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Key Clinical Message This case image describes the complex proposed etiologies of a case of superior vena cava syndrome. Hence, different diagnostic and therapeutic modalities are needed in a multidisciplinary team approach.

Key Clinical Message: This case image describes the complex proposed etiologies of a case of superior vena cava syndrome. Hence, different diagnostic and therapeutic modalities are needed in a multidisciplinary team approach. [ABSTRACT FROM AUTHOR]

Introduction: Behçet’s disease (BD) is a rare inflammatory autoimmune disorder characterized by recurrent oral and genital ulcers, uveitis, and other systemic manifestations. Reported neurological manifestations of BD include meningoencephalitis, cerebral venous thrombosis, intracranial hypertension (ICH), and cranial nerve palsies. Involvement of the superior vena cava (SVC) is rare but should be considered in patients with BD with suspected ICH. Case Report: A 32-year-old man presented with ICH as the initial manifestation of BD. He presented with a one-week history of facial and neck edema, headache, and blurry vision, followed by fever, sore throat, and oral blisters a few days later. Lumbar puncture (LP) was performed, and opening pressure was found to be elevated at >50 cmH2O and closing pressure of 36 cmH2O. The work-up included a chest CT, which identified thrombosis of the SVC extending into the brachiocephalic veins, and blood work which revealed elevated inflammatory markers. Further probing revealed a history of genital ulcers and a family history of a cousin with BD. The patient underwent mechanical thrombectomy and was treated with oral prednisone with symptom resolution. Conclusion: BD has a wide spectrum of symptomology and may present without common manifestations, making it challenging to diagnose. The aim of our report was to emphasize the importance exploring the rarer vascular, neurological, and cardiac symptoms of BD in order to avoid potentially dangerous sequala. A system approach may be necessary to diagnose and optimally treat these patients.

Objective: In the present report, we have described the technical and clinical outcomes of percutaneous thrombectomy in the deep veins of the upper extremity and thorax using the ClotTriever system (Inari Medical, Irvine, CA). Methods: Fourteen patients with symptomatic deep venous occlusive disease in the upper extremity deep veins and thoracic central veins who had undergone thrombectomy using the ClotTriever system between October 2020 and January 2022 were reviewed. The technical results, adverse events, imaging follow-up data, and clinical outcomes were recorded. Results: Fourteen patients (seven men and seven women; mean age, 53.6 ± 13.3 years) constituted the study cohort. Of the 14 patients, 9 (64.3%) had had DVT due to intravascular invasion or external compression from known malignancy, 2 (14.3%) had had infected thrombi and/or vegetation due to Staphylococcus aureus refractory to intravenous antibiotic therapy, and 3 (21.4%) had had a benign etiology for thrombus formation. The presenting symptoms included upper extremity and/or facial swelling (n = 14), upper extremity pain (n = 6), fever (n = 2), and dyspnea (n = 1). Thrombectomy with the ClotTriever system was successfully completed in all 14 patients. Seven patients (50.0%) had required additional venous stent reconstruction after thrombectomy to address the underlying stenosis. No major adverse events were noted. All the patients had experienced resolution of the presenting symptoms. Conclusions: For the management of symptomatic deep venous occlusive disease of the upper extremity deep veins and thoracic central veins, thrombectomy using the ClotTriever system was feasible with excellent technical and clinical success.

This case report describes a 56-year-old female who presented to the emergency department with diffuse facial and bilateral upper extremity swelling. The patient has a past medical history of Superior vena cava (SVC) syndrome secondary to a clot around her port-a-cath, adenocarcinoma of the lungs status post chemotherapy and radiation, hyperlipidemia, rheumatoid arthritis, diverticulitis status post colon resection, and hypothyroidism. Imaging confirmed the presence of a thrombus obstructing the SVC, likely due to her hypercoagulable state. This case report details the successful removal of a thrombus using the FlowTriever device by INARI in a patient with SVC syndrome. Although indicated for treatment of PE, FlowTriever has shown success in other conditions and nearly eliminates the risk of bleeding without the need for administering thrombolytics, as explained below in the setting of SVC syndrome.

