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Endovascular treatment of abdominal aneurysmal aortitis in Behçet’s disease

Marc-Antoine Vasseur, MD, S. Haulon, MD, J.P. Beregi, MD, T. Le Tourneau, MD, A. Prat, MD, and H. Warembourgh, MD, Lille, France

Arterial complications of Behçet’s disease are rare and affect mainly the and iliac . Perforation of the arterial wall is the most common lesion, predisposing to false or rupture. Open surgical repair is difficult, and anastomotic false often occur because of aortic wall fragility. We report here the case of using a bifurcat- ed stent to treat aortoiliac false aneurysms in a 37-year-old patient. Endovascular repair could be an alternative treatment of aneurysmal manifestations in Behçet’s disease. (J Vasc Surg 1998;27:974-6.)

Orogenital aphthosis, skin lesions and uveitis On examination, the patient still complained of pain on characterize Behçet’s disease (BD), described by a abdominal palpation and had no signs of orogenital aphtho- Turkish dermatologist in 1937.1 Vascular manifesta- sis. Blood examination revealed neither anemia nor an tions, although rare, may occur. Venous inflammatory syndrome, and C-reactive Protein was normal. is the most frequent vascular complication. In recent A marked pigtail aorta arteriography demonstrated an years, however, many authors have reported arterial irregular dilatation of the infrarenal aorta, a false aneurysm at the origin of the left iliac and a second false thrombosis or aneurysms that are the most severe aneurysm 1 cm below the ostium of the right internal iliac manifestations of BD because of surgical technical artery (Fig. 2A & 2B). Thoracic CT was normal, as was a difficulties or late postoperative anastomotic false duplex scan (DS) of the brachiocephalic arteries. aneurysms. We proposed endovascular treatment of these aortobi- For these reasons, we attempted endovascular iliac false aneurysms. First, we embolized the right inter- treatment of abdominal aneurysmal aortitis in a 37- nal iliac artery using coils to achieve thrombosis, which a year-old Mediterranean man who had BD. DS confirmed 48 hours later. There were no signs of pelvic . CASE REPORT Three days later, we placed a bifurcated stent below A 37-year-old Mediterranean man received a BD diag- the renal arteries through a right groin incision and left nosis in 1984 when he presented orogenital aphthosis and percutaneous femoral approach. We used a thermic mem- deep . The pathergy test (hyperactivity ory form self-expandable device, the VANGUARD endo- of the skin to simple trauma) was positive, and the patient prosthesis (Boston Scientific Corporation). This is made of was treated with oral anticoagulants (coumadin) and two components: the aortic section (22 mm diameter) colchicine thereafter. In 1996, he developed another deep with a single limb (10 mm diameter and 153 mm total venous thrombosis, which was treated with intravenous length) inserted in an 18 F introducer. At the trailing of heparin. the aortic section, the second controlateral limb inserts in During a strenuous effort in May 1997, he suffered a short branch (9 mm diameter) through a 10 F introduc- sudden back and abdominal pain associated with ileus. er. In the present case, the limb was 10 mm diameter and Contrast transit excluded a diagnosis of bowel hematoma. 100 mm long. Once in place, we expanded the nitinol Abdominal contrast enhanced computed tomography stent by gentle manual inflation with a 10-mm diameter (CT) showed aortic and iliac false aneurysms (Fig. 1). balloon (Ultrathin Meditech) filled with diluted contrast Prednisone (60 mg per day) was prescribed, and then the medium. The procedure time was 105 minutes, with 22 patient was referred to our institution. minutes of fluoroscopy and an injection of 140 ml of con- trast medium. A final angiography demonstrated throm- bosis of the aneurysms. Recovery was uneventful, and From the Department of CardioVascular Surgery, Cardiologic both DS and CT confirmed an excellent outcome (Fig. 3). Hospital. We discharged the patient from the hospital four days later Reprint requests: Marc-Antoine Vasseur, MD, Department of and prescribed 300 mg aspirin, 60 mg prednisone, and 1 Cardiovascular Surgery, Cardiologic Hospital, 59037 Lille Cedex, France. mg of colchicine per day. He was followed up for 6 Copyright © 1998 by The Society for Vascular Surgery and months with contrast enhanced CT and DS showing com- International Society for Cardiovascular Surgery, North plete regression of the aorta iliac false aneurysms and good American Chapter. patency of the graft. Clinical examination was normal, 0741-5214/98/$5.00 + 0 24/4/89713 without any groin complication.

