Diagnostic Value of Contrast-Enhanced Ultrasonography for Catheter-Related Right Brachiocephalic Vein and Superior Vena Cava Lesions in Patients Undergoing Hemodialysis-A Pilot Study.

OBJECTIVES: To evaluate the diagnostic efficacy of contrast-enhanced ultrasonography (CEUS) for detecting catheter-related right brachiocephalic vein (RBV) and superior vena cava (SVC) obstructions in patients undergoing hemodialysis (HD). METHODS: From June 1, 2021 to December 31, 2022, we enrolled 80 patients undergoing HD who had used or were using a central venous catheter as vascular access. We evaluated the diagnostic efficacy of conventional ultrasonography and CEUS for identifying RBV and SVC obstructions and compared them with that of digital subtraction angiography (DSA). In the stratified analysis, the SVC was divided into the upper and lower segments. In total, we analyzed 240 central venous segments, including the RBV. RESULTS: Among the RBV and SVC visualized by DSA, conventional ultrasonography and CEUS could visualize 67.92 and 100% of the vein segments, respectively; however, the lengths and diameters of the RBV and SVC were smaller than those recorded with DSA (P < .001). The diagnostic efficacy of CEUS for detecting catheter-related central venous obstruction was better than that of conventional ultrasonography, with a higher sensitivity (83.95 vs 41.98%), specificity (89.94 vs 53.46%), accuracy (87.92 vs 49.58%), and F1 score (82.42 vs 49.64%). CEUS showed good agreement (κ = 0.732) with DSA. In the stratified analyses, CEUS also showed higher sensitivity (83.93, 83.33, and 84.62%, respectively) and better agreement with DSA (κ = 0.635, 0.655, and 0.673, respectively) than conventional ultrasonography for detecting the RBV and the upper and lower segments of the SVC. CONCLUSIONS: CEUS had high sensitivity and specificity in diagnosing catheter-related RBV and SVC obstructions.

[Diagnostic clinical algorithm for mediastinal tumors]. / Klinicheskii algoritm diagnostikii i lecheniya opukholei sredosteniya.

OBJECTIVE: To optimize therapeutic and tactical algorithm in patients with mediastinal tumors. MATERIAL AND METHODS: The study included 300 patients with mediastinal tumors examined and treated in the Thoracic Surgery Department of the Hertzen Moscow Cancer Research Center between 2008 and 2019. Patients were stratified into 2 groups. The diagnostic group consisted of 160 patients including 80 ones after 89 ultrasound-assisted transthoracic biopsies and 80 patients after open biopsy through parasternal mediastinotomy. The treatment group consisted of 140 patients who underwent 145 resections of mediastinal tumors. We analyzed the results of diagnosis and treatment in both groups, mistakes, risk factors of non-informative biopsies and complicated course of the postoperative period. Subsequently, a clinical algorithm was developed. Its effectiveness in 50 patients was compared with 90 patients who constituted the historical control group. RESULTS: The final histological diagnosis after transthoracic ultrasound-assisted biopsy of the mediastinum was established in 71 out of 89 cases (80%). Informative value of ultrasound-assisted biopsy for thymic tumors was 100%, for metastatic mediastinal lesions - 92%. Repeated ultrasound-assisted biopsies were performed in 7 (9%) patients that made it possible to establish the final diagnosis in 5 (71%) cases. In the parasternal mediastinotomy group, the final correct histological diagnosis was made in 79 out of 80 (99%) patients. Analyzing thoracoscopic approach and traditional sternotomy or thoracotomy, we found surgery time decrease from 187 (150-215) to 140 (122-165) min (p<0.01), blood loss from 300 (200-350) to 50 (35-150) ml (p<0.01), postoperative hospital-day from 12 (9-14.5) to 7 (6-8) days (p<0.01). Mean dimension of tumor was 5 (4-6) cm in the thoracoscopy group and 7.5 (6-10) cm in the open surgery group (p<0.01). Postoperative complications occurred in 19 (13%) patients, mortality rate was 2.8%. Multivariate analysis revealed superior vena cava syndrome (OR=5.1, 95% CI=1.246-21.343; p=0.024) and invasion of the right pleural cavity (OR 4.2, 95% CI 1.12-7.69; p=0.006) as the most significant predictors of postoperative complications. The proposed algorithms made it possible to reduce the incidence of non-informative biopsy from 12% to 0% (p<0.05), misdiagnosis from 9% to 1% (p<0.05), exploratory surgeries from 23% to 0% (p<0.05). CONCLUSION: The developed algorithm is valuable to avoid the errors and improve the results of diagnosis and treatment of patients with mediastinal tumors.