Superior vena cava (SVC) obstruction, whether from benign or malignant causes, results in a variety of symptoms. It is a potential medical emergency when cerebral or laryngeal edema occurs. Endovascular therapy is the treatment of choice for patients in need of emergent relief of symptoms. This article will provide a review of SVC syndrome with a focus on endovascular treatment techniques. [ABSTRACT FROM AUTHOR]

Background: The mass immunization campaign against Coronavirus disease 2019 (COVID-19) has resulted in more patients presenting to the emergency department (ED) with concern for a vaccine reaction.Case Report: A 68-year-old man presented to the ED reporting an allergic reaction to the COVID-19 vaccine. He initially noted swelling of his face, neck, and right arm after receiving the first dose of the vaccine. After his second dose of the vaccine, the swelling became more pronounced and prompted him to seek care. On examination, he had fullness of the neck and engorgement of the left external jugular vein, which were exacerbated when the patient raised his arms above his head, consistent with Pemberton's sign. Apart from the swelling of the head and neck, there were no other findings consistent with an allergic reaction. The presence of Pemberton's sign prompted a computed tomography scan of the chest with contrast, which revealed a paratracheal mass measuring 4.5 × 2.0 cm with marked narrowing of the superior vena cava (SVC). The patient was admitted to the hospital for SVC syndrome, and further workup revealed a non-small cell lung cancer. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Patients may misattribute their symptoms to a COVID vaccine reaction when they are, in fact, experiencing a more serious underlying disease. This case highlights the importance of a thorough physical examination and maintaining a broad differential diagnosis. In this case, the presence of Pemberton's sign raised suspicion for SVC syndrome, and prompted further workup. [ABSTRACT FROM AUTHOR]

Abstract Background Superior vena cava syndrome is a relatively rare presentation in which diminished venous return to the heart produces congestion of the neck, face and upper extremities. Typically, a mediastinal mass produces external compression on the superior vena cava and reduces venous return. However, superior vena cava syndrome can present acutely in the setting of vena cava thrombosis. Multiple scoring systems are available to assist clinicians with appropriate timing of interventions for SVC syndrome. When specific criteria are met, endovascular intervention can be beneficial to patients to prevent rapid deterioration. Case presentation A 75-year-old female with no significant past medical history presented to the emergency department with increased facial swelling, nausea and vomiting which began the night prior to presentation. The patient underwent a CT chest which revealed a 3.2 × 3.0 × 3.8 cm spiculated mass compressing the right main bronchus and right pulmonary artery. The patient was intubated and interventional radiology was consulted. The patient underwent venography which showed extensive thrombosis of the innominate veins. Rheolytic thrombectomy with AngioJet™ was performed to alleviate clot burden and minimize risk of secondary pulmonary embolism. Kissing stents were placed in the bilateral innominate veins to maintain patency after thrombectomy. After the procedure, the patient was successfully extubated and had near complete resolution of facial swelling approximately 12 h post procedure. A follow up venogram performed on post procedure day 4 showed patent bilateral subclavian, innominate, and internal jugular veins as well as a patent superior vena cava. Conclusions Acute occlusion of superior vena cava can present with life threatening symptoms such as loss of airway. AngioJet™ thrombectomy is another tool available to interventional radiologists when a patient’s clinical condition necessitates treatment.

Superior vena cava (SVC) syndrome is caused by either extrinsic compression of SVC or intrinsic lesions within SVC leading to obstruction of SVC and consequently swelling of the face, neck, and upper extremeities. Iatrogenic incidence is on the rise due to the use of long-term indwelling catheters. SVC syndrome in extracorporeal membrane oxygenation (ECMO) has been defined in neonatal and paediatric ECMO cases. Only one case of SVC syndrome in adult has been defined while using a double-lumen ECMO cannula. Our case describes a case of SVC syndrome in a case on veno-venous ECMO (VV-ECMO) with an internal jugular vein (IJV) return cannula which is unheard of. A high index of suspicion is required to diagnose SVC syndrome. [ABSTRACT FROM AUTHOR]

Key message We herein report a case of lung cancer with recurrent superior vena cava (SVC) syndrome, which was treated with additional stent placement. Our report suggests the possibility that additional SVC stent placement is an option for treatment of tumour ingrowth, even in patients with poor performance status.