974 JOURNAL OF VASCULAR SURGERY Volume 27, Number 5 Vasseur et al. 975

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Fig. 1. Preoperative C.T. B

DISCUSSION BD has a peculiar geographic distribution. Although it is rare in Western Europe (5/100,000), it is more common in Mediterranean regions (190/100,000) or in Japan (10/100,000), where an association with HLA B5 has been proved.2-4 The International Study Group for Behçet’s Disease estab- lished five major criteria for the diagnosis: recurrent oral or genital aphthosis, eye lesions, skin lesions, and a positive pathergy test. BD can also involve the joints, gastrointestinal tract or central nervous system.5 The incidence of cardiovascular complications in BD is approximately 30%, although these complica- tions are apparently the major cause of death, which highlights the gravity of the condition. Therefore, some authors include cardiovascular complications as a sixth criterion for BD diagnosis.6 Of the vascu- Fig. 2A & 2B. Preoperative angiography: aortic and iliac false aneurysms (arrows). lar lesions reported, 25% affect the venous system exclusively and 7% the arterial system exclusively — 68% of the patients with vascular involvement have both types.7 The pathologic process appears to involve a vas- Deep venous thrombosis (DVT), involving culitis of the vasa vasorum with adventitial fibrosis, peripheral and the inferior or superior vena destruction of the elastic and muscular fibers of the cava, is the vascular complication most usually media and intimal thickening. This explains throm- reported. Recurrent superficial is bosis in small diameter vessels and dilatation or rup- an important risk factor for DVT, which we must ture in larger vessels.9 These particular conditions aggressively treat in these patients. Arterial lesions explain the technical difficulties associated with the can involve any artery with , thrombosis, and surgical treatment of aortoiliac lesions in BD and the false or true aneurysms.6 The ascending or descend- recurrent false aneurysms in anastomotic sites, which ing thoracic aorta, the aortic arch, pulmonary artery occur in 30% to 50% of the cases. To avoid complica- aneurysms, and aortic or mitral regurgitation are the tions, you must perform anastomosis, if possible, in main thoracic complications associated with BD.8 In macroscopically disease-free segments outside the brachiocephalic or limb arteries, both thrombosis inflammatory area where running sutures tend to cut and aneurysms are encountered. Aortoiliac lesions through the arterial wall. Preoperative prescription of are aneurysms or false aneurysms that can rapidly corticosteroids is useful to reduce this enlarge with the concomitant high risk of rupture and to prevent further anastomotic false aneurysms. even if they are small. Inflammation of the aortic Some authors propose wrapping the anastomoses, wall and surrounding tissues is often observed. using pledgeted sutures or performing aneurysmor- JOURNAL OF VASCULAR SURGERY 976 Vasseur et al. May 1998 rhaphy10 or simple ligation of the aneurysm when possible.11 This case is apparently the first to report the endoluminal grafting of aortoiliac aneurysm in BD. Parodi, May and Miahle12-14 already proved endovascular grafting of abdominal aortic aneurysms was possible. Short-term results are extremely vari- able from one device to another. If you strictly observe the anatomical criteria, then the prognostic outcome is good with the device that we used.15 The VANGUARD stent is self-expandable, held in place by a row of small barbs at the top of the prosthesis and has a good radial force. It is a full-length stent frame of nitinol annealed into a tubular zigzag con- figuration. Each opposing zigzag fastens together Fig. 3. Postoperative C.T.: thrombosis of the aortic with a 7/0 polypropylene ligature. A polyester fabric aneurysm and limbs of the endoprosthesis. of low porosity covers the entire stent, except for 12 mm at the top. One of the major problems encoun- tered with this device is a periprosthesic leak, espe- 2. Lie JT. Editorial: Vascular involvement in Behçet’s disease: cially when it occurs in the proximal neck because of arterial and venous and vessels of all sizes. J Rheumatol its aneurysmal evolution. This is particularly true in 1992;19:341-2. 