A Systematic Review and Meta-analysis of 24 Month Patency After Endovenous Stenting of Superior Vena Cava, Subclavian, and Brachiocephalic Vein Stenosis.

OBJECTIVE: This systematic review and meta-analysis aimed to appraise recent evidence assessing patency outcomes at various time points in patients with superior vena cava, subclavian, and brachiocephalic vein stenosis who had undergone stenting. DATA SOURCES: PubMed, Scopus, and Cochrane Library databases were searched for studies up to December 2022. REVIEW METHODS: Measured outcomes included technical success rate, primary, primary assisted, and secondary patency at various time points. A subgroup analysis was also conducted to compare malignant and benign obstruction. GRADE was used to assess the certainty of evidence. RESULTS: Thirty nine studies reporting outcomes in 1539 patients were included in the meta-analysis. Primary patency up to 1 year after the procedure was 81.5% (95% CI 74.5 - 86.9%). Primary patency declined after 1 year to 63.2% (95% CI 51.9 - 73.1%) at 12 - 24 months. Primary assisted patency and secondary patency at ≥ 24 months were 72.7% (95% CI 49.1 - 88.0%) and 76.6% (95% CI 51.1 - 91.1%). In the subgroup analysis, primary patency was significantly higher in patients with a malignant stenosis compared with a benign stenosis at 1 - 3 and 12 - 24 months. No significant difference was seen for pooled secondary patency rates when comparing the malignant and benign subgroups. GRADE analysis determined the certainty of evidence for all outcomes to be very low. CONCLUSION: Stenting is an effective intervention for benign and malignant stenosis of the superior vena cava, subclavian, and brachiocephalic veins. Primary patency rates were good up to 1 year after the procedure, with 81.5% of stents retaining patency at 6 - 12 months. Patency rates declined after 1 year, to 63.2% primary and 89.3% secondary patency at 12 - 24 months, showing improved outcomes following re-intervention. High quality evidence is lacking. More research is needed to investigate patency outcomes and the need for surveillance or re-intervention programme.

Fatal Hemoptysis With Pleural Effusions Secondary to Superior Vena Cava Obstruction as a Complication of Central Venous Catheterization.

This report outlines a rare case of superior vena cava (SVC) syndrome presenting with hemoptysis in a 33-year-old female Jehovah's Witness patient with a complex medical history, including systemic lupus erythematosus (SLE) and chronic hemodialysis dependency due to end-stage renal disease and a failed renal transplant. The SVC syndrome was attributed to occlusion from a right subclavian dialysis catheter. The management of this case was particularly challenging due to the patient's severe anemia and the development of a tension hemothorax following thoracentesis, compounded by her refusal of blood transfusions in adherence to her religious beliefs. A multidisciplinary approach, incorporating bloodless medical techniques such as erythropoietin and iron infusions alongside surgical interventions without blood transfusion, was successfully employed. This case sheds light on the evolving etiology of SVC syndrome and highlights the uncommon but potentially fatal occurrence of hemoptysis as a complication. It also emphasizes the importance of respecting patient values in complex medical decisions.

Post-tuberculosis fibrosing mediastinitis: A report of 3 cases.

Fibrosing mediastinitis is a rare entity generally caused by granulomatous disease. Most cases develop as a complication of histoplasmosis. Association with tuberculosis has been rarely documented. We report the case of 3 young patients who presented with superior vena cava syndrome several years after treated tuberculosis. Biological, radiological, and histological investigations led to the diagnosis of fibrosing mediastinitis.

First presentation of Graves' thyroid storm complicated by catastrophic antiphospholipid antibody syndrome: A case report.

Catastrophic antiphospholipid antibody syndrome is a rare and severe subtype of antiphospholipid syndrome with multisystemic organ failure due to thromboembolic events, resulting in high mortality rates. The association between catastrophic antiphospholipid antibody syndrome and autoimmune thyroid diseases is rarely reported in the literature. We report a case of a 35-year-old previously healthy female with Graves' thyroid storm, positive lupus antibodies, and probable catastrophic antiphospholipid antibody syndrome. Her hospital course was complicated by extensive venous thromboembolism, superior vena cava syndrome, thromboembolic strokes, and Takotsubo cardiomyopathy. Eventually, this led to an unfortunate death secondary to profound shock after 8 days despite emergent treatment. Our case report discusses the link between autoimmune thyroid disorders and catastrophic antiphospholipid antibody syndrome. We emphasize the difficulty in diagnosing catastrophic antiphospholipid antibody syndrome in extremely ill patients and stress the significance of considering it as a possible cause in thyrotoxicosis patients with multiple organ failure and hypercoagulability. Early recognition and prompt management are crucial in improving outcomes in these patients.

