Surgical Intervention for Giant Pulmonary Artery Aneurysm in Behcet Disease: A Case Report.

BACKGROUND Pulmonary artery aneurysm (PAA), defined as a pathologic dilatation of the PA greater than 1.5-fold the normal diameter, is a rare complication of Behçet disease. It is due to a weakening of the vessel wall for a great vessels' vasculitis, often asymptomatic and incidentally diagnosed on imaging studies. However, if ignored, it can lead to life-threatening complications such as rupture and massive hemoptysis. We report the case of a giant fast-growing PAA in a young patient with a history of Behçet disease in which an inadequate follow-up and poor patient information could had led to life-threatening complications. CASE REPORT A 37-year-old man with a history of Behçet disease presented to our Emergency Department with hemoptysis due to a right inferior lobar artery aneurysm measuring 52×33 mm. The aneurysm was detected years before, measuring 18 mm, but the patient and physicians missed the subsequent follow-up. After several attempts at embolization, the multidisciplinary board suggested to proceed with surgical intervention. Surgery was performed with an extracorporeal circulation system kept on stand-by due to the high hemorrhagic risk. By opening the fissure, the dilatation of the inferior lobar artery was clearly identified up to the origin of the middle lobar branch. Thus, a lower-middle bilobectomy was performed after the introduction of a suction cannula in the aneurysm, which facilitated its emptying and the subsequent maneuvers. CONCLUSIONS PAA is a rare disease, generally treated with medical therapy or interventional procedures. However, giant and fast-growing aneurysms are more likely to entail complications and often required immediate treatment. In this case, primary surgical intervention with a pulmonary bilobectomy appeared mandatory to avoid life-threatening events.

[Neurovascular pathology in a patient with neurofibromatosis type 1. Case report]. / Patología neurovascular en el paciente con neurofibromatosis de tipo 1. A propósito de un caso.

INTRODUCTION: Neurovascular involvement in patients with neurofibromatosis type 1 (NF1) presents with a wide spectrum of manifestations. Its frequency is low, albeit probably underestimated. There is currently no known specific treatment, and treatment is based on recommendations with limited evidence. This report describes a case of vascular dysplasia in a patient with NF1. CASE REPORT: A 67-year-old woman with a genetic diagnosis of NF1 and a history of multiple exeresis of neurofibromas in the left cervical region. The patient presented with a painful flare-up and swelling in the region. A cervical magnetic resonance imaging was performed, which showed signs of plexiform neurinoma growth and a lesion suggestive of aneurysm in the left cervical internal carotid artery. A subsequent computed tomographic angiography confirmed the presence of a thrombosed aneurysm with associated critical stenosis, and identified three additional aneurysms in the proximal left vertebral artery. Given the asymptomatic presentation and adequate haemodynamic compensation, the patient was prescribed a conservative treatment and clinicoradiological follow-up. CONCLUSIONS: Neurovascular alterations associated with NF1 are infrequent, and the optimal treatment for them is unknown. Studies to define its true prevalence, determine its pathophysiological substrate and estimate the risk of cerebrovascular complications more precisely are needed. This could provide more robust recommendations for the population of NF1 patients, especially in asymptomatic cases.

TITLE: Patología neurovascular en el paciente con neurofibromatosis de tipo 1. A propósito de un caso.Introducción. La afectación neurovascular en pacientes con neurofibromatosis de tipo 1 (NF1) cursa con un amplio espectro de manifestaciones y su frecuencia es baja, aunque probablemente infraestimada. En la actualidad, su tratamiento específico se desconoce y se basa en recomendaciones con bajo nivel de evidencia. Se describe un caso de displasia vascular en una paciente con NF1. Caso clínico. Mujer de 67 años con diagnóstico genético de NF1 e historia de exéresis múltiple de neurofibromas en la región cervical izquierda. La paciente presentaba un cuadro de reagudización dolorosa y tumefacción en dicha región, por lo que se le realizó una resonancia magnética cervical, que mostró signos de crecimiento de neurinomas plexiformes y una lesión sugestiva de aneurisma en la arteria carótida interna izquierda cervical. Un estudio de angiotomografía computarizada posterior confirmó la presencia de un aneurisma trombosado con estenosis crítica asociada e identificó tres aneurismas adicionales en la arteria vertebral izquierda proximal. Ante la presentación asintomática y la adecuada compensación hemodinámica, se decidió tratamiento conservador y seguimiento clinicorradiológico. Conclusiones. Las alteraciones neurovasculares asociadas a la NF1 son infrecuentes y su tratamiento óptimo se desconoce. Son necesarios estudios que definan con mayor precisión su prevalencia real, su sustrato fisiopatológico y una estimación del riesgo de complicaciones cerebrovasculares. De este modo, se podrían ofrecer recomendaciones más sólidas para la población de pacientes con NF1, especialmente en los casos asintomáticos.

