A Rare Presentation of Polyarteritis Nodosa (PAN).

Polyarteritis nodosa (PAN) is a connective tissue disease that affects arteries, causing necrotizing inflammation that can weaken the arterial walls, dilatation into aneurysms, and rupture in some cases. We present a case of a male with acute abdomen from aneurysmal rupture. The 48-year-old patient with a history of polysubstance use including cocaine and methamphetamines was admitted for acute hypoxic respiratory failure secondary to coronavirus disease 2019 (COVID-19) pneumonia and treated with broad-spectrum antibiotics and steroids. He also reported generalized abdominal pain and discomfort, and examination revealed abdominal distension that was diffusely tender on palpation, bowel sounds intact. Laboratory workup showed a progressive drop in hemoglobin requiring blood transfusions, no coagulopathy, anion gap metabolic acidosis, and lactic acidosis. Abdominal CT showed a 2 cm lobulated saccular aneurysm involving either the left gastric artery or splenic artery, associated with an extensive moderate amount of hemoperitoneum with hematomas (largest measuring up to 8.6 cm) abutting the gastric fundus and greater curvature of the stomach, which was likely secondary to aneurysmal rupture. Additionally, several other mesenteric vessels displayed some degree of dilation. Interventional radiology (IR)-guided splenic artery embolization for splenic artery aneurysm was done, after which his hemoglobin remained stable. The patient was given vaccine recommendations since splenic artery embolization would lead to asplenia. The aneurysms were attributed to either cocaine-related aneurysms or polyarteritis nodosa with visceral artery aneurysms. He denied rashes, oral ulcers, joint pain, subcutaneous nodules, blood in the urine, history of hepatitis or syphilis. Tertiary syphilis was ruled out after the Venereal Disease Research Laboratory (VDRL) test and rapid plasma reagin (RPR) test were negative. Complement C3 and C4 levels were normal. He was treated with high-dose IV methylprednisone after infection was ruled out. Due to the severity of PAN, therapy with IV cyclophosphamide therapy 15 mg/kg once every two weeks for three doses was initiated, followed by 15 mg/kg once every three weeks for three to six months (in combination with glucocorticoids prednisone 1 mg/kg body weight with slow taper). Cyclophosphamide was given with IV hydration and mesna. The presentation of PAN can vary widely. Most commonly, individuals experience symptoms such as fatigue, weight loss, fever, and chills. However, in rare cases, patients may present with isolated abdominal pain, similar to our patient. It's crucial to note that the rupture of an aneurysm can manifest as an acute abdominal issue, potentially leading to life-threatening situations. Immediate interventions to control bleeding are imperative in such cases. The treatment of PAN has a high success rate when a combination of cyclophosphamide and steroids is administered.

Brain Imaging Modalities for Cavernous Sinus Pathology With Migraine Features: A Case Report.

This case report delves into the unusual presentation of a 38-year-old female with a history of migraines, who initially presented with a severe right-sided headache and changes in vision in her right eye, which gradually improved. Although she had consulted with an eye specialist for these issues, she did not receive targeted treatment. This case underscores the necessity of vigilant evaluation and early intervention in a patient with seemingly benign symptoms, thereby highlighting the potential gravity of underlying conditions such as aneurysms. Our analysis and description of this case provide insights for clinicians to consider comprehensive assessment and to explore less common etiologies, resulting in improved patient outcomes.

Intravascular lithotripsy supported endovascular aneurysm repair.

As the use of endovascular aneurysm repair (EVAR) increases, anatomic constraints remain a challenge. In this case report, we describe the use of intravascular lithotripsy to facilitate EVAR in a patient with a severely calcified and stenotic aortic bifurcation. Future applications of intravascular lithotripsy could help expand the use of EVAR to patients with severely stenotic vasculature and optimize outcomes in the treatment of infrarenal abdominal aortic aneurysms.

Endovascular Treatment of Wide-Neck Bifurcation Aneurysm: Recent Trends in Coil Embolization with Adjunctive Technique.

