Major Artery Aneurysms Research Articles

Treatment strategies of ruptured intracranial aneurysms associated with moyamoya disease

Objective The purpose of this study was to present our experience in the management of ruptured intracranial aneurysms associated with moyamoya disease (MMD), and to discuss their treatment strategies and the timing of revascularization surgery. Patients and methods Thirteen patients who had ruptured intracranial aneurysms associated with MMD were enrolled in this study. Different treatment strategies were adopted based on the location of the aneurysms. Their clinical and radiologic features, treatment selection and outcomes were retrospectively reviewed. Results Among the five patients with major artery aneurysms in anterior circulation, three were embolized and two clipped. Among the five patients with major artery aneurysms in posterior circulation, three were treated by endovascular coiling. Among the three peripheral aneurysms, one was treated by endovascular embolization, one by aneurysmectomy, and the other one by revascularization alone. For the patients whose aneurysms were treated by endovascular embolization or surgery, a staged revascularization was performed on day 28 to day 87 after the first operation. For the two patients with aneurysms untreated directly, the timing of revascularization was 20 days and 54 days after hemorrhage, respectively. During the follow-up recurrent intracranial hemorrhage occurred in a patient, but not caused by the previous aneurysm. No other patients suffered recurrent intracranial hemorrhage or ischemic stroke. Complete occlusion was achieved in all the 11 aneurysms that had been clipped or embolized. Of the remaining three aneurysms that had not been directly treated, one disappeared spontaneously, whereas the other two remained stable. The direct and indirect bypasses were confirmed patent in the 11 patients who had undergone revascularization. Conclusion Our current treatment strategies and timing of revascularization may provide a benefit for the patients with MMD accompanied by ruptured intracranial aneurysms.

The prospects and pitfalls in the endovascular treatment of moyamoya disease-associated intracranial aneurysms.

Moyamoya disease (MMD) is characterized by progressive stenosis or occlusion of the distal internal carotid artery and simultaneous formation of collateral vasculature. The fragile alteration and increased hemodynamic stress in the intra- and extracranial vasculature would conjointly result in the formation of intracranial aneurysms in MMD patients. According to our classification, the MMD-associated aneurysms are divided into the major artery aneurysms (MAAs) and non-MAAs. The non-MAAs are further subdivided into the distal choroidal artery aneurysms, moyamoya vessel aneurysms, transdural collateral aneurysms, and anastomosis aneurysms. Currently, endovascular treatment (EVT) has become the main stream for the MMD-associated aneurysms. There is no difference to EVT for the MMD-associated MAAs of the non-stenosed major arteries with that in the non-MMD patients. While it is a big challenge to perform EVT for MMD-associated aneurysms in the stenosed arteries. Generally speaking, the parent arteries of the non-MAAs are slim, and super-selective catheterization is technically difficult. Most of the times, parent artery occlusion with liquid embolic agents or coils can only be performed. The vasculature in MMD patients is fragile; perioperative management and meticulous intraoperative manipulation are also very important to avoid complications during EVT. In spites of the complications, the EVT can bring good outcome in selected cases of MMD-associated aneurysms.

Clinical Features and Outcomes of Intracranial Aneurysm Associated with Moyamoya Disease.

Background and PurposeMoyamoya disease (MMD) is a rare form of intracranial stenoocclusive disease that can be associated with intracranial aneurysms. We evaluated the clinical features and outcomes of MMD-associated aneurysms while focusing on their locations.MethodsBetween January 1998 and December 2018 there were 1,302 adult and pediatric patients diagnosed as MMD at a single institution. These patients included 38 with 44 MMD-associated aneurysms. The MMD-associated aneurysms were classified into two groups based on their locations: major-artery aneurysms and non-major-artery aneurysms. The clinical and radiological data for patients with MMD-associated aneurysms were reviewed retrospectively.ResultsThe 44 MMD-associated aneurysms comprised 28 in major arteries and 16 in nonmajor arteries. All of the major-artery aneurysms were initially unruptured lesions, and follow-up angiography showed that 23 (82.1%) had an improved or stable status and 5 (17.9%) had a worse status. The non-major-artery aneurysms comprised 10 ruptured and 6 unruptured lesions, and follow-up angiography showed that 11 (68.8%) had improved or were stable and 5 (31.2%) had worsened. At the latest follow-up, there were four cases of unfavorable outcome: two initial hemorrhagic insults, one treatment-related morbidity, and one repeated-hemorrhage case.ConclusionsMMD-associated aneurysms occurred in 3.3% of the MMD cohort in this study, of which 63.6% were major-artery aneurysms and 36.4% were non-major-artery aneurysms. The major-artery group included 17.9% that became angiographically worse, while 31.2% were growing or hemorrhaging in the non-major-artery group.

Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities.

Rendu-Osler-Weber syndrome, or hereditary hemorrhagic teleangiectasia (HHT), is a rare autosomal dominant vascular disorder, characterized by multiple mucocutaneous teleangiectases with recurrent nasal and gastrointestinal bleedings and/or solid-organ arteriovenous shunts. We describe the first case to our knowledge of pancytopenia in a 53-year-old patient, with a known history of HHT and recurrent nasal and gastrointestinal bleedings, who was found to have a major splenic artery aneurysm and other uncommon vascular abnormalities. In the absence of other evident causes of pancytopenia, hypersplenism was diagnosed. The patient underwent coil embolization of the splenic artery aneurysm, followed by rapid and sustained increase of white blood cell and platelet count. Splenic artery aneurysms are extremely uncommon in HHT as only anecdotal cases have been reported to date. However, we believe that the aneurysm critically contributed to the progression of splenomegaly and the development of pancytopenia.

Abstract TP83: Impaired Cerebrovascular Reactivity to Acetazolamide in Parent Arterial Territory of Major Artery Aneurysms in Adult Patients with Moyamoya Disease

Background— Recent studies have suggested that haemodynamic stress probably induces the formation of major artery aneurysms around the circle of Willis in adult patients with moyamoya disease (MMD). Theoretically, cerebrovascular reactivity (CVR) to acetazolamide (ACZ) is gradually reduced in the conditions that cerebral perfusion pressure is decreased. If arterial narrowing progress, CVR to ACZ would be reduced (Kuroda’s Type 2 or 3 ischemia) in the parent arterial territory of these aneurysms owing to elevated cerebrovascular resistance. This study was aimed to test the hypothesis whether the CVR to ACZ is reduced in the parent arterial territory of the major artery aneurysms. Methods— This retrospective study included 172 adult patients with MMD diagnosed at our institutes between 1980 and 2011. Eight major artery aneurysms was found in 7 (4.1%) of these patients, including 5 women and 3 men at the mean age of 47.4 years. Aneurysm location and perfusion territory of the parent artery were determined on digital subtraction angiography. Regional cerebral blood flow (CBF) was quantitatively measured, using 123 I-IMP SPECT. Subsequently, ACZ was intravenously injected to determine the reactivity of CBF. CVR to ACZ in the parent arterial territory was calculated (n=8). CVR in the contralateral arterial territory of the parent artery was utilized as a control (n=6). Results— Four (50%) of 8 major artery aneurysms were located in the vertebrobasilar system. All aneurysms were saccular and small, ranging from 2.0 and 5.0 mm. CVR to ACZ were reduced in 5 (62.5%) of 8 parent arterial territory (Type 2 or 3). All the contralateral arterial territory of the parent artery had normal CBF and CVR to ACZ (Type 1). Compared with contralateral side (32.0 ± 10.7%), CVR to ACZ was not significantly reduced in the parent artery side (16.5 ± 19.0%, P=0.11; paired-t). Fisher’s exact test, however, demonstrated a significant association between the existence of major artery aneurysms and reduced CVR to ACZ in the parent arterial territory (P=0.03). Conclusions— These findings suggest that reduced CVR in the parent arterial territory of the major artery aneurysms indicates underlying hemodynamic vascular stress inducing aneurysm formation in adult patients with MMD.

Major Arterial Aneurysms and Pseudoaneurysms in Behcet's Disease: Results from a Single Centre

Objective: Behcet’s disease (BD) with arterial involvement is closely correlated with mortality and morbidity due to life-threatening complications such as arterial occlusion and aneurysm rupture. We aimed to determine the clinical characteristics of BD patients with aneurysms and pseudoaneurysms in the major arterial systems. Methods: Medical records of 30 BD patients diagnosed with aneurysms or pseudoaneurysms in the major arterial systems were reviewed to determine the clinical characteristics of BD, the sites and types of arterial aneurysms or pseudoaneurysms, laboratory test results, and response to treatment. Results: A total of 47 aneurysms and pseudoaneurysms (32 saccular aneurysms, eight fusiform aneurysms, and seven pseudoaneurysms) were detected in 30 patients. Most aneurysms and pseudoaneurysms (27 patients, 90%) had not ruptured. Symptomatic lesions presented in 21 patients (70%), and asymptomatic lesions were incidentally detected in nine (30%). Ten of the 30 patients (33.3%) presented two or more aneurysmal lesions. Recurrence was observed in five patients (16.7%) after treatment with stent graft (n = 3), graft interposition (n = 1), or graft embolization (n = 1). Conclusion: We suggest that BD patients diagnosed with major arterial aneurysms should be further evaluated to detect possible associated venous or arterial thrombosis formations or aneurysmal lesions at other sites.