Brachial plexopathy as a complication of axillary artery aneurysm in a case of Takayasu arteritis.

Takayasu arteritis is an inflammatory disease of unknown aetiology affecting large vessels. Medium vessel involvement is also well documented; however, neuropathy as a presenting manifestation is rare. In this case report, a young woman in her 20s presented with an 8-month history of intermittent claudication in the right upper limb progressing to rest pain with allodynia in C5-C8 distribution and painless right axillary mass. On examination, she had absent pulses in the right radial, brachial and subclavian artery with audible bruit in the right subclavian and abdominal aorta. CT angiogram showed features suggestive of Takayasu arteritis with a partially thrombosed aneurysm arising from the right axillary artery leading to compression of the right brachial plexus. This patient received treatment with methotrexate and oral corticosteroids. At 3 months follow-up, there was a reduction in the size of the aneurysm, resolution of compressive symptoms and normalisation of inflammatory markers.

Splenic artery aneurysm: a rare complication of autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD) is an important cause of renal dysfunction. It is the most common genetic disorder leading to end-stage kidney disease requiring dialysis. ADPKD is a multisystem disease and is linked to several extra renal abnormalities. Splenic artery aneurysms are rare in the general population. ADPKD is associated with cerebral artery aneurysms. However, splenic artery aneurysms are not a well-recognised complication of ADPKD. We report an unusual case of a splenic artery aneurysm found incidentally on abdominal CT imaging of a woman with known ADPKD.

Factors contributing to patency after aneurysmorrhaphy and outflow repair in arteriovenous fistula aneurysm treatment.

INTRODUCTION: Vascular access-related aneurysms (VARA) are a complication of arteriovenous fistulas. Repair techniques have been described in the literature with varied outcomes. MATERIALS AND METHODS: We conducted a prospective cohort study on patients who had VARA repair over 41 months. The indication for repair was an aneurysmal arteriovenous fistula (AVF) at risk of haemorrhage or difficulty in cannulation. Pseudoaneurysms, infected AVF and bleeding VARA were excluded. All patients underwent outflow stenosis treatment when present, followed by aneurysmorrhaphy. They were monitored periodically over 12 months, measuring functional primary and cumulative patency and access flow. We studied the patient demography, access flow and presence of outflow stenosis. Access flow was measured from the brachial artery (Qa) as a surrogate using ultrasonography. A Kaplan-Meier survival analysis was used to predict the primary and cumulative patency at 12 months and factors contributing to 12-month patency were analysed. RESULTS: A total of 64 patients were recruited for this study, of whom 58 completed the study. Most of the participants were male (67%) with a median age of 45 years. Forty-six patients (79.3%) had brachiocephalic fistula (BCF) aneurysms. Thirty-nine (67.2%) had preexisting outflow stenoses that required intervention. All patients underwent an aneurysmorrhaphy, of whom 12% had a cephalic arch vein transposition due to severe stenosis. Primary patency at 12 months was 86%, whereas the cumulative patency rate was 95%. Patency was significantly associated with younger age and showed a positive trend with higher preintervention Qa. Symptomatic recurrent stenosis developed in 17.2% of the cohort. CONCLUSION: Improving the patency of VARA entails the treatment of outflow stenosis and aneurysmorrhaphy. Surveillance is important to detect and treat recurrent outflow stenoses. The outcome is better among younger patients with pre-interventional access flow as measured in the brachial artery as a surrogate.

The Management of Aneurysmal Degeneration of Lower Extremity Cryopreserved Saphenous Vein Bypass Grafts.