Wide-neck bifurcation aneurysms (WNBAs) are sometimes challenging to treat. During endovascular treatment, it is important to prevent coil deviation and preserve normal vessels. Adjunctive balloon- and stent-assisted techniques have been developed. A meta-analysis of endovascular treatments of WNBAs revealed that only 40% of patients had complete occlusion. Recently, novel devices have been developed to expand the range of treatment options. Flow-diverter stents and intra-aneurysmal flow disruption devices do not require coils; however, coil embolization remains the standard procedure used by many neurointerventionists. This review describes the recent trends in adjunctive techniques for supporting coil embolization for WNBAs. We referred to literature on balloon-assisted techniques, stent-assisted techniques, Y-stenting, PulseRider, Barrel stents, Comaneci temporary stents, pCONUS, and eCLIPs. These reports showed that adequate embolization rates were generally greater than 80%, and the complete occlusion rate was as high as 94.6%. All devices had a relatively high occlusion rate; however, it may be inaccurate to simply compare each device because of the heterogeneity of the studies. It is important to select the best treatment for each individual case by considering not only literature-based efficacy and safety but also patient background, aneurysm characteristics, and operator experience.

Endovascular Treatment for Intracranial Artery Dissections in Posterior Circulation.

Intracranial artery dissections (IADs), although uncommon, are an important cause of cerebral infarction and subarachnoid hemorrhage (SAH). Some IADs can heal spontaneously after reconstitution of the vessel lumen with excellent prognosis. Meanwhile, others can progress to stroke that requires treatment. The incidence of IAD in the posterior circulation is higher than that in the anterior circulation. Anterior circulation dissections are more likely to develop into ischemia and posterior circulation lesions into hemorrhage. The mortality rate after IAD among patients with SAH is 19%-83%. Further, the mortality rate of IAD without SAH is 0%-3%. Patients with SAH commonly undergo surgery or receive neuroendovascular treatment (EVT) to prevent rebleeding. However, the treatment of IADs is empirical in the absence of data from randomized controlled trials. Recently, EVT has emerged and is considered for IADs because of its less invasiveness and perceived low rates of procedure-related morbidity with good efficacy. EVT strategies can be classified into deconstructive (involving sacrifice of the parent artery) and reconstructive (preserving blood flow via the parent vessel) techniques. In particular, the number of reports on reconstructive techniques is increasing. However, a reconstructive technique for ruptured IADs has not yet been established. This review aimed to provide an overview of IADs in the posterior circulation managed with EVT by performing a literature search.

A Review of Current Flow Diverters.

Flow diverter (FD) devices are new-generation stents placed in the parent artery at the aneurysmal neck to obstruct intra-aneurysmal blood flow, thus favoring intra-aneurysmal thrombosis. In Japan, about eight years have passed since health insurance approval was granted for FD devices, and FD placement to treat aneurysms has become widespread. Treatment indications have also been expanded with the introduction of novel devices. At present, three types of FD (Pipeline, FRED, and Surpass Streamline) are available in Japan. This report represents a compilation of available FD technologies and describes the current consensus on this treatment.

Endovascular Treatment of Bifurcation Aneurysms with the Woven EndoBridge: Product Features and Selected Results of Off-Label Use.

Treatment for wide-neck bifurcation cerebral aneurysms (WNBAs) is widely performed by endovascular treatment as well as open surgical clipping. However, due to factors such as the shape and size of the aneurysms, as well as the anatomical features of surrounding branch vessels, there are some cases in which simple coiling or conventional adjunctive techniques, such as balloon-assisted or neck bridge stent-assisted coiling, are not sufficient to achieve a satisfactory cure. Against this backdrop, the device known as the Woven EndoBridge (WEB) (MicroVention, Aliso Viejo, CA, USA) was developed and can be deployed directly into the aneurysm for treatment. Over a decade has passed since its development, and it is now used in many countries worldwide. This review provides insights into the evolution of the WEB device from its development to the date of this writing, highlighting the unique features of the device and its treatment indications. Additionally, it discusses the posttreatment course, perspectives on recurrence and retreatment, imaging assessments, and potential off-label use based on numerous studies primarily conducted in Europe and the USA.

Endovascular Treatments for Aneurysms Involving a Major Branch.