Abstract Background The incidence of superior vena cava syndrome within the United States is roughly 15,000 cases per year. Superior vena cava syndrome is a potentially life-threatening medical condition; however, superior vena cava syndrome is not fatal in the majority of cases. Superior vena cava syndrome encompasses a collection of signs and symptoms resulting from obstruction of the superior vena cava, including swelling of the upper body of the head, neck, arms, and/or breast. It is also associated with cyanosis, plethora, and distended subcutaneous vessels. Lung cancer, including both non-small cell lung cancer and small cell lung cancer, is the most common extrinsic cause of superior vena cava syndrome. Intrinsic disruption of superior vena cava flow can also precipitate superior vena cava syndrome. This case report describes an unusual presentation and potential etiology of superior vena cava syndrome. Case presentation Our patient was a 51-year-old black woman with locally advanced, stage IIIB non-small cell lung cancer who had no clinical symptoms of superior vena cava syndrome at the time of diagnosis. However, she did have radiographic evidence of superior vena cava stenosis caused by extrinsic compression from her large right hilar primary tumor. She was treated with definitive chemoradiation, receiving 60 Gy of external beam radiation therapy given concurrently with chemotherapy. Three months after completion of radiotherapy, she developed signs of superior vena cava syndrome, including breast and supraclavicular swelling. She had a chest computed tomography scan showing over 50% reduction in the size of a right hilar mass; however, she had continued radiographic stenosis of the superior vena cava. The distribution of stenosis appeared to be inferior to the caudal extent of pretreatment tumor volume. She had no other radiographic indications for superior vena cava syndrome. Conclusions Generally, superior vena cava syndrome is the result of extrinsic compression of the superior vena cava by tumor. Our patient’s case represents the development of superior vena cava syndrome after an excellent response of tumor with near-complete tumor response. We suspect chemoradiation therapy as a potential etiology for the precipitation of the superior vena cava syndrome, which is currently not well reported in the medical literature.

Purpose: To identify whether long-term symptom relief and stent patency vary with the use of covered versus uncovered stents for the treatment of benign SVC obstruction.Methods and Materials: We retrospectively identified all patients with benign SVC syndrome treated to stent placement between January 2003 and December 2015 (n = 59). Only cases with both clinical and imaging follow-up were included (n = 47). In 33 (70%) of the patients, the obstruction was due to a central line or pacemaker wires, and in 14 (30%), the cause was fibrosing mediastinitis. Covered stents were placed in 17 (36%) of the patients, and 30 (64%) patients had an uncovered stent. Clinical and treatment outcomes, complications, and the percent stenosis of each stent were evaluated.Results: Technical success was achieved in all cases at first attempt. Average clinical and imaging follow-up in years was 2.7 (range 0.1-11.1) (covered) and 1.7 (range 0.2-10.5) (uncovered), respectively. There was a significant difference (p = 0.044) in the number of patients who reported a return of symptoms between the covered (5/17 or 29.4%) and uncovered (18/30 or 60%) groups. There was also a significant difference (p = < 0.001) in the mean percent stenosis after stent placement between the covered [17.9% (range 0-100) ± 26.2] and uncovered [48.3% (range 6.8-100) ± 33.5] groups. No significant difference (p = 0.227) was found in the time (days) between the date of the procedure and the date of clinical follow-up where a return of symptoms was reported [covered: 426.6 (range 28-1554) ± 633.9 and uncovered 778.1 (range 23-3851) ± 1066.8]. One patient in the uncovered group had non-endovascular surgical intervention (innominate to right atrial bypass), while none in the covered group required surgical intervention. One major complication (SIR grade C) occurred that consisted of a pericardial hemorrhagic effusion after angioplasty that required covered stent placement. There were no procedure-related deaths.Conclusion: Both covered and uncovered stents can be used for treating benign SVC syndrome. Covered stents, however, may be a more effective option at providing symptom relief and maintaining stent patency if validated by further studies. [ABSTRACT FROM AUTHOR]