BACKGROUND: The use of cryopreserved saphenous veins (CSVs) for the treatment of lower extremity peripheral arterial disease is an attractive option when there is no available autogenous vein. Prior studies found CSVs are at risk for aneurysmal degeneration requiring reoperation. As the management of these complications and patient outcomes is not well described, the objective of this case series is to describe the open and endovascular management of degenerative CSVs at a tertiary community center. METHODS: All CSVs implanted for lower extremity bypass at our institution between 2001 and 2021 were retrospectively reviewed. All CSVs with evidence of aneurysmal change were included in this study. CSVs with evidence of active infection were excluded. The decision to intervene was left to the discretion of the operating surgeon. Demographic data, indications for the index operation, and details about subsequent interventions for degenerative CSVs were recorded. Study end points included limb salvage and continued patency. Demographic data, indications for the index operation, and details about subsequent interventions for degenerative CSVs were recorded. RESULTS: Seventeen bypasses were identified to have aneurysmal degeneration in 13 patients in the absence of infection between 2001 and 2021. Nine of the 13 patients were male, and the average age and body mass index during the index procedure were 72 and 28, respectively. Indications for the index bypass included acute limb ischemia (9), popliteal aneurysm (2), and chronic limb threatening ischemia with Rutherford's class IV (5) and V (1). The mean time between the index procedure and first graft revision due to aneurysmal changes was 4 years. Most of the aneurysms did not occur at the site of anastomosis with 13 occurring in the body of the graft. Thirteen grafts were managed with open surgery and 3 were managed with endovascular techniques. All endovascular repairs were managed via covered stenting. Patients were followed for an average duration of 7 years from the initial bypass and 2 years from their last aneurysmal repair. Limb salvage in this cohort was 87% with 2 limbs requiring amputation, all of whom underwent open reconstruction. The mortality rate in this series was 54% and no patients died due to complications from their graft. Continued patency on Kaplan Meier survival curve analysis was 79% at 6 months, 65% at 1 year, 54% at 3 years, and 27% at 5 years. CONCLUSIONS: In our experience, aneurysmal degeneration of CSV grafts was mostly managed with standard open surgical techniques, although endovascular therapy also proved acceptable. Limb salvage rates and continued patency of repair at 1 year in this cohort were acceptable. This case series highlights the importance of diligent surveillance for patients with CSVs.

Carotid Artery Aneurysm-Induced Mediastinal Hematoma Leading to Airway Compression-A Rare Complication of Internal Jugular Vein Puncture.

Carotid artery puncture is a common complication of internal jugular vein (IJV) catheterization. However, there are few reports about an aneurysm from the carotid artery that can develop into an occult mediastinal hematoma, leading to airway compression. In this case study, we present the case of a 71-year-old male who experienced an aneurysm and delayed mediastinal hematoma, ultimately resulting in airway compression after right jugular line insertion. Our findings highlight the importance of not only addressing local hematoma formation at the puncture site promptly, but also recognizing the potential for aneurysm extension into the mediastinum and the formation of an occult hematoma, which can lead to airway compression. Additionally, we provide a summary of landmark technique precautions that can help reduce the occurrence of such severe complications.

Isolated spinal artery aneurysm: etiology, clinical characteristics, and outcomes.