3. Shimuzu T, Ehrlich GE, Inaba G. Behçet’s disease (Behçet’s BD because of the arterial wall fragility. We were con- syndrome). Semin Arthritis Rheum 1979;8:233-60. cerned that false aneurysms could appear secondarily 4. Chajek T, Fainaru M. Behçet’s disease. Report of 41 cases because the self-expanding stent exerts continuous and a review of the literature. Medicine 1975;54:179-96. radial force. With precise preoperative measures and 5. International Study Group for Behçet’s Disease. Criteria for proper sizing of the graft, however, we thought this diagnosis of Behçet’s disease. Lancet 1990;335:1078-80. 6. Wechsler B, Le Thi Huong Du, De Gennes C, et al. risk should remain low. We decided to perform this Manifestations artérielles de la maladie de Behçet. Rev Méd procedure to avoid open surgery during the active Int 1989;10:303-11. period of the disease. Although late femoral false 7. Koc Y, Gullu I, Akpek G, et al. Vascular involvement in aneurysms may occur because of the femoral cut Behçet’s disease. J Rheumatol 1992;19:402-10. down and puncture, they are easier to treat in that 8. Tunaci A, Berkmen YM, Gokmen E. Thoracic involvement in Behçet’s disease: pathologic, clinical, and imaging features. location than in the aortoiliac position. Because it is Am J Roentgenol 1995;164:51-6. technically easier, endovascular management of this 9. Matsumoto T, Uekusa T, Fukuda Y. Vasculo-Behçet’s dis- kind of lesion has provided encouraging short-term ease: a pathologic study of eight cases. Hum Pathol 1991; results in this case. However, a longer follow-up for 22:45-57. CT and DS is necessary to evaluate the relationship 10. Takagi A, Kajiura N, Tada Y, Ueno A. Surgical treatment of non-specific inflammatory arterial aneurysms. J Cardiovasc between the endoprosthesis and the aortic wall, par- Surg 1986;27:117-24. ticularly in BD where the arterial wall is weak. 11. Tüzün H, Besirli K, Sayin A, Vural FS, Hamaryudan V, Hizli N, Yurdakul S, Yazici H. Management of aneurysms in CONCLUSION Behçet’s syndrome: an analysis of 24 patients. Surgery Despite its rarity in Western Europe, BD in some 1997;121:150-6. 12. Parodi JC. Endovascular repair of aortic aneurysms, arteri- cases is associated with vascular lesions, principally ovenous fistulas, and false aneurysms. World J Surg 1996 through aortoiliac aneurysms when arterial involve- 20:655-63. ment occurs. Open surgery, although often difficult, 13. May J, White GH, Yu W, Waugh R, Stephen MS, Harris JP. is the rule even with 30% to 50% of the patients Repair of abdominal aortic aneurysms by the endoluminal requiring second surgeries because of the occurrence method: outcome in the first 100 patients. Med J Aust 1996;165:549-51. of anastomotic false aneurysms. The endovascular 14. Mialhe C, Amicabile C. Traitement endovasculaire des approach could provide a new form of treatment, anévrysmes de l’aorte sous-rénale par l’endoprothése Stentor. particularly if long-term results are as positive as the Série préliminaire. J Mal Vasc 1995;20:290-5. short-term results presented here. 15. Blum U, Voshage G, Lammer J, et al. Endoluminal stent- grafts for infrarenal abdominal aortic aneurysms. N Engl J Med 1997;336:13-20. REFERENCES 1. Beçhet H. Uber rezidiverende aphtose, durch ein Virus verursachte Geschwure am Mund, am Auge und den Genitalien. Derm Wochenschr 1937;36:1152-7. Submitted Oct. 20, 1997; accepted Feb. 16, 1998.