Thrombolytic therapy in preterm infants: Fifteen-year experience.

OBJECTIVE: To report a single-center experience with thrombolytic therapy using recombinant tissue plasminogen activator (rt-PA) in preterm neonates with severe thrombotic events, in terms of thrombus resolution and bleeding complications. STUDY DESIGN: This retrospective study included 21 preterm neonates with severe venous thrombotic events admitted to the neonatal intensive care unit, identified in our pharmacy database from January 2001 to December 2016, and treated with rt-PA until complete or partial clot lysis, no-response or bleeding complications. Our primary outcome was thrombus resolution. RESULTS: Twenty-one preterm neonates were treated with rt-PA for an average of 2.9 cycles. Seventeen patients (80.9%) had superior vena cava thrombosis and superior vena cava syndrome. All patients had a central venous catheter, parenteral nutrition, mechanical ventilation, and sepsis. Fifteen patients (71.4%) were extremely preterm, 11 (52.4%) were extremely low birth weight, and seven (33.3%) were very low birth weight. The patency rate was 85.7%, complete lysis occurred in 11 (52.4%) patients, and partial lysis in seven (33.3%). Minor bleeding occurred in five (23.8%) patients, three patients (14.2%) had clinically relevant nonmajor bleeding events, and major bleeding occurred in six (28%) patients. CONCLUSION: In this study, the rate of thrombus resolution in preterm neonates treated with rt-PA were similar to the percentages reported in children and adolescents, with a high rate of bleeding. Therefore, rt-PA thrombolytic therapy should only be considered as a treatment option for severe life-threatening thrombosis in premature neonates for whom the benefits of the thrombolytic treatment outweigh the risks of bleeding.

Superior Vena Cava Syndrome and Hypoxic Ischemic Encephalopathy Secondary to a Massive, Right-Sided Immature Cervical Teratoma.

Cervical teratomas are a rare form of fetal teratoma that can grow to massive size. Generally, these masses can be surgically excised after birth with excellent physical and functional prognosis because the benign variants respect anatomical borders. The primary complications of these masses are associated with compromise of the trachea and esophagus: upper airway obstruction and polyhydramnios. We report the first documented occurrence of superior vena cava syndrome and hypoxic ischemic encephalopathy associated with a massive, right-sided cervical teratoma. This case highlights that when cervical teratomas are right-sided and sufficiently large, they can extend inferiorly and compromise central venous return to the heart. This unique presentation would likely have required fetal surgical excision to avoid catastrophic cerebral injury.

Superior vena cava obstruction and mediastinal mass detected by point-of-care ultrasonography.

Superior vena cava syndrome (SVCS) often relates to malignant causes such as lung tumors, metastatic cancer, or lymphomas. While the diagnosis relies nowadays on the use of contrast-enhanced thoracic computed tomography, ultrasonography may have an important value as a first-line imaging technique, particularly when used in point-of-care office-based settings. Here, we report the case of a 67-year-old male presenting with SVCS in whom ultrasound contributed to diagnosis.

Stent insertion for malignant superior vena cava syndrome: effectiveness and long-term outcome.