OBJECTIVE: Isolated spinal aneurysms (ISAs) are rare causes of subarachnoid hemorrhage (SAH), which encompass a highly heterogeneous group of clinical entities with multifarious pathogeneses, clinical characteristics, and treatment strategies. Therefore, knowledge about the ISAs remains inadequate. In this study, the authors present a comprehensive analysis of clinical data associated with ISAs at their institutions to enhance the understanding of this disease. METHODS: Patients with ISAs confirmed by spinal angiography or surgery at the authors' institutions between 2015 and 2022 were included. Data regarding clinical presentation, lesion location, aneurysm morphology, comorbidities, treatment results, and clinical outcomes were reviewed. RESULTS: Seven patients with ISAs were included in the study. Among them, 4 patients (57.1%) experienced severe headache, and 3 patients (42.9%) reported sudden-onset back pain. Additionally, lower-extremity weakness and urinary retention were observed in 2 of these patients (28.6%). Four of the aneurysms exhibited fusiform morphology, whereas the remaining were saccular. All saccular aneurysms in this series were attributed to hemodynamic factors. Conservative treatment was administered to 3 patients, 2 of whom underwent follow-up digital subtraction angiography, which showed spontaneous occlusion of both aneurysms. Four patients ultimately underwent invasive treatments, including 2 who underwent microsurgery and 2 who received endovascular embolization. One patient died of recurrent SAH, while the remaining 6 patients had a favorable prognosis at the latest follow-up assessment. CONCLUSIONS: The morphology of aneurysms may be associated with their etiology. Saccular ISAs are usually caused by pressure due to abnormally increased blood flow, whereas fusiform lesions may be more likely to be secondary to vessel wall damage. The authors found that a saccular spinal aneurysm in young patients with a significant dilated parent artery may be a vestige of spinal cord arteriovenous shunts. ISAs can be managed by surgical, endovascular, or conservative procedures, and the clinical outcome is generally favorable. However, the heterogeneous nature of the disease necessitates personalized treatment decision-making based on specific clinical features of each patient.

Endovascular and surgical management of splenic artery aneurysms.

BACKGROUND: Although true splenic artery aneurysms (SAA) are rare, due to advancements in imaging techniques, they are seen more frequently. The aim of this study is to present our strategy of managing patients with SAA. METHODS: Retrospectively, 13 patients who were treated in a tertiary university care center between 2012 and 2020 were included. Their demographic, clinical information, and post-operative complications were analyzed. RESULTS: Seven male and six female patients were evaluated between the ages of 27 and 73. The mean age was 49.8±13.2. The diameter of the aneurysm was between 17 and 80 mm with a mean range of 31.5±16 mm. Seven patients were treated with endovascular interventions (EV). Two patients were referred to surgery with failed attempt of EV, but patients refused surgery and were followed up consequently. Patients who had larger aneurysms with an increased risk of rupture underwent aneurysmectomy and splenectomy. Conservative management was decided on two patients initially: A patient who was previously operated on for a sigmoid colon tumor, and had an aneurysm size of 15 mm and another patient with a surgical history of thoracic aortic dissection with an aneurysm size of 18 mm. One patient who underwent surgery had post-operative pancreatic fistula and was treated with percutaneous drainage. The treatment of the remaining 12 patients was completed without any further complications. CONCLUSION: Splenic artery aneurysm treatment should be individualized. Endovascular treatment can be considered for patients with stable aneurysms larger than 2 cm in the elective setting. Open surgical treatment should be considered in patients with ruptured SAA or hemodynamically unstable, complicated patients.

Use of selective visceral angiography in surgical strategy planning for celiac artery aneurysm in the celiacomesenteric trunk.

BACKGROUND: The celiacomesenteric trunk (CMT) is a common duct of the celiac artery (CA) and the superior mesenteric artery originating from the aorta, which is an uncommon anatomical variant of visceral artery circulation. Because of the variety of visceral circulation in those with CMT, the visceral circulation associated with each branch should be evaluated prior to surgical treatment of visceral artery aneurysm in the CMT. CASE PRESENTATION: A 64-year-old woman was diagnosed with a CA aneurysm in the CMT. Aneurysmectomy of the aneurysm was performed successfully. On preoperative selective visceral angiography, the CA was seen to bifurcate into the common hepatic and splenic artery. The left gastric artery was directly isolated from the aorta and perfused to the common hepatic and splenic artery through collateral circulation. These findings showed that celiac artery embolization is anatomically feasible, even in cases of celiac artery aneurysm rupture. CONCLUSIONS: Selective visceral angiography can contribute to surgical strategy planning for CA aneurysm with CMT.

Surveillance of a Transplant Kidney Harboring a Stable Renal Artery Aneurysm: A Case Report.

Renal artery aneurysms (RAAs) may occur in patients with transplanted kidneys, either through de novo development or as a preexisting feature of the donor kidney. How this vascular condition progresses in patients on immunosuppressive therapy after transplantation is poorly understood, and to our knowledge, consensus guidelines for treating transplant patients with RAA have not been developed. We present the case of a kidney allograft recipient on triple immunosuppressive therapy in whom postoperative imaging revealed a 13-mm renal artery aneurysm in the renal hilum not amenable to endovascular intervention. We review systemic influences on aneurysm formation and how matrix metalloproteinases may interact with immunosuppressive medications. Surveillance imaging over 5 years has shown a stable aneurysm, and the patient has maintained stable renal function with adequate creatinine levels and no adverse symptoms.