Remarkable advances have been made in the endovascular treatment of intracranial cerebral aneurysms. These advances include various adjunctive techniques, increased indications for endovascular treatment, and improved treatment results. Furthermore, the number of cerebral aneurysm treatments using flow diverters (FDs) is expected to increase. However, the reported long-term rate of branch artery occlusion after FD treatment has been reported is 15.8%. Moreover, the complete aneurysm obliteration rate is low if normal branches arise from an aneurysm neck or dome. Flow diverter placement for ophthalmic artery, posterior communicating artery, and anterior choroidal artery aneurysms is often difficult because these normal branches often arise from the aneurysm neck or dome. Therefore, in many cases, coil embolization, which can occlude the aneurysm while preserving branch vessels, should be selected. Although not yet established, various adjunctive techniques and other endovascular treatments that can be performed safely have been reported. Treatment must be planned after understanding the advantages and disadvantages of each treatment method.

Reducing Abdominal Aortic Aneurysm Progression by Blocking Neutrophil Extracellular Traps Depends on Thrombus Formation.

Neutrophil extracellular traps (NETs) are implicated in the pathogenesis of abdominal aortic aneurysm (AAA), located in adventitia and intraluminal thrombus. We compared the therapeutic potential of targeting upstream or downstream effector molecules of NET formation in 2 murine AAA models based on angiotensin II or peri-adventitial elastase application. In both models, NETs were detected in formed aneurysms at treatment start. Although NET inhibitors failed in the elastase model, they prevented progression of angiotensin II-induced aneurysms with thrombus, which resembles established human disease (including thrombus development). Blockade of upstream NET mediators was more effective than interference with downstream NET molecules.

Single Branch Endovascular Aortic Repair Procedure for an Abdominal Aortic Aneurysm in a Patient With Horseshoe Kidney: A Case Report.

Introduction: Horseshoe kidney (HK) is an anatomical variant characterised by abnormalities in the position, rotation, and vascular supply of the kidney, with functioning renal masses on both sides of the vertebral column fused together at the isthmus. Due to the altered pattern of kidney vasculature, endovascular aortic repair for aortic abdominal aneurysm (AAA) in the presence of HK requires vascular anatomy specific planning. Report: A 68 year old male, with multiple comorbidities, presented with an asymptomatic AAA and HK. The kidney vasculature was characterised by the presence of three arteries: two arising laterally at the same level and a third polar artery arising from below. The polar artery was 6 mm in diameter and larger than the other two; therefore, in order to preserve this artery, a custom-made device with a single side branch was implanted below the main renal arteries. A balloon expandable covered stent was used to complete the side branch into the polar renal artery. The follow-up computed tomography angiography revealed a successful outcome, with total aneurysm exclusion, branched graft patency, no endoleak, and unchanged renal function. Discussion: This case report shows a possible surgical solution for a case of HK with AAA and the importance of accurate endovascular planning. Large polar arteries, if present, need to be preserved, and custom-made devices in the modern endovascular era permit that. This approach could represent the best option for complicated patients.

Clinical outcomes and complications of eyelid versus eyebrow approaches to supraorbital craniotomy: systematic review and indirect meta-analysis.

OBJECTIVE: Eyebrow supraorbital craniotomy is a versatile keyhole technique for treating intracranial pathologies. The eyelid supraorbital approach, an alternative approach to an eyebrow supraorbital craniotomy, has not been widely adopted among most neurosurgeons. The purpose of this systematic review and meta-analysis was to perform a pooled analysis of the complications of eyebrow or eyelid approaches for the treatment of aneurysms, meningiomas, and orbital tumors. METHODS: A systematic review of the literature in the PubMed, Embase, and Cochrane Review databases was conducted for identifying relevant literature using keywords such as "supraorbital," "eyelid," "eyebrow," "tumor," and "aneurysm." Eyebrow supraorbital craniotomies with or without orbitotomies and eyelid supraorbital craniotomies with orbitotomies for the treatment of orbital tumors, intracranial meningiomas, and aneurysms were selected. The primary outcomes were overall complications, cosmetic complications, and residual aneurysms and tumors. Secondary outcomes included five complication domains: orbital, wound-related, scalp or facial, neurological, and other complications. RESULTS: One hundred three articles were included in the synthesis. The pooled numbers of patients in the eyebrow and eyelid groups were 4689 and 358, respectively. No differences were found in overall complications or cosmetic complications between the eyebrow and eyelid groups. The proportion of residuals in the eyelid group (11.21%, effect size [ES] 0.26, 95% CI 0.12-0.41) was significantly higher (p < 0.05) than that in the eyebrow group (6.17%, ES 0.10, 95% CI 0.08-0.13). A subgroup analysis demonstrated significantly higher incidences of orbital, wound-related, and scalp or facial complications in the eyelid group (p < 0.05), but higher other complications in the eyebrow group. Performing an orbitotomy substantially increased the complication risk. CONCLUSIONS: This is the first meta-analysis that quantitatively compared complications of eyebrow versus eyelid approaches to supraorbital craniotomy. This study found similar overall complication rates but higher rates of selected complication domains in the eyelid group. The literature is limited by a high degree of variability in the reported outcomes.