Two cases of vertebral perfusion disturbances in computer tomography imitating metastatic lesions in the course of superior vena cava thrombosis.

Skeletal metastases are frequently observed in various malignancies. In some cases, they are asymptomatic and can be found incidentally in various imaging methods in patients without known malignant tumors. In this case study 2 cases of vertebral perfusion disturbances are presented that imitate vertebral metastatic lesions in computer tomography in the course of superior vena cava thrombosis. The first patient was referred to our clinic for chest and abdominal computer tomography (CT) for staging due to a known tumor in the anterior mediastinum. The second patient was referred for chest CT due to swelling in the upper extremity and neck, with the suspected diagnosis of a tumor or pulmonary embolism. In both cases, CT scans showed metastases suspected lesions in the upper thoracic vertebral bodies. In both cases, additionally, the thrombosis of superior vena cava (SVC) and vena brachiocephalica was confirmed (in the first case due to tumor compression in the upper mediastinum, in the second case due to the presence of pacemaker leads). In control CT scans after anticoagulation treatment, there were no suspected lesions in the vertebral bodies, which confirmed the diagnosis of vertebral perfusion disturbances in the course of SVC thrombosis in both patients. In conclusion, in rare cases of metastases suspected lesions of thoracic vertebral bodies in contrast-enhanced computer tomography among patients with a diagnosis of superior vena cava thrombosis vertebral perfusion disturbances should be included in differential diagnosis protocol.

Cavoatrial junction stenting in vascular hemodialysis catheter malfunction.

In patients undergoing hemodialytic treatment via intravascular catheters, stenosis or occlusion of central veins is common. Despite an extensive characterization of Superior Vena Cava Syndrome (SVCS) no data is available about CavoAtrial Junction (CAJ) stenosis. We report the case of two patients with a story of multiple catheter failures due to thrombosis or infection. Computed tomography (CT) showed radiological signs of CAJ stenosis confirmed at the following venography. In absence of other feasible options to place a vascular access, the two underwent stenting with Gore Viabahn VBX balloon expandable endoprosthesis (W.L. Gore & Associates, Flagstaff, AZ, USA) of the CAJ stenosis. Completion venography showed complete resolution of the stenosis in both patients. No complications occurred during the procedures. At a mean follow-up of 878 ± 559 days no signs of in-stent restenosis or recoil were found. The present cases emphasize the feasibility and safety of CAJ stenting, underlining the importance of preserving CAJ and upper veins patency in hemodialysis access.

Femoral to abdomen tunneling at the bedside for medium/long term venous access.

BACKGROUND: Femoral to abdomen tunneling of small-bore central venous catheters is a bedside technique for patients with contraindications to a thoracic approach, or as an alternative to a lower extremity catheter exit site. METHOD: A femoral to abdomen tunneling technique was implemented for patients receiving medium and long-term intravenous treatments with contraindications to the thoracic venous approach or as an alternative to a lower extremity catheter exit site. All venous access devices were inserted with ultrasound guidance under local anesthesia, and catheter tip placement assessed by post procedural radiography. RESULTS: In this case series, from January 2020 to January 2023, a total of eight FTA-tunneled venous access devices were inserted. There were seven ambulatory patients and one bedbound patient. The median length of the subcutaneous tunnel was 20 cm, ranging from 15 to 27 cm. The median length of the intravenous catheter to the terminal tip was 31 cm, ranging from 23 to 40 cm. Tip location was confirmed by post-procedural abdominal radiograph. The catheter tip locations were interpreted to be at the level of T8-T9 (2), T12 (1), L4 (2), L2 (2), L1(1).No insertion or post insertion related complication was reported. Six patients completed the scheduled intravenous treatment. One patient was unable to be tracked due to transfer to an outside facility. One catheter initially demonstrated to be coiled over the left common iliac vessel was repositioned using a high flow flush technique. There was one reported catheter dislodgment by the nurse providing care and maintenance. The overall implant days were 961, with a median dwell time of 125 days ranging from 20 to 399 days. CONCLUSION: Femoral to abdomen tunneling provides an alternative exit site useful in select patients with complex intravenous access. The data of this small retrospective review suggests this a safe and minimally invasive bedside procedure.