Purpose: To assess the outcomes of endovascular management for superior vena cava (SVC) syndrome secondary to fibrosing mediastinitis (FM). Methods: Between January 2004 and December 2016, 10 consecutive patients with endovascularly managed SVC syndrome secondary to FM were identified in an institutional database. Venograms were performed to assess the severity and location of the lesion and allow measurement for stent selection. Standard stenting and angioplasty techniques were utilized to establish luminal patency. The safety, feasibility, clinical success, and the primary and secondary patency were evaluated. Kaplan-Meier survival analysis was used to determine median duration of stent patency. A log-rank test was used to test differences in prior stent use. Results: Our cohort was predominantly female (7/10) with an average age of 42.2 years. Of the 10 patients, 3 had undergone endovascular stenting at an outside institution prior to referral to our institution due to new, recurrent, or worsening symptoms. All patients underwent venography demonstrating stenosis (8/10) or occlusion (2/10) at initial presentation. Stenting or angioplasty was technically successful in 9 (90.0%) patients. Eight of 10 patients had primary stenting, while 1 achieved vascular patency and symptom resolution with angioplasty alone. Median duration of primary patency was 31.3 months (95% confidence interval: 5.9-103). Six (54.5%) patients required secondary revision procedures. Median duration of secondary patency was 6.1 months, with 25% of revisions occurring by 4 months and 75% occurring by 20.9 months. All treated patients (9/9) reported symptomatic relief at 1-month follow-up, establishing a clinical success rate of 100%. There were no 30-day adverse effects related to the procedure. Conclusion: This study demonstrates that endovascular therapy is a safe and feasible approach for managing FM-related SVC syndrome. [ABSTRACT FROM AUTHOR]

Chronic upper extremity (UE) occlusive disease and superior vena cava syndrome (SVCS) can be debilitating to the patient. Chronic UE venous occlusion is more often because of a benign lesion, whereas SVCS is more likely the result of malignant obstruction. Endovascular treatment with percutaneously placed intravascular stents is increasingly being used to provide relief of symptoms. [ABSTRACT FROM AUTHOR]

Abstract: Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35 year old male patient who presented with acute symptoms of dyspnoea, facial puffiness, engorged neck veins and hoarseness of voice. With the clinical picture consistent with the superior vena caval (SVC) syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S‐100. Cytogenetic analysis by fluorescent in‐situ hybridization (FISH) demonstrated the t(X: 18) translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three‐cycles of neo‐adjuvant (ifosfamide 2400mg/m2 on days 1‐5 and doxorubicin 37.5 mg/m2 on days 1 & 2) chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further post‐operative treatment with adjuvant chemo‐radiotherapy was provided. Patient was under regular surveillance at our clinic and remains free of symptoms one‐year after treatment completion. But after 14 months of treatment completion patient again had symptoms of progressive dyspnea, hoareness of voice and mild facial puffiness over a period of 2 months. On further investigating he was found to have right‐sided centrally located mass with cystic and necrotic changes with extension and compression of trachea, SVC, right upper lobe bronchus and its branches. Histopathological examination of the biopsy from the lesion revealed adenoid cystic carcinoma of the lung. Rest of the metastatic work up was within normal. Immunohistochemistry of the specimen revealed c‐Kit positivity. In view of the morbid second surgery he was put on Imatinib 400mg once a day and celecoxib 200mg twice a day. After 4 months patient had partial response and presently continuing with the same regimen. Extensive literature search didn't reveal much information on combined primary mediastinal sarcoma and adenoid cystic carcinoma of lung. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate success and safety of needle (sharp) recanalization as a method to re-establish access in patients with chronic central venous occlusions.Materials and Methods: Thirty-nine consecutive patients who underwent this procedure were retrospectively reviewed to establish success rate and associated complications. In all cases, a 21- or 22-gauge needle was used to restore connection between two chronically occluded segments after conventional wire and catheter techniques had failed. The needle was guided toward a target placed through a separate access by fluoroscopic guidance. When successful, the procedure was completed by placing a catheter, ballooning the segment, and/or stenting.Results: The procedure was successful in 37 of the 39 patients (95%). The vast majority of the treated lesions were in the SVC and/or right innominate vein. Occlusions ranged in length between 10 and 110 mm, and the average length of occluded venous segment was 40 mm in the treated group. There were four minor (SIR classification B) complications involving pain management after the procedure. There were two major (SIR classification D) complications both of which involved hemorrhage into the pericardium treated with covered stents (5.1%).Conclusions: Sharp recanalization is a viable procedure for patients who have exhausted standard wire and catheter techniques. The operator performing this procedure should be familiar with potential complications so that they can be addressed urgently if needed. [ABSTRACT FROM AUTHOR]