Saccular abdominal aortic aneurysm in adolescence with tuberous sclerosis.

Key clinical message: Abdominal aortic aneurysm complicated by tuberous sclerosis is rare, particularly in patients over the age of 10. It is important to screen for abdominal aortic aneurysm in adolescents diagnosed with tuberous sclerosis regularly. Abstract: A 15-year-old girl who was diagnosed with tuberculous sclerosis complicated with a saccular aortic abdominal aneurysm (AAA), measuring 19 × 18 mm in diameter. The patient underwent open repair of AAA using a 11 mm straight prosthetic graft. It is important to screen for AAA in adolescents diagnosed with tuberous sclerosis regularly.

Effect of Statin Treatment in Patients with Aneurysmal Subarachnoid Hemorrhage: A Network Meta-Analysis.

BACKGROUND: There are knowledge gaps regarding the relative efficacy of statins for aneurysmal subarachnoid hemorrhage (aSAH). This study aims to examine the comparative effectiveness and determine the ranking of different statins with network meta­analysis in patients with aSAH. METHODS: MEDLINE, Embase, Pubmed, and Cochrane Central Register of Controlled Trials were searched from database inception until December 15, 2022. Outcomes included delayed cerebral ischemia (DCI), functional recovery, and mortality. Relative risk (RRs) ratios and associated 95% confidence intervals (CIs) were estimated. The values derived from surface under the cumulative ranking curve were obtained to rank the treatment hierarchy in the analysis. RESULTS: We identified 13 trials involving 1,885 patients. Atorvastatin 20 mg (RR 0.68, 95% CI 0.53-0.86), pravastatin 40 mg (RR 0.51, 95% CI 0.31-0.77), and simvastatin 80 mg (RR 0.54, 95% CI 0.40-0.70) were superior to the placebo in preventing DCI. Additionally, simvastatin 80 mg (RR 0.60, 95% CI 0.42-0.84) and pravastatin 40 mg (RR 0.56, 95% CI 0.32-0.93) were associated with a decreased risk of DCI than simvastatin 40 mg. Comparisons across treatment durations suggested that short-term (RR 0.62, 95% CI 0.50-0.76) statin therapy reduced risk of DCI. CONCLUSIONS: Simvastatin 80 mg might be the most effective intervention in reducing DCI. Additionally, short-term therapy might provide more benefits. Further research with longer follow-up is warranted to validate the current findings in patients with aSAH who are at high risk of DCI.

LXRα Promotes Abdominal Aortic Aneurysm Formation Through UHRF1 Epigenetic Modification of miR-26b-3p.

