Abstract
Introduction
To report total endovascular treatment for a rare case of Crawford extent IV thoraco-abdominal aortic aneurysm (TAAA) using custom-designed branched device in a patient with Behçet’s disease.
Methods
A 50 years’ old man with history of BD was accidentally diagnosed Crawford extent IV TAAA during computed tomography follow-up after left nephrectomy of renal carcinoma. The aneurysm extended from descending aorta to right common iliac artery with a maximum diameter of 6.2 cm.
Results
The endovascular procedure wassuccessfully performed using custom-designed branched component to cannulate visceral arteries, bifurcated endograft and iliac legs to exclude the aneurysm sac in abdominal aorta and an iliac branched device to preserve the right internal iliac artery. The patient was discharged without any complication. Computed tomography angiogram at one month after endovascular repair demonstrated total exclusion of the aneurysm, patent visceral branches and right internal iliac artery. No complication occurred to six-month follow-up.
Conclusion
Endovascular treatment of stable TAAA in patients with Behc?et's disease using custom-designed branched device is feasible, microinvasive and safe. The long-term efficacy needs to be observed.
Introduction
Behçet’s disease (BD) is a rare systemic inflammatory disorder with unknown reason, manifests as oral and genital ulcerations, skin lesions, uveitis and vasculitis. Vascular involvement occurs in up to 40% of BD patients, and approximately 19% of artery lesions are arotic aneurysms, which is the major cause of death in BD patients. 1
Open surgery for aortic aneurysm in BD carries 30–50% risk of anastomotic pseudoaneurysm, even a potential to exacerbate the process of BD.2–4 Endovascular treatment with adjunctive immunosuppressive therapy has been documented carrying less procedural trauma and higher survival rate than open repair, and a shift from open surgery to endovascular repair in BD has been recommended.2,3,5–8
However, treatment of thoraco-abdominal aortic aneurysm (TAAA) in BD still remains challenging. In this paper, we are reporting successful endovascular treatment for Crawford extent IV TAAA in a BD patient using custom-designed branched device.
Case report
Written informed consent for patient information and images to be published was obtained. A 50 years’ old Spanish man diagnosed TAAA was admitted to our centre in January 2020. He had a history of BD and was adhered to azathioprine and glucocorticoids treatment for 24 years. This patient developed vascular complication despite prolonged immunosuppressive treatment. The TAAA was accidentally discovered during computed tomography follow-up after left nephrectomy because of renal carcinoma. As shown in Figure 1, computed tomography angiogram (CTA) demonstrated a Crawford extent IV TAAA with a maximum diameter of 6.2 cm. The aneurysm extended from the 12th intercostal space to the right iliac artery bifurcation involving the visceral aortic segment and the origins of the renal, superior mesenteric and celiac arteries. A 50% stenosis was found at the origin of superior mesenteric artery (SMA) (Figure 1(c)). Both positron emission tomography (Figure 2) and laboratory test (erythrocyte sedimentation rate: 4 mm/h, normal range: 1–20 mm/h) found that this patient was in inactive stage.
CTA scan demonstrates a Crawford extent IV TAAA: (a) the aneurysm extends from descending aorta to right common iliac artery; (b) the maximum diameter of the aneurysm is 6.2 cm; (c) arrow shows stenosis at the origin of SMA.
PET scan shows low FDG uptake by the aneurysm, indicating that the BD was in inactive stage.
Because the patient was asymptomatic, endovascular treatment was planned to use a custom-designed (CMD) branched component (Cook Medical, Bloomington, IN, USA) to preserve the visceral arteries, a bifurcated endograft for the abdominal aorta and an iliac branch device to preserve the right internal iliac artery. A written informed consent was obtained. The procedure was performed with general anaesthesia and with the navigation assistance of Syngo iGuide Toolbox (Siemens Healthcare GmbH, Erlangen, Germany). First, the CMD component was advanced and deployed at the descending aorta according to the manufacture’s instruction. Second, a ZBIS-12–45-41 Iliac Branch Device (Cook Medical, Bloomington, IN, USA) was implanted into the right iliac artery, and then a 9 mm × 37 mm BeGraft balloon expandable covered stent (Bently InnoMed GmbH, Hechingen, Germany) was deployed into the right internal iliac artery. Next, a ZFEN-D-12–28-109 (Cook Medical, Bloomington, IN, USA) abdominal bifurcated endograft was deployed through the left femoral artery access, with its proximal end anchored in the CMD component overlapping by 3 cm. A ZISL-16-42 (Cook Medical, Bloomington, IN, USA) leg extension was implanted for bridging the right IBD component and the abdominal bifurcated endograft. Next, ipsilateral leg extension ZISL-16-59 and ZISL-16-77 (Cook Medical, Bloomington, IN, USA) was deployed into left common iliac artery. Finally, the visceral bridging endografts were completed via brachial access. In brief, right renal artery was cannulated with a 8 mm × 27 mm BeGraft balloon expandable covered stent (Bently InnoMed GmbH, Hechingen, Germany) at the level of the stump to gain access to the renal artery ostium and a 8 × 50 mm Viabahn endograft (Gore Inc., Arizona, USA) relining the previous BeGraft and extended two centimetres into the renal artery. SMA and celiac artery were cannulated with 9 mm × 50 mm and 8 mm × 50 mm Viabahn endograft (Gore Inc., Arizona, USA) respectively.
