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.

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.

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.

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.

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.

Superior vena cava obstruction and cardiovascular implantable electronic devices-a new era of leadless devices.

Cardiovascular implantable electronic devices (CIEDs) such as pacemakers and implantable cardioverter defibrillators require the placement of a transvenous lead through the superior vena cava (SVC), which can be difficult if there is stenosis or obstruction of the SVC. Moreover, SVC syndrome may occur after the lead is inserted even if the SVC was intact before the implantation. Therefore, there is need of an appropriate strategy for handling stenosis or obstruction of SVC during lead placement. In addition, advances are being made in CIEDs that do not require transvenous leads, and thus CIEDs without a transvenous lead should be considered depending on the indications and urgency of the particular case. This manuscript is divided into (I) device therapy for patients with SVC obstruction and (II) therapeutic strategy for SVC obstruction after lead implantation. In patients with SVC syndrome, treatment of the SVC occlusion should be based on the individual pathophysiology, and depending on the indications and urgency of the case, treatment with CIEDs that do not require transvenous leads should be considered. Further data must be accumulated to clarify the long-term prognosis of device implantation after treatment of SVC occlusion. In addition, transvenous lead extraction is now widely used for device-related SVC obstruction, and this procedure also merits further accumulation of data.

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.

End-stage renal disease in a critical patient with superior vena cava syndrome with central vein catheters inserted via the superficial femoral vein: A case study.

Superior vena cava syndrome (SVCS) is caused by obstruction to the blood flow through this vein. Indwelling central venous devices, such as cardiac pacemakers and haemodialysis catheters have emerged as the most common benign aetiology of SVCS. SVCS is particularly severe in patients with end-stage renal disease who require continuous renal replacement therapy plus infusion therapy. The presence of SVCS results in a reduction of available venous access for affected patients. Therefore, venous access plays a crucial role in the management of these patients. The importance of dealing with vascular access (VA) in critical patients with these conditions cannot be overstated. This case describes an 81-year-old man with respiratory failure who had end-stage renal disease complicated with SVCS. Using ultrasound-guided puncture, we inserted a peripherally inserted central catheter (PICC) into the superficial femoral vein to meet his infusion requirements in intensive care. After successful placement, the catheter tip position was adjusted using imaging to position the tip relative to the haemodialysis catheter. Whenever patients with severe renal dysfunction are treated, central veins should be preserved. Safe PICC access is possible via the superficial femoral vein to protect the last central VA for rational use. This meets urgent needs for infusion and deserves promotion.

Emergency radiation therapy in modern-day practice: Now or never, or never again ?

Radiation therapy plays a fundamental role in oncological emergencies such as superior vena cava syndrome (SVCS) and metastatic epidural spinal cord compression (MESCC). These are two examples of critical complications of metastatic cancer in terms of pain and functional impact (respiratory and/or neurological). The aim of this review is to explore the current indications, treatment options and outcomes for emergency radiotherapy regarding to these complications.Regarding SVCS, studies are mostly retrospective and unanimously demonstrated a beneficial effect of radiotherapy on symptom relief. Spinal cord compression remains an indication for urgent radiotherapy, and should be combined with surgery when possible. The innovative stereotactic body radiotherapy (SBRT) showed promising results, however this technique requires small volumes and more time preparation and therefore is often unsuitable for SVCS and MESCC emergencies.This review concluded that radiotherapy has a central role to play within a multimodal approach for SVCS and MESCC treatment. Further prospective studies are needed to confirm the effectiveness of radiation and establish the criteria for selecting patients to benefit from this treatment option.

Superior vena cava syndrome in chronic intestinal failure patients: When the going gets tough.

BACKGROUND & AIMS: Catheter-related venous thrombosis is a severe complication of home parenteral nutrition (HPN) with potentially devastating consequences such as superior vena cava syndrome (SVCS). Early recognition and awareness of factors leading to its development are of paramount importance. However, studies are lacking in HPN patients focusing on this topic. In this study, we aimed to determine the incidence of SVCS in HPN patients and describe SVCS-related outcomes. METHODS: This retrospective cohort study comprised all adult HPN patients who developed SVCS between 2000 and 2022 at our national HPN referral center. Primary outcome was the incidence of SVCS. Secondary outcomes include SVCS-related symptoms, tip location of central venous access device (CVAD) post-insertion and at time of SVCS, diagnostics and treatment. RESULTS: SVCS was diagnosed in 38 of 616 patients (6%), with an annual cumulative incidence rate ranging between 0 and 4.2%. Most common presenting symptoms were facial edema (82%) and arm edema (50%). Post-insertion, 17% (6/36) of patients had a correct position of the CVAD tip and 11% (4/36) during SVCS diagnosis. Computed tomography was the most used diagnostic imaging technique (66%). Sixty-three percent of patients started, 11% switched, and 21% continued anticoagulant treatment. CONCLUSIONS: The incidence of SVCS is relatively high in our vulnerable HPN population. It is key to recognize whenever such patients present with vascular obstruction-related symptoms and treat them in an early stage by a multidisciplinary team.

Risk factors for hypotension in patients with hemodialysis-associated superior vena cava syndrome.

