The diagnosis and treatment of aortitis in Behcet s disease. Pr Imed Frikha - Sfax - Tunisia
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1 The diagnosis and treatment of aortitis in Behcet s disease Pr Imed Frikha - Sfax - Tunisia
2 I do not have any potential conflict of interest
3 Introduction Multisystemic vasculitis. It affects : - Mucocutaneous - Ocular - Locomotor - Central nervous - Cardiovascular - Respiratory - Gastro-intestinal
4 Introduction First description : Hulusi Behçet Turkish dermatologist. Second or third decade of live. Men : 4/1.
5 Geography Mediterraneen and asiatic countries. Turkey :300/ Japan-Tunisia :10/100000
6 France : 35% Spain : 28-33% Italy: 25% Lebanon: 15-25% Turkey : 17-30% Morocco : 30-45% Tunisia : 25-45% Algeria : 23-30% Corea Japan China 5-15% Prevalence of vascular manifestations In Behçet s syndrome
7 Diagnosis
8 Diagnosis Pathergy test Genetic predisposition : HLA BW51 Clinical course of Behçet disease is characterized by reemissions and exacerbations.
9 Vasculo-Behçet s 3,6-24 %. Venous +++
10 Vasculo-Behçet s Artery involvement : % - Aneurysm : 65 % - Occlusion : 35 % Major cause of death because the risk of rupture. Interval betwen initial diagnosis of BD and diagnosis of vasculo-behçet is 6-8 years.
11 Occlusion
12 Aneurysms First case of arterial involvement reported by Mishima in Aorta. Pulmonary artery. Femoral. Others. Multiple aneurysms : 18 %.
13 Abdominal Aorta
14 Pulmonary artery
15
16 Pathogeni s True aneurysm vessel wall weaknes Dilatation Inflammatory cells (neutrophis, lymphocyts) infiltrate the media and adventitia with the destruction of elastic and muscle cells in the media
17 Pathogeni s Pseudoaneury sm Transmural necrosis Perforation of the vessel wall Occlusion of the vasa-vasorum
18
19 Clinical presentation Thoracic and abdominal aneurysms are discovered in the chronic stage with vague symptoms( back pain and abdominal disconfort). Complications : - Rupture : 60 %. *Duodenum *Vena cava *Vertebral -Retroperitoneal fibrosis.
20 Explorations Duplex Sonography. Arteriography by arterial puncture must be avoided intravenous digital substraction angiography. CT scan. MRA. PET scan ( assessing inflammatory activity).
21
22 Treatment Medical Surgery Endovascular
23 Medical treatment Immunosuppressive and corticosteroides therapy. Should be started in patients with arterial disease before endovascular treatment or surgery and should be continued after the procedure to ovoid early and late postprocedure complications.
24 Medical treatment Protocole EULAR Solumedrol and cyclosporin. Duration : 1 year ( normalized CRP). Colchicin. Anti-platelet. Antivitamine K.
25 Surgery Challenging pathology for cardio-vascular surgeon. Aneurysm should be repared at the time of the detection because of the high risk of rupture. Agressive approch.
26 Methods Aneurysmorraphy. Simple patching : -smal defect. -supra-renal aneurysm. High recurrence+++
27 Perforation of medial abdominal aorta Arterial patch Perforation
28 2 8 False aneurism of medial abdominal aorta: arterial patch
29 Methods Abdominal aorta tube graft : PTFE- Dacron. Aorto-bi-iliac bypass.
30 Difficulties Periadventicite difficulty of dissection. Fragile wall false aneurysm. dropping anastomotic Aortico-duodenal fistula.
31 Precautions Little traumatic clamping. Anastomosis on intact arteries far away from aneurysm. Suture support : patch-biologic glue. Wrapping with the aneurysm wall and omental flap.
32 Results Poor. Mortality :10-30%. Morbidity : - Occlusion. - Recurrence : %. - Graft-enteric fistula :0.4-4 %.
33 Endovascular therapy First reported case : Vasseur in 1998.
34
35 Advantages Lower mortality : 0,6-3,5 %. High technical succes rate. Complete regression of the aneurysm ( 3 months).
36 Complications Rate : 19 %. Endoleaks rupture. Pseudoaneurysm at the puncture site. Recurrent aneurysm after EVAR ( local mechanical stress of stent graft).
37
38
39 Other aortic localization Ascending aorta. Thoraco-abdominal aorta.
40
41 Personal Study
42
43 Case 1 M 46 y. Diagnosis BD : 10 y. Abdominal pain - Fievre. CRP 250.
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48 Case 2 M 53 y. Diagnosis BD : 5 y. Abdominal pain. CRP 120.
49
50
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54 Case 3 M 54 y. Diagnosis BD : 18 y. Abdominal pain. CRP 150.
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60 Case 4 M 38 y. Diagnosis BD : 7y. Fortuitous. CRP 50.
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63 Case 5 M 47y. Diagnosis BD : 17y. Abdominal pain. CRP 90.
64
65 Case 6 W 27y. Diagnosis BD : 0 y. Thoracic pain-choc. CRP 237.
66
67
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69 Technique
70
71 Conclusion Rare. Aortitis serious. Surgery difficult. Endovascular +++ Recurrence. Medical treatment.
72 Thank You
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