Outcomes of one-stage and two-stage aneurysm repair in arteriovenous fistulae.

OBJECTIVE: Maintenance of long-term arteriovenous access is important in long-term care for patients with end-stage renal disease. Arteriovenous access is associated in the long term with the development of fistula aneurysms (FAs). This study aims to evaluate the outcomes of staged FA treatment in dialysis access arteriovenous fistulae (AVF). METHODS: A retrospective review of all patients over a 12-year period with primary autogenous AVF was undertaken at a single center. Patients undergoing elective open aneurysm repair were identified and were categorized into three groups: single FA repair (single, control group) and staged and unstaged repair of two FAs (staged and unstaged). A staged repair was a procedure in which the initial intent was to treat both aneurysms in the AVF and in which the most symptomatic aneurysm was treated first. When the incision from the first surgery had healed, the second symptomatic aneurysm in the AVF was treated. An unstaged repair was a procedure in which the initial intent was to repair both symptomatic aneurysms simultaneously. All patients had a fistulogram before the FA repair. Thirty-day outcomes, cannulation failure, line placement, reintervention, and functional dialysis (continuous hemodialysis for 3 consecutive months) were examined. RESULTS: Five hundred twenty-seven patients presented with FA that met requirements for open intervention; 44% underwent single FA repair, whereas the remaining 34% and 22% underwent staged and unstaged repair of two FAs, respectively. The majority of patients were diabetic and Hispanic. Ninety-one percent of the patients required percutaneous interventions of the outflow tract (37%) and the central veins (54%). Thirty-day major adverse cardiovascular events were equivalent across all modalities. Thirty-day morbidity and early thrombosis (<18 days) were significantly higher in the unstaged group (4.3%) compared with the two other groups (1.3% and 2.1%, single and staged, respectively), which led to an increased need for a short-term tunneled catheter (8.9%) compared with the two other groups (3.4% and 4.4%, single and staged, respectively), Unstaged repair resulted in an increased incidence of secondary procedures (5.0%) compared with the two other groups (2.6% and 3.1%, single and staged, respectively). Functional dialysis at 5 years was equivalent in the single and staged groups but was significantly decreased in the unstaged group. CONCLUSIONS: Open interventions are successful therapeutic modalities for FAs, but unstaged rather than staged repair of two concurrent FAs results in a higher early thrombosis, an increased secondary intervention rate, and a need for a short-term tunneled central line. Staged and single FA repairs have equivalent results. In the setting of two symptomatic FAs, staged repair is recommended.

Acute Thrombosis of an Aneurysm of the Small Saphenous Vein: A Case Report.

BACKGROUND: A venous aneurysm (VA) is a focal dilatation of a nonvariceal vein (diameter increased by at least 1.5 times compared to the adjacent upstream or downstream venous segment), which carries a risk of venous thromboembolism (VTE) when located in the deep veins of the lower limbs but also when it affects the veins above the muscle fascia. CASE: We report the case of a 40-year-old woman who presented with a painful and disabling mass-like lesion of the upper third of the right calf. A Doppler ultrasound (DUS) examination revealed a small saphenous vein (SSV) dilated at the saphenopopliteal junction (SPJ) and thrombosed along its entire length. Anticoagulant treatment was initiated but although the thrombosis regressed on DUS, the functional impotence and the persistence of pain prompted a stripping of the SSV. Histology allowed the diagnosis of SSV's primary aneurysm. DISCUSSION: Dilated segments of the saphenous vein are not always varicose veins (VVs). True VAs can develop on veins located above the muscular fascia and, like deep VAs, may trigger VTE. Small saphenous vein aneurysms have seldom been reported, and only a few clinical observations have described the thrombosis of a SSV aneurysm. Any mass in the popliteal fossa or upper part of the calf should be examined by DUS to look for an SSV aneurysm. Excision of the saphenous vein with junctional ligation is advised if thrombosis is present at the time of diagnosis.