Safety and efficacy of large-bore uncovered stents for treating malignant superior vena cava syndrome.

PURPOSE: To investigate the safety and efficacy of large-bore uncovered stents for treating malignant superior vena cava syndrome. METHODS: This retrospective study included 115 patients (89 men, 26 women; mean age 63.2 years; range 21-83 years) who underwent endovascular large-bore (≥18 mm in diameter) uncovered stent placement between August 2015 and July 2022. One patient was lost to follow-up. Therefore, 114 patients were available for follow-up. RESULTS: Stent placement was technically successful in all 115 patients. Minor procedure-related complications occurred in nine (7.8 %) patients. One hundred eight (93.9 %) patients experienced complete or marked symptomatic relief (Kishi score ≤ 2) at a mean of 3 days after procedure. The cumulative stent patency rates were 98.2 %, 95 %, 93.7 %, 91.5 %, 83.5 %, and 83.5 % at 1, 3, 6, 12, 18, and 24 months, respectively. Stent occlusion occurred in ten (8.8 %) of 114 patients at a mean of 215 days (range 1-732 days) due to thrombosis (n = 7) and tumor ingrowth (n = 3). Stent occlusion did not occur in 21 patients who underwent subsequent central venous catheter insertion. The median patient survival time was 159 days (95 % confidence interval 102-216 days). Univariate and multivariate Cox regression analysis revealed adjuvant anticancer treatment (p = 0.001) and tumor response (p < 0.001) as independent predictors of patient survival. CONCLUSIONS: Endovascular placement of large-bore uncovered stents was a safe and effective treatment for malignant superior vena cava syndrome. Large-bore stent placement can effectively prevent stent occlusion by tumor ingrowth in most cases, and it can provide a sufficient diameter for subsequent insertion of central venous catheters.

Lower extremity peripherally inserted central catheter placement ectopic to the ascending lumbar vein: A case report.

BACKGROUND: Peripherally inserted central catheters (PICCs) are an essential infusion route for oncology patients receiving intravenous treatments, but lower extremity venipuncture is the preferred technique for patients with superior vena cava syndrome (SVCS). We report the case of a patient with a lower extremity PICC ectopic to the ascending lumbar vein, to indicate and verify PICC catheterisation in the lower extremity is safe and feasible. And hope to provide different perspectives for clinical PICC venipuncture to get the attention of peers. CASE SUMMARY: On 24 August 2022, a 58-year-old male was admitted to our department due to an intermittent cough persisting for over a month, which worsened 10 d prior. Imaging and laboratory investigations suggested the patient with pulmonary malignancy and SVCS. Chemotherapy was not an absolute contraindication in this patient. Lower extremity venipuncture is the preferred technique because administering upper extremity venous transfusion to patients with SVCS can exacerbate oedema in the head, neck, and upper extremities. The patient and his family were informed about the procedure, and informed consent was obtained. After successful puncture and prompt treatment, the patient was discharged, experiencing some relief from symptoms. CONCLUSION: Inferior vena cava catheterisation is rare and important for cancer patients with SVCS, particularly in complex situations involving ectopic placement.

Management of malignant superior vena cava syndrome.

Superior vena cava (SVC) syndrome occurs due to obstructed blood flow through the SVC. It can present clinically on a spectrum, between asymptomatic and life-threatening emergency. Patients commonly report a feeling of fullness in the head, facial, neck and upper extremity edema, and dyspnea. On imaging, patients commonly have superior mediastinal widening and pleural effusion. The majority of cases are due to malignant causes, with non-small cell lung cancer, small cell lung cancer, and lymphoma the most commonly associated malignancies. When evaluating patients, a complete staging workup is recommended, as it will determine whether treatment should be definitive/curative or palliative in intent. If the patient requires urgent treatment of venous obstruction, such as in the cases of acute central airway obstruction, severe laryngeal edema and/or coma from cerebral edema, direct opening of the occlusion by endovascular stenting and angioplasty with thrombolysis should be considered. Such an approach can provide immediate relief of symptoms before cancer-specific therapies are initiated. The intent of treatment is to manage the underlying disease while palliating symptoms. Treatment approaches most commonly employ chemotherapy and/or radiation therapy depending on the primary histology. Mildly hypofractionated radiation regimens are most commonly employed and achieve high rates of symptomatic responses generally within 2 weeks of initiating therapy.