PURPOSE: To determine the clinical effectiveness and long-term outcome of stent insertion for malignant superior vena cava (SVC) syndrome. MATERIALS AND METHODS: From June 2010 to April 2016, 47 patients with malignant SVC syndrome were treated with stent insertion in our center. Data regarding the technical success, clinical success, and long-term outcome were collected and analyzed retrospectively. RESULTS: SVC stent insertion was successfully performed in all patients. A total of 65 stents were used. No procedure-related complication occurred in these patients. The mean SVC pressure gradient decreased from 17.8 mmHg before stent insertion to 7.6 mmHg after stent insertion (P < 0.001). Clinical success was 100%. During a mean follow-up period of 6 months (range 10 days-13 months), 25 patients underwent subsequent anti-cancer treatment. Six patients (12.8%) experienced re-obstruction of stent 1 to 189 days (median 76 days) after stent insertion. All patients died during the follow-up. The median stent patency time and survival were 339 and 167 days, respectively. The cumulative 3-, 6-, and 12-month stent patency rates were 93.4, 87.4, and 81.2%, respectively. The cumulative 3-, 6-, and 12-month survival rates were 83, 38.3, and 2.1%, respectively. The independent predictors of prolonging survival after stent insertion were lower tumor stage (P = 0.018) and subsequent anti-cancer treatment after stent insertion (P = 0.009). CONCLUSION: Stent insertion is a simple, safe, and effective method for patients with malignant SVC syndrome. Subsequent anti-cancer treatment after stent insertion may increase the survival.

Anaesthetic Management for Caesarean Delivery Patient with Obstructed Superior Vena Cava by Mediastinal Mass.

Maternal anatomical and physiological changes occur during pregnancy, particularly with cardiovascular and respiratory systems. Pregnant women with large mediastinal mass additionally have an increased risk of cardiopulmonary compromise. We report a 31-year-old patient at 30 weeks of gestation with a large anterior mediastinal tumour with superior vena cava obstruction. Her presenting symptom was progressive and severe dyspnea due to the large mediastinal mass. An ultrasound-guided biopsy was performed and the pathologic result was Hodgkin's lymphoma. Termination of pregnancy and delivery of the neonate were performed using a Caesarean delivery under spinal anesthesia in the sitting position. The perioperative anaesthetic management was successful without any serious complications, and the postoperative outcomes were very impressive.

Arrhythmogenic right ventricular cardiomyopathy in monozygotic twin sisters, and persistent left superior vena cava in one complicating implantation of ICD.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized histologically by fibro-fatty replacement of heart muscle, and clinically by ventricular arrhythmias and right ventricular dysfunction. This report presents monozygotic twins with ARVC, suggesting a genetic abnormality as the most probable cause.

Surgical treatment of an invasive thymoma extending into the superior vena cava and right atrium.

Although invasive thymoma commonly infiltrates neighbouring mediastinal structures, its extension into the superior vena cava (SVC) and consequent SVC occlusion are rare. In such cases, the urgent removal of the thymoma and radical resection of the infiltrated SVC representreasonable options, since induction therapy is time-consuming and useless for symptom resolution. A case of invasive thymoma extending into the SVC and right atrium (RA) with SVC syndrome is reported. The patient underwent a combined resection of the invasive tumor and SVC under cardiopulmonary bypass (CPB), and the SVC and bilateral brachiocephalic vein (BCV) were reconstructed with an autologous pericardial 'Y' conduit. After 40 months of follow-up, the patient showed a patent graft and no tumor recurrence.

Intracaval and intracardiac extension of invasive thymoma complicated by superior and inferior vena cava syndrome.

We present a case of an aged male with invasive thymoma that extended into the right atrium and led to superior and inferior vena cava syndrome. The patient initially presented with edema of the face and bilateral lower extremities. Echocardiography revealed a mass within the right atrium. Imaging studies demonstrated an anterior mediastinal tumor that continuously occupied the bilateral brachiocephalic veins, superior vena cava, and right atrium. Pathological diagnosis of the tumor biopsy was highly suspicious of thymoma. Due to the high risk of wide spread of the tumor, treatments including resection of the tumor were impossible. Several days later he died, and an autopsy was performed. The tumor was type B2 thymoma invading bilateral brachiocephalic veins, superior vena cava and right atrium. Multiple tumor emboli within the pulmonary arteries were identified. Direct cause of death was deemed to be tumor strangulation at the tricuspid orifice. In addition to the superior vena cava syndrome, inferior vena cava syndrome including ectasia of the intrahepatic vessels was confirmed along with pericarditis. To our knowledge, this is the first English report of an autopsy case of intracardiac thymoma extension, and a detailed literature review of similar cases is also presented.

