D reconstructions. A and b. Occlusion of the origin of digestive trunks, with the existence of a large hypertrophied abnormal vessel above the aneurysm(blue arrow) that distally supplies the celiac trunk and SMA. Page 11 of 22

12 Fig. 9: At output level of the digestive trunks (superior mesenteric) an abnormal, also permeable vessel(red arrow) that connects as well with the superior mesenteric and celiac trunk is observed. Page 12 of 22

13 Fig. 10: Image after placement of coated prosthesis (45mm long Stent). Page 13 of 22

14 Findings and procedure details We studied 4 patients between 25 and 53 years old, 2 male and 2 female. The common symptom was hypertension. All of them had aortic stenosis, in 2 patients it was located in distal thoracic aorta, in 1 patient near the celiac trunk and superior mesenteric artery (SMA) and, in the last patient, above the renal arteries; the mean stenosis was above 60%. Occlusion of the celiac trunk, SMA and both renal arteries were found in one patient, with development of collateral circulatory pathways. In another patient there was occlusion of the celiac trunk and SMA, without renal involvement, in which retrograde flow came through two collateral vessels. We found pre-stenotic fusiform saccular aneurysms in 2 patients and a thrombosed saccular aneurysm of the left renal artery in another. 3 patients were treated with STENT and one patient received only medical treatment. After treatment, 3 patients show controlled blood pressure with 2 or 3 drugs, one patient is asymptomatic without medication. Page 14 of 22

15 Images for this section: Fig. 11: Case years old man. Angiography where a severe stenosis distal thoracic aorta is seen (a). Through a left femoral introducer, a naked prosthesis mounted on a balloon catheter is advanced and deployed in the stenotic segment (b and c), dilation is expanded with 16mm and 20mm balloons (d). Final result obtaining a 20mm proximal calibre in the area of the prosthesis (e Page 15 of 22

16 Fig. 12: Thoraco-abdominal CT angiography with coronal (a) and axial ( b) images, after Stent placement, where permeability of the thoraco-abdominal aorta is seen. Page 16 of 22

17 Conclusion CT angiography is a radiological usefull study that determines the characteristic findings for the diagnosis and subsequent treatment and follow-up of midaortic syndrome in adult patients. Page 17 of 22

18 Images for this section: Fig. 13: Case years old woman. Fusiform dilatation of thoraco-abdominal aorta with proximal endcranial to the diaphragmatic hiatus and distal end near to the origin ofthe renal arteries. The images show a maximum longitudinal extension of about 81 mm and their maximum axial diameters are about 44 x 39 mm. Fig. 14: These images in coronal reconstructions show a poly-lobed shape in its upper and lower ends. Page 18 of 22

19 Fig. 15: Abdominal CT angiography in sagital (a) and MIP reconstruction of coarctation (b). Page 19 of 22

20 Fig. 16: Consecutive axial slices of thoracoabdominal CT angiography,just where described lower caliber starts. Page 20 of 22

21 Fig. 17: Abdominal CT angiography with axial images. Permeability of visceral branches and absence of collateral circulation. a. Outflow of the celiac trunk, b. Outflow of the superior mesenteric artery. c. Outflow of the right renal artery. d. Outflow of the right renal artery.the celiac trunk originates from the aneurysm itself and superior mesenteric artery in its lower and poly-lobed area. Page 21 of 22

22 References Carmen Sebastià, MD, Sergi Quiroga, MD, Rosa Boyé, MD, Mercedes Perez-Lafuente, MD. Aortic Stenosis: Spectrum of diseases Depicted at Multisection CT,. Radiographics 2003; 23: S79-S91. Chocrón de Benzaquen, S; Muñoz Lopez M; Madrid Aris AD, Síndrome de Aorta Media. Anales de pediatría, 2011; 75: 33-9, vol 75 núm 01. Connolly JE, Wilson SE, Lawrence PL, Fujitany RM. Middle aortic síndrome: distal thoracic and abdominal caorctation, a disorder with múltiple etiologies. J Am Coll Surg 2002; 194: Pubmed. Heung Bae Kim, Khashayar Vakii, Biren P. Modi, Michael Fergudon. A Novel Treatment for the Midaortic Syndrome. N engl J Med 2012, 367: VD Lewis, SG Meranza, GK McLean, JA O Neill, Jr, HD Berkowits and DR Burke. The midaortic syndrom: Diagnosis and treatment, Departament o f Radiology. Hospital of the University on Pennsylvania. Page 22 of 22