The patient accepted monitoring in intensive care unit for three days, and the mean artery pressure was maintained above 80 mmHg. After another four days of hospital stay in general ward, the patient was discharged without complication. For follow-up, CTA will be performed post-operatively at the 1st, 6th, and 12th month and yearly after. One month after the endovascular treatment, CTA scan demonstrated complete thrombosis of the aneurysm sac without endoleak, patentcy of visceral and iliac branches (Figure 3). During the six-month follow-up, no complication was observed.
CTA scan at 1-month follow-up shows patent visceral branches and right internal iliac artery (a) and complete aneurysm sac thrombosis without endoleak (b).
Discussion
Endovascular repair of aortic aneurysm involving visceral branches in BD still remains difficult. In this report, the patient demonstrated a Crawford type IV TAAA extending from the descending aorta to iliac artery, which is the most challenging aortic lesion. A literature search was performed in the PubMed database to identify all publications on endovascular or open repair for TAAAs in BD in the English language, with searching term (‘Thoracoabdominal Aortic Aneurysm’ AND ‘Behçet’s disease’. As shown in Table 1, three reports demonstrated open surgical repair of TAAA in 3 BD patients,9–11 including one case experienced recurrent anastomotic pseudoaneurysm. 11 Three articles reported treatment for TAAA using endovascular repair with visceral debranching technique in 5 BD patients.2,8,12 Balcioglu et al. 8 reported successful endovascular treatment combined with visceral debranching in three Crawford type IV TAAA in BD patients. Despite hybrid procedure carrying less surgical trauma, patients are equally like to suffer significant risk of anastomotic pseudoaneurysm to traditional open repair. Also, not all types of TAAA are suitable for hybrid procedure, which needs to anastomose debranching graft in the iliac artery. However, the necessity to anchor iliac leg and exclude the right common iliac artery aneurysm made debranching procedure infeasible in this patient. Moreover, both the history of left nephrectomy and the need to reconstruct all visceral branches exerted great difficulties on debranching procedure in this patient.
Literature review on endovascular or open repair for TAAA in BD.
SMA: superior mesenteric artery; BRA: bilateral renal artery; CA: celiac artery; HA: hepatic artery; CMD: custom-designed.
Total endovascular treatment for aortic aneurysm involving visceral arteries in BD has been documented by three reports. Marone et al. 6 reported successful treatment of a ruptured Crawford extend V TAAA using endovascular repair with a parallel stent-graft to preserve SMA. Belczak et al. 7 reported successful treatment of a recurrent para-renal pseudoaneurysm secondary to initial endovascular repair of ruptured abdominal aortic aneurysm, using a CMD 4-fenestration component. Xia et al. 13 reported using in vitro fenestration technique to reconstruct right renal artery in a ruptured TAAA BD patient with occluded CA, SMA and left renal artery. These successful attempts make total endovascular treatment for TAAA in BD a feasible option. To the best of our knowledge, the current report is the first attempt to treat TAAA in BD using CMD branched endograft.
However, endovascular treatment for aneurysms in BD is not without any risk, complications induced by chronic vascular inflammation, such as graft infection, stent occlusion and recurrent pseudoaneurysm at the margin of endograft or puncture site can be up to 40%.2,3,5,14 This patient has been adhered to immunosuppressive therapy for many years, which is helpful to control the chronic inflammation and reduce the complications. Also, the PET/CT scan, which has emerged as a potential tool to diagnose and follow-up inflammatory AAAs, 15 demonstrated low 18F-fluorodeoxyglucose uptake in the aorta, indicating a low activity and severity of inflammation.16,17 And the patient continued immunosuppressive treatment post-operatively and followed by the rheumatology group. Target artery instability might lead to catastrophic complications, especially when there was a 50% stenosis in the origin of SMA and a solitary functioning kidney. In the current case, we use Viabahn self-expandable endografts as bridging stents. Despite novel balloon expandable endografts have potential advantages, such as lower profile, ability to flare and decreased number of steps for cannulating each branch, they were associated with higher rate of late target artery instability, including endoleak, stent stenosis and occlusion compared with self-expandable endografts. 18 Also, there still lacks evidence of using balloon expandable endografts in arteries in BD. In contrast, the well performance of Viabahn self-expandable endografts in arteries in BD has been documented by previous reports.2,6
Although the incidence of paraplegia is estimated to be 2.5–8%, which is lower than that of open surgical repair, SCI remains one of the most feared complications of extensive stent graft exclusion of thoraco-abdominal aorta. 19 Several preventive strategies were used to reduce the risk of SCI as we previously described. 20 During the procedure, neuromonitoring was performed for early identification of spinal cord malperfusion allowing for immediate measures to improve spinal cord perfusion. As internal iliac arteries are important collaterals in the spinal cord perfusion network, the patency of right internal iliac artery was preserved by an IBD endograft. Also, the mean artery pressure was kept above 80 mmHg to maintain spinal cord perfusion pressure. Indeed, this patient did not develop spinal cord ischemia during hospital stay or follow-up.
Conclusion
Total endovascular repair of stable TAAA with CMD branched component in BD patient was feasible, microinvasive and safe. The long-term efficacy needs to be observed.
Footnotes
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: Mian Wang is supported by ‘China Scholarship Council’ from Ministry of Education of the People’s Republic of China.