Major arterial aneurysms and pseudoaneurysms in Behçet's disease: results from a single centre

Major arterial aneurysms and pseudoaneurysms in Behçet's disease: results from a single centre

Incidental major artery aneurysms in patients with non-hemorrhagic moyamoya disease

Major artery aneurysms may be found incidentally while evaluating moyamoya disease (MMD). The purpose of this study was to delineate the prevalence and characteristics of these uncommon aneurysms with a brief mention of their management and outcomes. We conducted a retrospective review of 249 adult patients who were diagnosed with non-hemorrhagic MMD. Cerebral angiograms obtained at the time of initial diagnosis were carefully reviewed to identify incidental aneurysms located on a major intracranial artery or near its tributaries. Aneurysms originating from distal peripheral arteries or moyamoya vessels were ineligible for this study. Nine patients (3.6%) were found to have 13 major artery aneurysms, 7 (54%) of which were located in the posterior circulation, especially in older patients with bilateral MMD. The sizes of all aneurysms were less than 10mm. Surgical clipping was performed on two aneurysms, and endovascular coiling on six (five posterior circulation aneurysms). Ischemic complications occurred in two patients after clipping of an anterior communicating artery aneurysm and in one patient after the second coiling of a recanalized basilar tip aneurysm. Incidental major artery aneurysms can be found in 3.6% of adult patients with non-hemorrhagic MMD, an observed frequency that increases with age. About half of these aneurysms are located in the posterior circulation, particularly in older patients with bilateral MMD. Considering the risks of treatment-related complications, more information about the natural course of these aneurysms is needed to design proper management strategies both for the aneurysms and MMD.

Ruptured Intracranial Aneurysms Associated With Moyamoya Disease -Three Case Reports-

Three cases of ruptured intracranial aneurysm associated with moyamoya disease are presented. Endovascular treatments were performed successfully in two patients with major artery aneurysms. One patient with a collateral aneurysm was managed conservatively and follow-up angiography 1 year later demonstrated spontaneous disappearance of the aneurysm. Our experience suggests that although aneurysms associated with moyamoya disease show differences in evolution and location, endovascular treatment of major artery aneurysms is safe and effective, and peripheral aneurysms which cannot be directly accessed for surgery or endovascular embolization may be treated conservatively.

Major arterial aneurysms and pseudoaneurysms in Behçet's disease: results from a single centre

Objective: Behçet's disease (BD) with arterial involvement is closely correlated with mortality and morbidity due to life-threatening complications such as arterial occlusion and aneurysm rupture. We aimed to determine the clinical characteristics of BD patients with aneurysms and pseudoaneurysms in the major arterial systems.Methods: Medical records of 30 BD patients diagnosed with aneurysms or pseudoaneurysms in the major arterial systems were reviewed to determine the clinical characteristics of BD, the sites and types of arterial aneurysms or pseudoaneurysms, laboratory test results, and response to treatment.Results: A total of 47 aneurysms and pseudoaneurysms (32 saccular aneurysms, eight fusiform aneurysms, and seven pseudoaneurysms) were detected in 30 patients. Most aneurysms and pseudoaneurysms (27 patients, 90%) had not ruptured. Symptomatic lesions presented in 21 patients (70%), and asymptomatic lesions were incidentally detected in nine (30%). Ten of the 30 patients (33.3%) presented two or more aneurysmal lesions. Recurrence was observed in five patients (16.7%) after treatment with stent graft (n = 3), graft interposition (n = 1), or graft embolization (n = 1).Conclusion: We suggest that BD patients diagnosed with major arterial aneurysms should be further evaluated to detect possible associated venous or arterial thrombosis formations or aneurysmal lesions at other sites.

