A 25-year-old man was transferred to our hospital owing to a clinical suspicion of abdominal aortic aneurysm. The patient reported persistent left upper abdominal pain for one month. He also had recurrent oral ulcers for 5 years 7–8 times each year. Four years earlier, he underwent carotid artery stenting due to rupture of a pseudoaneurysm of the right internal carotid artery, followed by pseudoaneurysm formation at the puncture site of the right femoral artery, and bypass surgery with a prosthetic graft was performed. He had genital ulcers along with nodular rash and folliculitis in both lower limbs for one year. A computed tomography angiography (CTA) performed prior to admission to our hospital demonstrated a 58-mm-diameter pseudoaneurysm located in his abdominal aorta at the level of the superior mesenteric artery (SMA). The proximal end of the aneurysm was just below the celiac artery (CA), and the distal end was just parallel to the right renal artery. The proximal portion of the SMA was totally occluded due to compression of the pseudoaneurysm, while the distal runoff of the SMA was patent through collateral circulation from the CA and inferior mesenteric artery (IMA) (Fig. 1). As measured by preoperative CTA, the average diameter of the aorta at 20 mm above the CA was 21 mm. At 20 mm below the right renal artery, the average diameter of the aorta was 17 mm.
The patient was diagnosed with vasculo-Behcet’s disease and received immunosuppressive medication before surgical intervention. Methylprednisolone 40 mg combined with 560 mg of tocilizumab, later replaced by 30 mg of prednisone daily; 1 g of mycophenolate mofetil (MMF) twice daily; and 10 mg of methotrexate (MTX) weekly were administered. Laboratory findings showed a C-reactive protein level of 10.87 mg/l and an erythrocyte sedimentation rate (ESR) of 2 mm/h before the operation.
Endovascular repair with physician-modified fenestrated endograft (PMFE) was planned for this patient. After a 22–80 mm Ankura cuff endograft (Lifetech, Shenzhen, China) was deployed on a sterile back-table in the operating room, three fenestrations were created according to perioperative planning: 8*8 mm at 15 mm from the proximal top of the endograft and in the 12:00 o’clock direction for the CA; 6*6 mm at 54 mm distal to the “o” mark of the stent graft and in the 21:00 o’clock direction for the right renal artery (RRA); and 6*6 mm at 60 mm distal to the “8” mark of the stent graft and in the 9:00 o’clock direction for the left renal artery (LRA) (Fig. 2). Fenestrations were reinforced by suturing metal coils (COOK Medical, Bloomington, IN, USA) as radiopaque markers. Finally, the endograft was re-encased in the sheath of the delivery system.
Before the procedure, bone and aortic 3D models were reconstructed from the preoperative CTA scan on a workstation (Advantage Workstation; GE Healthcare). These models were then fused with live fluoroscopy (Fig. 3A). Two Cobra catheters were advanced through the left femoral artery into both renal arteries for protection. The PMFE was deployed from the right femoral artery. Accurate deployment of the PMFE and orientation of the hole towards the CA was guided by a 3D fused image. A 5–40 mm balloon (Powerflex, Cordis) was delivered into the CA to facilitate adjustment of the PMFE for orientation of the holes towards the RAs. After successful cannulation of the two renal arteries from the left brachial artery, the two Cobra catheters were removed, and two 6–25 mm self-expanding covered stents (Viabahn, Gore) were implanted in both renal arteries. The proximal ends of both stents were flared by a 10–40 mm balloon (Advance 35LP, COOK). The PMFE was completely deployed, and the delivery system was retrieved. Completion angiography demonstrated that the pseudoaneurysm was completely excluded, and no endoleak was observed. The CA and RAs were patent, and perfusion of the distal segment of the SMA through collateral arteries was satisfactory (Fig. 3B). The total procedure time was 150 minutes, fluoroscopy time was 32 minutes, intraoperative bleeding was 100 mL, and the contrast volume used was 80 mL.
After endovascular treatment, the abdominal pain of this patient was obviously relieved. He received immunosuppressive therapy with 40 mg of intravenous methylprednisolone daily perioperatively and changed to 25 mg of oral prednisolone daily after discharge. MMF and MTX treatment was continued with supplementation of calcium and vitamin D.
At the 18-month follow-up, CTA demonstrated successful exclusion of the pseudoaneurysm and patent CA, SMA and RAs (Fig. 4). Oral prednisolone was continued and suggested to be reduced by 2.5 mg every 2 weeks. ESR and CRP levels were within the normal ranges.