Hereditary aortic aneurysms and dissections, such as Marfan syndrome, differ in that they occur in younger patients without generally recognized risk factors, have a predilection for the thoracic rather than the abdominal aorta, and are at risk for dissection even at smaller aortic diameters. Early diagnosis, careful follow-up and early intervention, such as aggressive antihypertensive treatment and prophylactic aortic replacement to prevent fatal aortic dissection, are essential for better prognosis. Molecular genetic testing is extremely useful for early diagnosis. However, in actual clinical practice, the question often arises as to when and to which patients genetic testing should be offered, since the outcome of the tests can have important implications for the patient and the relatives. Pre- and post-test genetic counseling is essential for early intervention to be effective.

Objective: Celiac artery aneurysm is a relatively rare disease, but the number of incidentally discovered cases is rising with the increased use of imaging examinations for diagnosis. We herein report cases of celiac artery aneurysms incidentally discovered at our facility. Methods: We focused on 20 cases of celiac artery aneurysms incidentally discovered at our facility in the past 10 years. Results: The mean aneurysm diameter at the time of diagnosis of the cases of incidentally discovered celiac artery aneurysms was 15.1 mm, and 15 cases were fusiform aneurysms (75.0%). In terms of etiology, dissection was suspected in 8 cases (40.0%), whereas 9 cases had compression by a median arcuate ligament (45.0%). Regarding treatment, 16 cases were followed up (80.0%), 4 cases received endovascular treatment (coil embolization), and 1 case underwent surgical operation caused by increased aneurysm diameter following coil deviation. Conclusion: Many cases of incidentally discovered celiac artery aneurysms did not show a tendency to enlarge, and most could be followed up. In cases where treatment is indicated, it is crucial to consider the localization of aneurysms and the risk of organ ischemia when developing treatment strategies.

Objective: To evaluate the early clinical outcomes of endovascular aneurysm repair (EVAR) with physician-modified branched endoprosthesis for iliac artery aneurysms unsuitable for manufactured iliac branch endoprosthesis (W. L. Gore & Associates Incorporated, Flagstaff, AZ, USA). Methods: A total of 8 cases of iliac artery aneurysms between October 2019 and April 2023, underwent EVAR with physician-modified branched endoprosthesis. The endoprosthesis was made of AFX leg extension (Endologix Incorporated, Irvine, CA, USA) with hydrogel-reinforced fenestration to provide a secure connection with the branching graft. Their early clinical outcomes and computed tomography (CT) data were retrospectively analyzed. Results: Viabahn (W. L. Gore & Associates Incorporated, Flagstaff, AZ, USA) were used as branching grafts for the reconstruction of internal iliac artery or superior gluteal artery in 7 cases. Moreover, coil embolism of inferior gluteal artery was performed in 3 cases. Although 1 case had a type 2 endoleak from the iliolumbar artery, a reduction of aneurysmal diameter was observed in all of the cases. Furthermore, no complications such as buttock claudication were observed in patients. Conclusion: EVAR with physician-modified branched endoprosthesis for the iliac artery aneurysm was less costly, performed within a reasonable time, and demonstrated favorable early outcomes with respect to preventing buttock claudication and the reduction of the aneurysmal diameter.

We report an extremely rare case of ruptured tuberculous external iliac artery aneurysm. A 75-year-old woman was admitted to another hospital 8 months ago for pleural inflammation and was treated with antibiotics. Mycobacterium tuberculosis was detected in the pleural fluid submitted at that time. She had been suspected of having an iliopsoas abscess by her local doctor due to left lower abdominal pain and fever for 2 weeks, and was treated with antibiotics, but the symptoms did not improve. A contrast-enhanced CT scan was performed, and a diagnosis of ruptured infected left external iliac artery aneurysm was made. Emergency laparotomy was performed, and the patient underwent aneurysmectomy, drainage, and extra-anatomical bypass with rifampicin-immersed Dacron graft. Tubercle bacilli were detected locally, and histopathological examination revealed specific tuberculous lesions on the aneurysmal wall, leading to the diagnosis of tuberculous aneurysm. The patient was discharged from the hospital on the 68th postoperative day after with anti-tuberculosis drugs. The patient continued chemotherapy for one year after surgery, and no recurrence has occurred for more than five years after an end of chemotherapy.