The 9p21 Myocardial Infarction Risk Allele Increases Risk of Peripheral Artery Disease in Older People

A common variant at chromosome 9p21 (tagged by the rs1333049 or rs10757278 single-nucleotide polymorphism) is strongly associated with myocardial infarction and major arterial aneurysms. An association with peripheral arterial disease (PAD) was also reported in a sample younger than 75 years, but this disappeared on removal of respondents with a myocardial infarction history, resulting in an odds ratio of 1.09 for PAD (P=0.075). We aimed at estimating the association of this variant with an Ankle-Brachial Index (ABI) and PAD in 3 older populations. We used data from the InCHIANTI, Baltimore Longitudinal Study of Aging, and Health, Aging, and Body Composition studies. In 2630 white individuals (mean age, 76.4 years), the C allele at rs1333049 was associated with lower mean ABI measures and with an increased prevalence of PAD. These associations remained after removal of baseline and incident myocardial infarction cases over a 6-year follow-up for both ABI (-0.017 ABI units; 95% CI, -0.03 to -0.01; P = 1.3 x 10(-4)) and PAD (per allele odds ratio, 1.29; 95% CI, 1.06 to 1.56; P = 0.012). These associations also remained after adjustment for known atherosclerosis risk factors, including diabetes mellitus, smoking, hypercholesterolemia, and hypertension. The C allele at rs1333049 is associated with an increased prevalence of PAD and lower mean ABI. This association was independent of the presence of diagnosed myocardial infarction and atherosclerotic risk factors in 3 older white populations.

Endovascular Treatment of a Superior Mesenteric Artery Aneurysm Secondary to Behcet’s Disease with Onyx (Ethylene Vinyl Alcohol Copolymer)

Behcet's disease is a complex multisystemic chronic inflammatory disease that is characterized by oral and genital aphtous ulcers and vasculitis. Aneurysms of major arteries are the most important cause of mortality in Behcet's disease. Four patients with superior mesenteric artery (SMA) aneurysms related to Behcet's disease have been reported in the literature. We report here the first successful endovascular treatment of a giant, wide-necked SMA aneurysm secondary to Behcet's disease. We performed a balloon-assisted embolization technique using ethylene vinyl alcohol copolymer (Onyx, ev3, Irvine, CA, USA). There were no signs of recurrence during 2-year follow-up.

Major renal artery aneurysm as cause of hydronephrosis treated by renal preservation surgery

We report a case of a giant renal artery aneurysm causing hydronephrosis that initially had erroneously been thought to be a renal cyst. Giant renal artery aneurysms greater than 10 cm in diameter are very rarely reported. The aneurysm was repaired successfully with kidney preservation and closure of the arterial fistula.

Aneurysm of Arteriovenous Fistula in Uremic Patients: Is Endograft a Viable Therapeutic Approach?

One of the complications of arteriovenous fistulas in chronic hemodialyzed patients is the onset of an aneurysm which can be at risk of rupture. Traditional surgical repair is not always feasible and may not be successful in these cases, leading therefore to the loss of a functioning vascular access and requiring in any case the temporary use of a central venous catheter to allow regular hemodialysis sessions. We applied to this kind of aneurysm the same experience developed in the management of major arterial aneurysms and we considered endografting repair a good alternative in this case. In this paper we present the successful treatment of an arteriovenous fistula aneurysm using that technique. A distal radio-cephalic arteriovenous fistula in one of our patients presented an aneurysm with high risk of rupture. The endografting repair with percutaneous insertion of a WallgraftTM endoprosthesis was well tolerated and the vascular access could be used the day after, without the need for a central venous catheter insertion.

Direct surgery for major artery aneurysm associated with Moyamoya disease

Moyamoya disease is often accompanied by intracranial major artery aneurysms in the posterior circulation which acts as collateral channels in place of the stenotic internal carotid arteries. These major artery aneurysms are considered to have high risk of enlargement and rupture due to increased hemodynamic stress. Direct surgical intervention has been recommended for the treatment of these aneurysms, but the direct approach to them is often difficult due to interference by intertwined abnormal vessels. We have performed direct surgery for seven major artery aneurysms in five patients with Moyamoya disease. Of these three aneurysms located in the anterior circulation were successfully clipped via pterional or interhemispheric approach. Of four posterior circulation aneurysms (two at the junction of the basilar artery and the superior cerebellar artery and two at the P1–P2 junction of the posterior cerebral artery), one was approached via pterional route because collateral vessels in the basal cistern was judged not to be rich on angiograms. However, the operative field was interfered by abundant fragile collateral vessels and it was difficult to reach the distal portion of the basilar artery. In contrast, in the other three cases in which the subtemporal approach was employed, there weren't any problems in exposures of the aneurysms. Our experiences indicate that subtemporal approach is superior than the pterional approach to reach the distal portion of the basilar artery in patients with Moyamoya disease.