Superior vena cava stenting in the 21st century.

Superior vena cava obstruction (SVCO) is most commonly due to malignant or thrombotic causes. The traditional treatments of surgery, radiotherapy or chemotherapy either have a high morbidity and mortality rate, or a lag period of a few weeks prior to response. Superior vena cava stenting is able to provide rapid relief of symptoms safely in the vast majority of patients. It has now become the first-line treatment for malignant SVCO, and is increasingly being used in benign SVCO. The complication and success rates compare very favourably with the other treatment modalities.

[Syndrome of vena cava superior in the injured persons with traumatic instability of a sternocostal skeleton as a consequence of polytrauma].

There were analyzed 8 clinical observations of treatment of syndrome of vena cava superior in the injured persons in traumatic instability of a sternocostal skeleton. Peculiarities of course of traumatic process, concerning the trauma severity objective prove, were established.

Investigation of the efficacy and feasibility of combined therapy of PD-L1-enhanced exogenous peripatetic adoptive natural killer (NK) cells in combination with antiangiogenic targeted therapy in the treatment of extensive-stage small cell lung cancer.

A 67-year-old male patient presented with extensive-stage small cell lung cancer with the primary lesion located in the right upper lung, accompanied by multiple metastases to the pleura and abdominal cavity with enlarged mediastinal lymph nodes. A combination therapy approach was used to target the patient's multiple systemic metastases after localized radiotherapy. The approach involved adoptive transfer of programmed death ligand 1 (PD-L1) enhanced exogenous natural killer (NK) cells, along with antiangiogenic treatment. Allogeneic cord blood NK cells were infused back into the patient over two consecutive days. On the first day, the treatment was followed by a dose of 1200 mg of atezolizumab. Subsequently, the patient received a daily dose of 10 mg of anlotinib administered orally for 14 days. This was followed by a 7-day break, and each cycle lasted 21 days. After delivering localized radiation to the primary lesion in the right lung and metastatic mediastinal lymph nodes, complete remission was achieved in the local lesion, effectively avoiding the risk of superior vena cava syndrome. Following six cycles of combined therapy, most of the metastatic lesions had disappeared, and the remaining metastatic lesions had significantly reduced in size. The recent therapeutic effect resulted in partial remission. The combination therapy of immune checkpoint inhibitor PD-L1-enhanced exogenous adoptive transfer NK cells, along with antiangiogenic targeted treatment, demonstrated a satisfactory short-term effect, with disappearance of most of the metastases and noticeable shrinkage in the remaining metastatic lesions.

Superior vena cava syndrome-induced hemoptysis.

Superior vena cava (SVC) syndrome resulting from obstruction of the blood flow to the superior vena cava is rarely reported to present with life-threatening hemoptysis. The pathogenesis and the underlying mechanism are still not well described in the literature. We report a unique case of a 27-year-old man known to have end-stage kidney disease (ESKD) on hemodialysis that presented with shortness of breath and life-threatening hemoptysis that developed during the dialysis session. Computerized tomography with contrast (CTPA) confirmed the presence of a large, calcified thrombus within the SVC along with the formation of multiple collaterals which was diagnostic for SVC syndrome. Attempts for revascularization and stenting failed, and the patient had a prolonged and stormy course while admitted, including difficult alternative dialysis access that unfortunately resulted in death eventually. Here we are highlighting the importance of recognition of hemoptysis as a presentation of SVC syndrome by explaining the underlying pathogenesis and possible management options.