The goal of thoracic endovascular aortic repair (TEVAR) for chronic Stanford type B aortic dissection (TBAD) is to avoid rupture by inducing false lumen thrombosis. However, branch arteries originated from false lumens have been reported to cause residual blood flow and inhibit thrombosis, resulting in false lumen dilatation even after TEVAR. We describe a chronic TBAD case in which intercostal arteries embolization combined with TEVAR promoted false lumen thrombosis leading to favorable aortic remodeling 1 year after the procedure. The patient was a 49-year-old man with a thoracic aortic aneurysm found incidentally on computed tomography (CT). The dissection onset time was unclear as he had not displayed any prior symptoms, but the images of aortic wall (which were thickened and had an unmovable septal flap) suggested a chronic dissection. The patient was young enough to tolerate open surgery, so a 2-staged hybrid procedure was planned. A total arch replacement combined with frozen elephant trunk was performed with moderate hypothermic circulatory arrest (28°C) and selective antegrade cerebral perfusion. Three months later, we performed TEVAR with intercostal artery embolization. During the procedure, false lumen angiography revealed 7 intercostal arteries originating from the false lumen, and we embolized 4 of these with orifices enough wide to insert a catheter. Subsequently, we performed TEVAR to close intimal tears of the descending thoracic aorta. The postoperative course was uneventful, and the patient was discharged on the eighth day after surgery without complications. Postoperative CT scans after 1 year showed false lumen shrinkage and favorable aortic remodeling. This case indicates that intercostal artery embolization combined with TEVAR in chronic TBAD could be effective in promoting thrombosis in false lumens, but further study is needed to confirm the types and numbers of arteries which should be embolized to promote these effects.

A 76-year-old man who had undergone open graft replacement for a ruptured abdominal aortic aneurysm. The abdominal aorta on the central side of the artificial vessel replacement became aneurysm, the patient underwent stent graft insertion with abdominal debranching. On postoperative day 78, a Computed Tomography (CT) showed an abscess cavity, which was diagnosed as graft infection. We decided that re-open graft replacement and peripheral revascularization and open drainage would be difficult to tolerate due to the invasive of the surgery and his general condition. CT-guided puncture was performed in the supine position, and a catheter was placed in the abdominal aortic aneurysm contiguous with the abscess cavity, followed by open drainage, saline flushing, and local and systemic administration of antibiotics. Fever and inflammatory reaction improved, and the patient continued to have no recurrence after removal of the catheter, and was discharged.

The patient was a 79-year-old man transported to our critical care center with a complaint of sudden severe pain in the lower right abdomen. At admission, he was in shock with a systolic blood pressure 70 mmHg. Contrast-enhanced computed tomography revealed a right external iliac aneurysm with maximum minor diameter of 57 mm and a hematoma surrounding the aneurysm, and the patient was diagnosed as having a ruptured right external iliac aneurysm. Emergency graft replacement was performed on the same day. The patient’s postoperative course was generally good, and he was discharged on postoperative day 30. We report a rare case of rupture of an isolated external iliac aneurysm with review of the literature.

An 80-year-old man with stomach pain during dialysis was admitted to our hospital. Abdominal contrast-enhanced computed tomography (CT) and angiography showed superior mesenteric artery (SMA) stenosis and inferior mesenteric artery (IMA) occlusion; therefore, chronic mesenteric ischemia (CMI) was diagnosed. It was suggested that endovascular procedure may not be suitable because of severe calcification of the aorta. Accordingly, surgical revascularization was planned. Furthermore, considering that the abdominal aorta and right iliac artery were significantly calcified, anti-anatomical route bypass from left external iliac artery with great saphenous vein was selected. Postoperatively, the patient did not develop stomach pain.

Arterial thrombosis is a rare complication of nephrotic syndrome. Herein, we report a case of acute arterial occlusion and abdominal aortic thrombosis in a 54-year-old patient with nephrotic syndrome. While under evaluation for bilateral limb edema, the patient complained of right limb pain and numbness. Computed tomography revealed right popliteal artery thromboembolism and abdominal aortic thrombosis. We performed emergent surgical thrombectomy for the right limb and introduced anticoagulant therapy. Three days postoperatively, computed tomography showed reocclusion of the right popliteal artery. We performed hybrid surgery with thrombectomy and balloon angioplasty, and used an antithrombin agent for postoperative anticoagulant therapy. No thromboembolism recurrence was observed and the abdominal aortic thrombosis was reducing. This case highlights the importance of recognizing abdominal aortic thrombosis and limb thromboembolism as complications of nephrotic syndrome because they may result in an unfavorable outcome, like amputation.

The patient was a 72-year-old man. He admitted to the hospital with a chief complaint of abdominal pain, and a CT scan revealed abdominal aortic and iliac artery aneurysms. He had a horseshoe kidney with the inferior pole branch of the left renal artery whose orifice was located 76 mm proximally from the terminal aorta. The iliac artery aneurysm was 35 mm in diameter and there were comorbid risks including a history of cerebral hemorrhage; therefore, we performed endovascular aneurysm repair. We initially considered AFX as the device of choice for short distances from terminal aorta; however, there seemed to be a risk of covering the branch orifice by the AFX fabric due to the severe aortic angulation. We finally selected Gore Excluder C3 whose proximal stent can be squeezed after partial deployment for “repositioning”. We inserted the balloon and dilated in the contralateral gate and carefully pulled whole device downward and repositioned it. After the operation, there were no endoleaks, the pole renal branch was patent, and the renal function did not deteriorate.