Thoracoabdominal aorta in Marfan syndrome: MR imaging findings of progression of vasculopathy after surgical repair.

To determine the natural history of vasculopathy of the thoracoabdominal aorta in patients with Marfan syndrome after composite graft repair of the aortic root. A total 224 magnetic resonance (MR) images obtained in 48 patients with Marfan syndrome over a period of 2.3-9.4 years (mean, 5.0 years) were retrospectively reviewed. On each image, the diameter of the thoracoabdominal aorta was measured and the presence of dissection or major peripheral artery aneurysms was determined. In 31 (65%) of the 48 patients, no statistically significant change (3 mm or less increase in diameter) in the diameter of the aorta occurred during the study (group 1); in the remaining 17 (35%) patients, a significant change occurred (greater than 3 mm increase) (group 2). The mean initial diameter of the native aorta was slightly larger in group 2 (mean, 27 mm +/- 8 [standard deviation]) than in group 1 (mean, 23 mm +/- 6). In group 1, the mean rate of dilation was 0.07 mm/y +/- 0.2; in group 2, the rate was 2.3 mm/y +/- 3.3. Two patients with aortic dissection were in group 1, whereas 14 such patients were in group 2 (P < .001). Aneurysms that involved major peripheral arteries were present in four (13%) of the 31 group 1 patients and in 12 (71%) of the 17 group 2 patients (P < .001). Surgical intervention was necessary in two group 1 patients and in 14 group 2 patients (P < .001). A subset of patients with Marfan syndrome manifested multiple forms of vasculopathy, including progressive aortic dilation, dissection, and peripheral artery aneurysm after composite aortic graft repair of the ascending aorta. Patients with these characteristics merit more frequent MR follow-up since further surgery was often necessary in these individuals.

Intracranial Aneurysms in Moyamoya Disease: Report of Ten Cases and Review of the Literature

The occurrence of unilateral and bilateral moyamoya disease may be associated with intracranial aneurysms. These aneurysms are of two types: major-artery aneurysms and peripheral-artery aneurysms. The

Asymptomatic Aneurysms and HIV Infection

Aneurysms of major cerebral arteries in two children, aged 11 and 12 years, infected with human immunodeficiency virus from blood transfusions are reported from the National Institutes of Health, Bethesda, MD, and Georgetown University Hospital, Washington, DC.

Cerebral artery aneurysms in children infected with human immunodeficiency virus

More than 250 children treated at our institution on antiretroviral treatment protocols have been monitored with brain imaging studies. We documented the occurrence and progression of aneurysms of major cerebral arteries in two children with advanced human immunodeficiency virus infection. In both cases these lesions remained clinically silent initially, despite progression to marked dilation.

Moyamoya Vessels associated with Multiple Cerebral Aneurysms

A case of multiple cerebral aneurysms associated with so-called moyamoya disease is reported. The patient was a 52-year-old male attacked by subarachnoid hemorrhage. The patient was confused and signs of meningeal irritation were noted. Computed tomography (CT) scan showed the ventricular hemorrhage. Right carotid angiogram showed moyamoya vessels in the basal ganglia. The other angiograms showed three aneurysms, which were located at the origin of the posterior communicating artery (ICA-PCA) on the left, at the posterior cerebral-posterior communicating artery origin (PCA-PcomA) on the right, and the distal site of the posterior choroidal artery (PchA) on the left. Two aneurysms located at the origin of PCA and ICA were clipped by the transsylvian approach, the former being reached through the moyamoya vessels. Spontaneous regression of an aneurysm at the peripheral portion of PchA were angiographically documented 10 days after the second operation. There were no changes around the basal ganglia on post-operative CT. Histological examination of a part of the flat and small middle cerebral artery (M1) gained during operation revealed proliferation of intima, elongated elastica interna and loss of media. Forty-four reported cases of so-called moyamoya disease with aneurysm were discussed. As for operative indication of the aneurysm associated with moyamoya vessels, the authors consider that the aneurysm classified after Okamoto, et al. as Type III or major artery aneurysm, was almost saccular and the operative approach does not always injure moyamoya vessels.