BACKGROUND: Abdominal aortic aneurysm (AAA) is a severe aortic disease without effective pharmacological approaches. The nuclear hormone receptor LXRα (liver X receptor α), encoded by the NR1H3 gene, serves as a critical transcriptional mediator linked to several vascular pathologies, but its role in AAA remains elusive. METHODS: Through integrated analyses of human and murine AAA gene expression microarray data sets, we identified NR1H3 as a candidate gene regulating AAA formation. To investigate the role of LXRα in AAA formation, we used global Nr1h3-knockout and vascular smooth muscle cell-specific Nr1h3-knockout mice in 2 AAA mouse models induced with angiotensin II (1000 ng·kg·min; 28 days) or calcium chloride (CaCl2; 0.5 mol/L; 42 days). RESULTS: Upregulated LXRα was observed in the aortas of patients with AAA and in angiotensin II- or CaCl2-treated mice. Global or vascular smooth muscle cell-specific Nr1h3 knockout inhibited AAA formation in 2 mouse models. Loss of LXRα function prevented extracellular matrix degeneration, inflammation, and vascular smooth muscle cell phenotypic switching. Uhrf1, an epigenetic master regulator, was identified as a direct target gene of LXRα by integrated analysis of transcriptome sequencing and chromatin immunoprecipitation sequencing. Susceptibility to AAA development was consistently enhanced by UHRF1 (ubiquitin-like containing PHD and RING finger domains 1) in both angiotensin II- and CaCl2-induced mouse models. We then determined the CpG methylation status and promoter accessibility of UHRF1-mediated genes using CUT&Tag (cleavage under targets and tagmentation), RRBS (reduced representation bisulfite sequencing), and ATAC-seq (assay for transposase-accessible chromatin with sequencing) in vascular smooth muscle cells, which revealed that the recruitment of UHRF1 to the promoter of miR-26b led to DNA hypermethylation accompanied by relatively closed chromatin states, and caused downregulation of miR-26b expression in AAA. Regarding clinical significance, we found that underexpression of miR-26b-3p correlated with high risk in patients with AAA. Maintaining miR-26b-3p expression prevented AAA progression and alleviated the overall pathological process. CONCLUSIONS: Our study reveals a pivotal role of the LXRα/UHRF1/miR-26b-3p axis in AAA and provides potential biomarkers and therapeutic targets for AAA.

Endovascular reconstruction of the aortic arch with new-generation dual-branch and triple-branch endograft: design, applicability, indications, outcomes, and future directions.

INTRODUCTION: Repair of aortic arch pathologies such as degenerative aneurysms and dissections still represents a significant challenge for vascular and cardiac surgeons. We focused on the design, evolution, implementation, and prospects of endovascular dual branch technology for treatment of pathologies in the aortic arch. AREAS COVERED: The literature search was conducted in a non-systematic fashion through the PubMed and Scopus databases; articles deemed relevant to the scope of the work and fully published in English language from January 12,000 until December 2023 were considered for inclusion. Two companies so far have developed and launched in market a dedicated arch dual branch platform: Terumo Aortic and Cook Medical. EXPERT OPINION: A common limitation to the available technology is represented by the fact that urgent cases (i.e. large or rapidly growing aneurysms, symptomatic patients) cannot be treated because the customization process generally takes between 6 and 8 weeks, and there is an inherent risk for aorta-related adverse events to happen during the waiting time. While the future holds much promise, several technical hurdles still exist and further developments (in device design, operators' skills, patients' selection, and outcomes assessment) are needed to establish the safety and effectiveness of treatment particularly over the long-run.

Current research status and future of endovascular treatment for basilar artery aneurysms.

Aneurysms occurring along the basilar artery (BA) account for <1% of all intracranial aneurysms. Endovascular treatment (EVT) in particular is recommended for large unruptured BA aneurysms and ruptured BA aneurysms. Given that EVT techniques vary, a detailed review of EVT for BA aneurysms is necessary. In this review, the following issues were discussed: the anatomy and anomalies of the BA, the classification of BA aneurysms, the natural history of BA aneurysms, the status of open surgery, the use of EVT for various types of BA aneurysms and the deployment of new devices. According to the findings of this review and based on our experience in treating BA aneurysms, traditional coiling EVT is still the optimal therapy for most BA aneurysms. However, in some BA aneurysms, flow diverter (FD) deployment can be used. In addition, there are also some new devices, such as intrasaccular flow disruptors and stent-like devices that can be used to treat BA aneurysms. In general, EVT can yield good clinical and angiographic outcomes for patients with BA aneurysms. In addition, recent new devices and techniques, such as new-generation FDs generated via surface modification and virtual reality simulation techniques, show promise for EVT for BA aneurysms. These devices and techniques may further improve EVT outcomes for BA aneurysms.

Midterm follow-up after embolization of intracranial aneurysms proximal to the circle of Willis with the Silk Vista flow diverter: the I-MAMA registry.