The patient was a 79-year-old man. He had a history of atrial fibrillation and was taking warfarin. In August 2021, he was hospitalized with COVID-19 pneumonia at the previous hospital and started treatment with remdesivir and dexamethasone. During treatment, symptoms of ischemia in the right upper limb appeared, and despite medical treatment, the symptoms gradually worsened. On the 8th hospital day, necrosis of the second finger appeared, and CT showed thrombotic occlusion of the right brachial artery. He was transferred to our hospital for advanced therapy. We performed emergent thrombectomy with the Fogaty catheter and the organized thrombus at the bifurcation of the brachial artery was removed. However, recurrence of ischemic symptoms was observed on the 6th postoperative day (POD), and thrombectomy was performed again. Although the blood flow temporarily improved, it recurred in the evening and the thrombus was removed again. The improvement in blood flow was poor and the muscles of the forearm were swollen. So we perform fasciotomy additionally. After that, the ischemic state continued, and amputation was considered, but POD13 after the first operation, the respiratory condition deteriorated rapidly and progressed to ARDS. He died the next day.

The common iliac arteriovenous fistula (AVF) was previously performed by open surgery, but it was high risk due to the adhesions around the AVF and the possibility of massive bleeding because of hyper-vascularity. We herein report a case of endovascular repair of a common iliac AVF. An 84-year-old woman was admitted to our hospital because of left lower limb edema. She had a clinical history of abdominal total hysterectomy at age of 82 years. Enhanced computed tomography (CT) revealed an AVF between the right common iliac artery and the left common iliac vein. Because of the high risk of open surgery and severely frail, we conducted endovascular repair for the AVF using the GORE EXCLUDER stentgraft system. The first contralateral leg was deployed to occlude the right internal iliac artery, and the second leg was deployed over the aortic bifurcation to the right external iliac artery to seal the origin of the AVF. Postoperative enhanced CT confirmed occlusion of the AVF. The postoperative course was uneventful, and the left lower limb edema improved quickly.

Recently, endovascular treatment of renal artery aneurysms (RAAs) has been widely used. Especially, RAAs arising from the main renal artery trunk can be best managed with stent-graft treatment. We reported a case of 70-year-old man accidentally diagnosed with right RAA (29×22 mm saccular aneurysm at main renal artery trunk), coronary artery disease (left main trunk and three vessel disease), and left common iliac artery aneurysm (CIAA) by preoperative examination of an inguinal hernia. We treated his renal artery aneurysm with stent-graft treatment using Gore VIABAHN VBX following off-pump coronary artery bypass grafting for coronary artery disease. We decided to use VIABAHN VBX that could be more dilated using a larger balloon to adapt to different proximal and distal diameters of his renal artery. Postoperative enhanced computed tomography showed the aneurysm completely thrombosed, and endoleak was absent. After that, he received stent graft treatment for the CIAA, and his clinical course was good.

Ureter-iliac artery fistula (UAF) is a relatively rare disease but occasionally causes fatal complications. We report a case of right UAF. A 78-year-old man was admitted with postrenal failure due to right ureteral stenosis and treated by inserting a double-J stent, which was replaced regularly. Four years after starting ureteral stenting, he needed hemodialysis treatment. As the right double-J stent was removed, he complicated with hematuria and anemia. Cystoscopy showed a high number of clots in the bladder. Although the fistula was not confirmed in contrast-enhanced computed tomography scan, a right UAF was highly suspected. The fistula was treated by direct closure of the external iliac artery fistula and right nephroureterectomy. The postoperative course is good, with no hemorrhagic episodes.

There are few reports of IgG4-related vascular lesions involving arteries other than the aorta, and there are some cases in which imaging and pathological examinations did not show a typical picture. In this report, a case of an IgG4-related femoral aneurysm is described. The patient was a man in his 70s who was diagnosed with an aortic arch aortic aneurysm, a superior mesenteric aneurysm, bilateral common iliac aneurysms, and bilateral femoral aneurysms by a previous physician 7 years before his first visit to our department. Four years earlier, a previous doctor performed a prosthetic vascular replacement of the aortic arch to enlarge the diameter of the arch lesion. Computed tomography 4 years after surgery showed enlargement of the diameter of the right femoral aneurysm, and he was referred to our department. Immunological tests were performed for vasculitis, and IgG4 was high (702 mg/dL). Prosthetic vascular replacement of the aneurysm was performed, and a collection of IgG4-positive plasma cells was found in the aneurysm wall, leading to the diagnosis of IgG4-related vascular lesions. Surgical treatment was performed to prevent rupture, but there are reports that steroids suppress the expansion of blood vessel diameter in this disease, and it is necessary to carefully consider the possibility of medical treatment with early diagnosis.