Angiographic findings of pulmonary arterial involvement in Behcet's Disease: Do they correlate with symptoms and acute phase response?

INTRODUCTION: Behcet's disease (BD) is a chronic inflammatory disorder with arterial vasculitis. Although, pulmonary artery aneurysm (PAA) is accepted as the prototypic arterial disorder, an increasing presence of pulmonary artery thrombosis (PAT) with or without aneurysms was also reported in recent studies. In this study, we aimed to describe computed tomography pulmonary angiography (CTPA) findings of pulmonary involvement and its correlation with symptoms and acute phase response in BD. METHOD: In this retrospective study, 153 CTPA of BD patients were assessed by two radiologists. Clinical and laboratory data were collected from the patient files. Pulmonary artery involvement (PAI) was defined as thrombus or aneurysm in CT angiography. RESULTS: Most of (85.6 %) our patients were male and median age was 33.7 ± 10 years during angiographic assessments. Sixty-two (40.5 %) angiographies presented a thrombus: 14 subsegmental, 29 segmental, 13 lobar and 6 main branches. Among these, 82.3 % (n = 51) had bilateral involvement. Isolated PAT was present in 58 (93.5 %) angiographies with only 4 (2.6 %) angiographies displaying an aneurysm together with a thrombus. Pulmonary infarction was detected in 9 angiographies. Forty-four (29.3 %) patients, almost all of them under immunosuppressive treatments for other indications, were screened for asymptomatic pulmonary involvement (without any symptoms or increased acute-phase response (APR)), and one fourth of these were diagnosed as having a segmental or subsegmental PAT. CONCLUSION: Our results show that isolated pulmonary thrombosis is the main form of PAI, and isolated pulmonary aneurysm formation is rare in our BD cases. In the presence of pulmonary symptoms with or without increased APRs, involvement of segmental or more proximal parts of pulmonary arteries is most commonly detected. We also observed that PAI may be seen in about one fourth of especially male BD patients without symptoms or increased APR. Our results suggest that BD patients with pulmonary symptoms should be screened by CTPA for PAI, however, further research is needed to clarify the role of routine CTPA screening in asymptomatic BD patients.

Midterm outcomes of arteriovenous fistula aneurysm repair with simultaneous inflow revision or outflow tract revascularization.

OBJECTIVE: The aim of this study was to investigate the 2-year outcomes of extensive revisions aimed at simultaneously addressing arteriovenous fistula (AVF) aneurysms, outflow stenosis, and/or high flow volumes. METHODS: This was a retrospective cohort study in a tertiary referral center. The study population comprised hemodialysis patients with aneurysmal AVFs requiring revision. Subjects were eligible if they were ≥18 years of age and had aneurysm repair associated with inflow revision or treatment of outflow lesions. Patients were followed-up for a minimum of 2 years, and the primary outcome measure was cumulative patency. RESULTS: Our cohort comprised 93 patients with a mean age of 56 ± 12 years. The most frequent access type was brachiocephalic fistula (61%), and the median access age at the time of revision was 66 months (interquartile range, 46-93 months). The median access flow volume was 2300 ml/min (interquartile range, 1281-2900), and 31 subjects (33%) presented aneurysms associated with both high flow and venous obstruction. Aneurysm repair consisted of aneurysmorraphy in the overwhelming majority (95%). Inflow revision was performed in a total of 49 cases (53%), and outflow tract obstruction was treated in 71 patients (72%), with 27 patients (29%) receiving a simultaneous 3-component revision. At 2 years, the primary, primary assisted, and cumulative patencies were 60% ± 1%, 91% ± 3%, and 94% ± 3%, respectively. The most frequent indication for first-time reinterventions was outflow tract stenosis (24%). Aneurysm recurrence was observed in 13 subjects (14%) during the entire follow-up and was responsible for reinterventions in seven of them (8%), with a 2-year freedom from aneurysm-related reintervention rate of 95% ± 3%. CONCLUSIONS: Extensive AVF revision with simultaneous aneurysm repair, inflow revision, and outflow tract revascularization is feasible, safe, and associated with favorable 2-year patency rates.