The flushing phenomenon may represent a physiologic or a pathologic reaction. Although flushing is usually benign, it is prudent that the physician remains aware of potentially life-threatening conditions associated with cutaneous flushing. A thorough investigation should be performed if the flushing is atypical or not clearly associated with a benign underlying process. The diagnosis often relies on a pertinent history, review of systems, physical examination, and various laboratory and imaging modalities, all of which are discussed in the 2 articles in this continuing medical education series. This article reviews flushing associated with fever, hyperthermia, emotions, menopause, medications, alcohol, food, hypersensitivity reactions, rosacea, hyperthyroidism, dumping syndrome, superior vena cava syndrome, and neurologic etiologies. [ABSTRACT FROM AUTHOR]

NUT carcinoma is an aggressive malignancy defined genetically by a balanced translocation of the NUT gene on chromosome 15q14, most commonly associated with the bromodomain-containing protein 4 (BRD4) gene on 19p13.1 but less frequently with variant genes, including BRD3 and NSD-3. We present a case report of a metastatic pulmonary NUT carcinoma found to have a BRD3-NUT fusion and to have only focal pan-cytokeratin staining. Biopsy of the pulmonary mass revealed dyscohesive cells with enlarged nuclei, prominent nucleoli and high nuclear to cytoplasmic ratio without areas of squamous differentiation. Initial immunohistochemical stains were positive for NUT, p63 and retained SMARCA4, while negative for Lu-5 (pan-cytokeratin), TTF-1, p40, S100 protein, OCT-4, HMB-45, SMA, and PAX-8. Tempus ×T assay revealed a BRD3-NUTM1 fusion gene. Post-mortem analysis revealed an ill-defined mass abutting the trachea and superior vena cava, as well as a perirenal mass., (© 2023 Wiley Periodicals LLC.)

Objective: In the present report, we have described the technical and clinical outcomes of percutaneous thrombectomy in the deep veins of the upper extremity and thorax using the ClotTriever system (Inari Medical, Irvine, CA)., Methods: Fourteen patients with symptomatic deep venous occlusive disease in the upper extremity deep veins and thoracic central veins who had undergone thrombectomy using the ClotTriever system between October 2020 and January 2022 were reviewed. The technical results, adverse events, imaging follow-up data, and clinical outcomes were recorded., Results: Fourteen patients (seven men and seven women; mean age, 53.6 ± 13.3 years) constituted the study cohort. Of the 14 patients, 9 (64.3%) had had DVT due to intravascular invasion or external compression from known malignancy, 2 (14.3%) had had infected thrombi and/or vegetation due to Staphylococcus aureus refractory to intravenous antibiotic therapy, and 3 (21.4%) had had a benign etiology for thrombus formation. The presenting symptoms included upper extremity and/or facial swelling (n = 14), upper extremity pain (n = 6), fever (n = 2), and dyspnea (n = 1). Thrombectomy with the ClotTriever system was successfully completed in all 14 patients. Seven patients (50.0%) had required additional venous stent reconstruction after thrombectomy to address the underlying stenosis. No major adverse events were noted. All the patients had experienced resolution of the presenting symptoms., Conclusions: For the management of symptomatic deep venous occlusive disease of the upper extremity deep veins and thoracic central veins, thrombectomy using the ClotTriever system was feasible with excellent technical and clinical success., (© 2023 The Author(s).)