Safety and Effectiveness of Abre Self-Expanding Venous Stent for Treatment of Superior Vena Cava Syndrome.

PURPOSE: Superior vena cava (SVC) syndrome is a constellation of symptoms that results from partial or complete SVC obstruction. Endovascular SVC stenting is an effective treatment for SVC syndrome with rapid clinical efficacy and low risk of complications. In this study, we assess the technical and clinical outcomes of a cohort of patients with SVC syndrome treated with the AbreTM self-expanding venous stent (Medtronic, Inc, Minneapolis, MN, USA). METHODS: An institutional database was used to retrospectively identify patients with SVC syndrome treated with AbreTM venous self-expanding stent placement between 2021-2023. Patient demographic data, technical outcomes, treatment effectiveness, and adverse events were obtained from the electronic medical record. Nineteen patients (mean age 58.6) were included in the study. Thirteen interventions were performed for malignant compression of the SVC, 5 for central venous catheter-related SVC stenosis, and 1 for HD fistula-related SVC stenosis refractory to angioplasty. RESULTS: Primary patency was achieved in 93% of patients (17/19). Two patients (7%) required re-intervention with thrombolysis and angioplasty within 30 days post-stenting. Mean duration of clinical and imaging follow-up were 228.7 ± 52.7 and 258.7 ± 62.1 days, respectively. All patients with clinical follow-up experienced significant improvement in clinical symptoms post-intervention. No stent related complications were identified post-intervention. CONCLUSIONS: Treatment of SVC syndrome with the AbreTM self-expanding venous stent has high rates of technical and clinical success. No complications related to stent placement were identified in this study.

If not angioedema, what is it? Diagnostic approach to facial edema.

Facial edema is a relatively frequent clinical presentation encountered in patients seen in allergology and dermatology clinics. The differential diagnosis is broad, and sometimes the definitive diagnosis can be a challenge for the clinician. Facial angioedema itself encompasses different etiopathologies (histaminergic, bradykinergic, etc.) that must be distinguished from other causes of facial edema, such as allergic contact dermatitis, granulomatous conditions, inflammatory causes, infections, neoplasms or paraneoplastic syndromes, autoimmune diseases, among other entities hereby referred as miscellanea. A proper diagnostic approach is essential to order the appropriate tests, as well as to prescribe a targeted treatment. This review focuses on entities that present with facial edema and summarize their characteristic clinical features.

Hemorrhagic pericardial tamponade in a hemodialysis patient with catheter-related superior vena cava syndrome: a case report.

BACKGROUND: Iatrogenic complications of endovascular treatment for central venous stenosis have not yet been reported. Here we present a case of a patient on maintenance hemodialysis who developed catheter-related superior vena cava syndrome and subsequently suffered from hemorrhagic pericardial tamponade after undergoing percutaneous transluminal angioplasty and stenting. CASE PRESENTATION: A 72-year-old male patient presented with uremia, and had been receiving maintenance hemodialysis for the past five years. The patient initially presented with dysfunction of the dialysis catheter (a cuffed tunneled double-lumen catheter in the right internal jugular vein). Imaging examination revealed a segmental occlusion of the superior vena cava stretching from the distal end of the dialysis catheter up to right atrium entrance, apparent compensatory dilatation of the azygos vein, and abundant subcutaneous collaterals. The patient underwent percutaneous transluminal balloon dilatation and stenting (covered stent) of the superior vena cava in the Cath Lab. During the procedure, with forceful advancement of the guidewire, it was observed to progress for a distance before a "smoke" appeared, and an outward spillage of contrast agent was visible, which suggested a possible vessel puncture leading into the mediastinum. Unfortunately, postoperative hemorrhagic pericardial tamponade occurred and the patient developed cardiogenic shock. He experienced symptoms included chest tightness and breath shortness with a recorded blood pressure of 84/60mmHg. After draining 600 ml of bloody fluid through pericardiocentesis, the patient's symptoms alleviated and his condition improved. CONCLUSIONS: The case emphasizes the need for increased attention to iatrogenic endovascular injuries during catheter placement and endovascular treatment, such as causing pericardial hemorrhage leading to cardiac tamponade.