PURPOSE: The aim of this registry was to assess technical success, procedural safety and mid- to long-term follow-up results of the Silk Vista "Mama" (SVM) flow diverter (BALT, Montmorency, France) for the treatment of proximal intracranial aneurysms. METHODS: Between August 2020 and March 2022, data from nine Italian neurovascular centres were collected. Data included patients' clinical presentation, aneurysms' size, location and status, technical details, overall complications and mid- to long-term angiographic follow-up. RESULTS: Forty-eight aneurysms in 48 patients were treated using the SVM. Most aneurysms were small (≤ 10 mm: no. 29, 60%) and unruptured (no. 31, 65%); 13 aneurysms were recurrent after coiling or clipping. 37/48 aneurysms involved the internal carotid artery (77%). Optimal opening and complete wall apposition of the device were achieved in 46 out of 48 cases (96%). Four intra- or periprocedural complications occurred (two thrombotic complications successfully resolved, one cerebellar ischemia, one perirenal hematoma), without new neurological deficit. No significant intra-stent stenosis or stent displacement was observed during follow-up. No FD-related morbidity nor mortality was reported. At midterm (6-12 months) to long-term (> 12 months) follow-up, complete aneurysm occlusion (OKM D) was achieved in 76% of cases. Eighty-eight percent of patients had complete aneurysm occlusion or entry remnant (OKM D + C). CONCLUSIONS: Our experience suggests that the new generation of low-profile SVM flow diverter for the treatment of proximal intracranial aneurysms is safe and effective, with low rates of intraprocedural complications and acceptable mid- to long-term occlusion rate.

Risk factors of 30-day and long-term mortality and outcomes in open repair of thoracoabdominal aortic aneurysm.

BACKGROUND: Open repair of thoracoabdominal aortic aneurysm (TAAA) was characterized by significant risk of postoperative mortality and morbidity. The aim of this study was to determine the perioperative predictors of early and long-term mortality in patients undergoing open repair of TAAA. Besides, the postoperative outcomes in patients with open repair of TAAA were described. METHODS: This is a single-center retrospective study, and 146 patients with open repair of TAAA from January 4, 2011, to November 22, 2018 was involved. Categorical variables were analyzed by the Chi-square test or Fisher's exact test, and continuous variables were analyzed by the independent sample t-test and the WilCoxon rank-sum test. Multivariate Logistic regression and Cox regression were applied to identify the predictors of 30-day and long-term mortality, respectively. The Kaplan Meier curves were used to illustrate survival with the Log-rank test. RESULTS: The 30-day mortality was 9.59% (n = 14). Older than 50 years, the intraoperative volume of red blood cell (RBC) and epinephrine use were independently associated with postoperative 30-day mortality in open repair of TAAA. Long-term mortality was 17.12% (n = 25) (median of 3.5 years (IQR = 2-5 years) of follow-up). Prior open thoracoabdominal aortic aneurysm (TAAA) repair, aortic cross-clamping (ACC) time, intraoperative volume of RBC and use of epinephrine were independently correlated with long-term mortality. CONCLUSIONS: Identifying perioperative risk factors of early and long-term mortaliy is crucial for surgeons. Intraoperative volume of RBC and use of epinephrine were predictors of both early and long-term mortality. In addition, patients of advanced age, prior open repair of TAAA and prolonged ACC time should be paid more attention.

A case report of a giant coronary artery aneurysm masquerading as a ventricular mass.

Background: A coronary artery aneurysm is a dilation exceeding 1.5 times the diameter of the patient's largest coronary vessel. They are rare, varying in prevalence between 1.4 and 4.9%. Additionally, they carry a high risk of potential complications, including thrombosis and myocardial infarction, with a risk of rupture. We present an interesting case of a patient with initial imaging suggesting a mass in the right ventricle. Case summary: This patient initially presented with acute hypoxic respiratory failure related to pulmonary oedema. His course was complicated by symptomatic ventricular tachycardia and an inferoposterior myocardial infarction. Further investigation revealed a left anterior descending artery and circumflex artery thrombosed aneurysm projecting into the right ventricle. Multimodal imaging was used to arrive at his diagnosis. He continues to do well on medical therapy for coronary artery disease and heart failure. Discussion: Clinicians should be vigilant for this rare pathology, which may be easily missed yet poses a high mortality risk. Our case demonstrates the benefit of multimodal imaging, as this patient's aneurysm was initially mistaken for a ventricular mass.