We report a case of a 34 −year −old lady who presented with anemia and bad obstetric history. She was diagnosed to have Systemic Lupus Erythematosus (SLE) with Antiphospholipid Antibodies (APLA) Syndrome. She also had dilated and tortuous veins over the chest and upper abdomen. CT Venography was done, and she was detected to have complete thrombotic occlusion of superior vena cava, right brachiocephalic and right internal jugular veins, with multiple systemic venous collaterals. Patient was started on steroids and anticoagulation therapy which resulted in improvement of her general condition. This case is noteworthy as the patient was asymptomatic for extensive major vessel thrombosis and SVC syndrome. [ABSTRACT FROM AUTHOR]

An elderly man presented with clinical features of superior vena cava (SVC) syndrome restricted to the right side. Detailed evaluation suggested the diagnosis of unilateral SVC syndrome due to adenocarcinoma (presumably) from lung. With this case, we discuss the clinical presentation and causes of this rare clinical entity. Also, unilateral signs of SVC syndrome warrant a thorough clinical evaluation as it can be a presenting feature of a spectrum of diseases ranging from benign to malignant etiologies [ABSTRACT FROM AUTHOR]

Surgicel, an absorbable haemostat, is widely used in cardiovascular surgery. An 81-year-old woman, who was diagnosed with ischaemic mitral regurgitation, underwent mitral valve plasty and coronary artery bypass grafting. On postoperative day two, her superior vena cava (SVC) pressure gradually rose to 38 mmHg and she developed low output syndrome. Emergent surgery revealed that the cause of SVC syndrome was external compression from a haematoma at the posterior surface of the SVC, which formed around the Surgicel. [ABSTRACT FROM AUTHOR]

Objective: In the present study, we evaluated the technical and clinical outcomes of thoracic central vein reconstruction for superior vena cava (SVC) syndrome using kissing Viabahn VBX stent grafts (W.L. Gore & Associates, Flagstaff, AZ)., Methods: All adult patients with SVC syndrome who had undergone attempted bilateral brachiocephalic vein-to-SVC reconstruction using kissing VBX stent grafts at an academic hospital between August 2019 and February 2021 were reviewed. The technical results, adverse events, imaging follow-up findings, and clinical outcomes were recorded. Patency over time was assessed using Kaplan-Meier analysis., Results: A total of 28 patients (16 women and 12 men; mean age, 52.0 years) constituted the study cohort. Of the 28 patients, 17 (60.7%) had had benign and 11 (39.3%) malignant etiologies. The presenting symptoms included neck swelling (n = 17; 60.7%), bilateral upper extremity swelling (n = 15; 53.6%), dyspnea (n = 7; 25%), unilateral upper extremity swelling (n = 4; 14.3%), and dysphagia (n = 1; 3.6%). SVC reconstruction with VBX stent grafts in a kissing configuration was successfully completed in 27 of the 28 patients (96.4%). Four major adverse events were noted in the benign etiology subgroup (23.5%), including intraprocedural hemopericardium (n = 3) and delayed pneumothorax (n = 1). Of the 28 patients, 27 (96.4%) had experienced resolution of their presenting symptoms. The mean clinical follow-up for the living patients was 358.8 ± 77.2 days (range, 78-645 days). The mean imaging follow-up for the living patients was 272.6 ± 91 days (range, 26-594 days). The primary, primary-assisted, and secondary patency rates at 12 months were 71.8%, 88.8%, and 100%, respectively., Conclusions: For the management of SVC syndrome, thoracic central vein reconstruction with kissing VBX stent grafts was feasible with a high rate of symptom resolution and acceptable patency. However, this technique should not be recommended for those with benign SVC syndrome owing to the high risk of cardiac tamponade., (Copyright © 2022 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)