OBJECTIVE: We analyzed the risk factors for hypotension in patients with hemodialysis-associated superior vena cava syndrome (SVCS) and effectiveness of endovascular intervention in hypotension related to SVCS. METHODS: This was a retrospective cohort study. A total of 194 maintenance hemodialysis patients diagnosed with SVCS who were admitted to the Department of Nephrology, West China Hospital of Sichuan University from January 2019 to December 2021 were selected and divided into a hypotension group and a nonhypotension group. Demographic and clinical data were compared. Hypotension simply refers to blood pressure levels of <90/60 mm Hg on a nondialysis day. All patients received endovascular intervention. RESULTS: Hypotension was found in 85 of the 194 patients. The following factors were significantly different between the hypotension and nonhypotension groups: body mass index, history of hypertension, tunneled-cuffed catheter as the means of dialysis access, azygos ectasis, SVC stenosis of >70% or occlusion, occlusion at the cavitary junction, serum calcium, diastolic left ventricular (LV) posterior wall thickness, LV end-diastolic volume, stroke output, and LV ejection fraction. Multivariate logistic regression analysis showed that hypertension history (OR, 0.314; P = .027), tunneled-cuffed catheter as vascular access (OR, 3.997; P < .001), SVC stenosis of >70% or occlusion (OR, 5.243; P < .001), LV posterior wall thickness (OR, 0.772; P = .044), and serum calcium (OR, 0.146; P = .005) were independent risk factors for hypotension. The mean values of systolic and diastolic blood pressure after intravascular treatment were significantly elevated from those before intervention (P < .001). The primary patency rates of SVC were 66.8%, 58.7%, and 50.0% at 3, 6, and 12 months after the procedure. CONCLUSIONS: The incidence of hypotension in patients with hemodialysis-associated SVCS is high. The identification of risk factors of hemodialysis-related hypotension provides insight into potential treatment strategies. Endovascular treatment is expected to improve hypotension related to SVCS in hemodialysis patients.

Case review and imaging: Treatment of central venous catheter associated fibrin sheath and chronic thrombus with the ClotTriever system.

Fibrin sheath formation is a leading culprit of central venous catheter malfunction. The complete removal of fibrin sheaths is an essential component of maintaining catheter patency, preventing future restenosis, and decreasing the risk of bloodborne infections. Treatment of fibrin sheaths includes pharmacologic therapy, balloon angioplasty, catheter exchange, and mechanical stripping. In this article 3 cases are reviewed, 2 patients had long-term hemodialysis catheter malfunction and 1 had complications related to a chest port. On imaging, superior vena cava stenosis, occlusion, and/or filling defect were identified for all patients, as well as findings suggesting the presence of fibrin sheath. Description of these cases detail a new technique for fibrin sheath removal utilizing the ClotTriever System (Inari Medical, Irvine, CA), which is a mechanical thrombectomy device used for the treatment of deep vein thrombosis. This technique allowed for complete removal of the fibrin sheath via a minimally invasive interventional procedure which did not require access through the central venous catheter lumen.

Superior vena cava syndrome induced by lung hyperinflation in chronic obstructive pulmonary disease: a case report.

BACKGROUND: Superior vena cava syndrome is rarely attributed to chronic obstructive pulmonary disease. CASE PRESENTATION: We present the case of an 82-year-old Japanese man who experienced gradually progressive dyspnea on exertion. His physical examination revealed small vascular dilatations on his chest and upper abdominal skin characterized by blood flow from head to leg, indicating superior vena cava syndrome. Radiographic findings included lung hyperinflation with a drop-like heart on chest X-ray, and emphysematous changes on computed tomography. The superior vena cava appeared extremely narrow and slit-like, with no adjacent mass or giant bulla. Pulmonary function testing indicated a forced expiratory volume in 1 second of 0.82L (44.4% of predicted value) and a forced expiratory volume in 1 second/forced vital capacity of 31.29%. A diagnosis of chronic obstructive pulmonary disease was made. We discuss how longitudinal forces can narrow the superior vena cava, particularly when it protrudes toward the lung field due to its anatomical location in the upper mediastinum. The absence of mediastinal adipose tissue may render the superior vena cava susceptible to compression, resulting in a loss of its typical columnar structure. The protrusion of the superior vena cava toward the lung field may be a contributing factor to superior vena cava narrowing in chronic obstructive pulmonary disease. CONCLUSION: This case represents the first reported instance of superior vena cava syndrome associated with chronic obstructive pulmonary disease, characterized by lung hyperinflation, in the absence of a giant bulla.

Dexmedetomidine Used to Maintain Spontaneous Ventilation in a Patient With Anterior Mediastinal Mass and Superior Vena Cava Syndrome.

Background: Anterior mediastinal masses (AMMs), which can be benign or malignant, are a common cause of superior vena cava (SVC) syndrome. Because of their location, AMMs can cause significant airway compromise during the perioperative period, so anesthetic management of a patient with SVC syndrome can present significant challenges. Case Report: A patient presented with SVC syndrome secondary to a large AMM. After careful consideration and discussion with the patient about the risks and benefits of various approaches, the decision was made to provide sedation using dexmedetomidine as the sole agent during image-guided biopsy. Conclusion: Patients who present with AMMs require careful anesthetic planning. Dexmedetomidine can be effective in achieving the primary objective of maintaining spontaneous respiration.