From compression to diagnosis: identification of superior vena cava syndrome using point-of-care ultrasound in the emergency department.

BACKGROUND: Superior vena cava (SVC) syndrome is an urgent condition arising from restricted blood flow through the SVC, often linked to factors like malignancy, thrombosis, or infections. Typically, confirmation of the diagnosis involves computed tomography. However, many patients experience respiratory distress and cannot lie supine. Given the increasing integration of point-of-care ultrasound in emergency medicine, it is important to be familiar with findings that are suggestive of this important condition. CASE REPORT: In this case report, we highlight a young patient presenting to the emergency department with superior vena cava syndrome symptoms, successfully diagnosed using point-of-care ultrasound. CONCLUSION: This case highlights the utility of point-of-care ultrasound based diagnosis of SVC syndrome and upper arm deep venous thrombosis in a patient with underlying malignancy which ultimately led to early involvement of relevant speciality for initiation of treatment.

T-cell Lymphoblastic Lymphoma Unveiling As Superior Vena Cava Syndrome in a 19-Year-Old Male.

Superior vena cava syndrome (SVCS) is a collection of signs and symptoms resulting from superior vena cava obstruction which is either partial or complete. SVCS is a rare clinical entity, often associated with various malignancies. T-cell lymphoblastic lymphoma (T-LBL) primarily of the mediastinum (thymus) is a rare and aggressive non-Hodgkin lymphoma that can lead to SVCS. We discuss the case of a 19-year-old male who arrived at our emergency department with symptoms of cough, breathlessness, and facial puffiness along with swelling in the right anterior mediastinum for two weeks suggestive of acute SVCS. An anterior mediastinal mass was confirmed on a chest X-ray and computed tomography. A biopsy of the mass revealed primary mediastinal (thymic) T-LBL. This case report focuses on the unique presentation of a T-LBL as SVCS in a 19-year-old male. Moreover, it highlights the need for vigilance among healthcare providers in recognizing this atypical complication and underscores the critical importance of early diagnosis and timely intervention.

Vascular access challenges in hemodialysis patients with superior vena cava syndrome.

BACKGROUND: Superior vena cava syndrome in hemodialysis patients resulting from previous or current use of a tunneled central vein catheter is a rare but potentially severe condition. Two aspects have to be addressed during management and treatment: the restoration of central venous flow and the creation of an alternative vascular access to guarantee hemodialysis. RESEARCH DESIGN: Conforming to the current guidelines and literature, we present a stepwise approach and discuss therapeutic options. The removal of the tunneled central vein catheter should be attempted and a native vascular access created whenever feasible. RESULTS: First, an upper extremity AVF should be preserved or, as in our case, made functional. Endovascular treatment of CVSO should primarily consist of balloon dilatation. Placement of a stent or stent graft should be considered as a secondary option. HeRO graft placement may be considered in recurrent CVSO and recanalization with a Surfacer. LL-AVF or AVG need to be discussed and may be an alternative for certain HD patients when the risk of lower limb ischemia and infection is considered. CONCLUSION: Several therapeutic options are available and the basic principles are well established in the literature, although the level of evidence is not high. Therefore, we propose a stepwise and interdisciplinary approach to guide the challenging decision-making process in SVC.

Surgical resection of an intraluminal tumor in the azygos vein with an unknown primary site causing superior vena cava syndrome.

Intraluminal tumor in the azygos vein is a rare disease that can cause superior vena cava (SVC) syndrome. Radiotherapy and endovascular stenting with or without chemotherapy are reported to have a high clinical success rate for the management of SVC syndrome with malignancy, but a poor survival rate. Here, we report a 69-year-old man who presented with swelling of the face and upper extremities, who was diagnosed with SVC syndrome caused by an intraluminal tumor in the azygos vein. Enhanced chest computed tomography revealed an intraluminal mass with a filling defect from the azygos vein to the SVC, with no extravascular extension or dissemination of the primary tumor. Surgical resection of the mass en bloc with the azygos vein and SVC reconstruction was performed. A poorly differentiated carcinoma was diagnosed on postoperative pathological evaluation. Twelve months after resection, the patient was well with no signs of recurrent disease. This case highlights that surgical resection should be considered as a treatment of choice for the management of SVC syndrome caused by an intraluminal malignancy in the azygos vein.