Endovascular correction of cerebrovenous anomalies in multiple sclerosis: a retrospective review of an uncontrolled case series.

Endovascular intervention for obstruction to venous drainage of the head and neck is an established treatment for disorders such as superior vena cava syndrome. Some patients with multiple sclerosis have been observed to have anomalies of the veins draining the head and neck. It is possible that some symptoms associated with multiple sclerosis may be secondary to disturbed venous flow. In an uncontrolled clinical series of 40 patients who had been previously diagnosed with multiple sclerosis, anomalies of the venous drainage of the head and neck were observed, including venous stenoses of the internal jugular veins. In 38 of 40 patients, venous stents were placed with restoration of luminal dimensions and abrogation of the venous pressure gradient. The angiographic and hemodynamic improvement was associated with improvement in symptomatology, most particularly in cognitive and constitutional symptoms that may be related to cerebrovenous flow. Serious complications included death in one subject and stent embolization requiring open heart surgery in another. In conclusion, in this series, endovascular intervention to correct venous stenosis associated with multiple sclerosis was associated with improvement in symptoms possibly related to disturbed venous hemodynamics. However, given the serious adverse events in this small series, a randomized clinical trial is required to confirm these findings, and to determine if the procedure has any effect on the progression of multiple sclerosis, or untoward long-term adverse effects.

Hemophagocytic lymphohistiocytosis can mimic the superior vena cava syndrome.

We report a case of a 3-year-old boy with hemophagocytic lymphohistiocytosis (HLH), with enlarged cervical lymph nodes causing internal jugular vein compression, who initially presented a condition similar to the superior vena cava syndrome. Laboratory data along with neck node and bone marrow biopsies confirmed the HLH. Genetic analysis revealed the patient to be compound heterozygous for 2 variations of the perforin gene, c.1620 A>G and c.562C>G. This case featuring a rare initial manifestation of HLH that has not been previously reported, points to the necessity of considering this disease when symptoms similar to superior vena cava syndrome are encountered.

[Clinicopathologic study of 40 cases of mediastinal tumours of haematopoietic and lymphoid tissues].

OBJECTIVE: To study clinical and histopathological features, and diagnosis of mediastinal tumours of haematopoietic and lymphoid tissues (MTHL). METHODS: Forty cases of MTHL were analyzed for clinicopathology by microscopy and immunohistochemical staining and in situ hybridization, according to the updated 2008 WHO classification of tumours of haematopoietic and lymphoid tissues. RESULTS: In 40 cases of MTHL, there were 20 males and 20 females. The ratio of male/female was 1:1. The mean age was 31.8 years and median age was 29 years (range, 12 - 70 years).Superior vena cava syndrome was observed in 28 cases. The specimens of 4 cases were obtained by lumpectomy, whereas 36 cases by biopsy (25 cases by thoracoscopy, 1 by core needle aspiration). Twenty cases lay in anterior mediastinum, and 2 in posterior, 1 in superior, 8 in anterior and superior, 2 in posterior and superior, 2 in anterior and middle, 1 in middle and anterior mediastinum.Frozen section were performed in 28 cases, and 17 cases were diagnosed as tumours of haematopoietic and lymphoid tissues (consistency ratio was 60.7%). Twelve cases were classical Hodgkin lymphomas (cHL) (8 were nodular sclerosis subtype, and 3 were mixed cellarity, 1 was lymphocyte-rich subtype), and 10 were primary mediastinal (thymic) large B cell lymphoma (PMBCL), 10 were precursor lymphocyte neoplasm [8 were T lymphoblastic leukemia/lymphomas (T-LBL), 2 were B-LBL], 1 was MALT lymphoma, 1 was composite lymphoma (PMBCL and cHL), 2 were myeloid sarcomas, 4 were gray zone lymphomas (GZL) (3 had morphology reminiscent of cHL, and 1 of DLBCL, all cases were positive for CD20, PAX5, CD30 and CD15).EBER were detected in 11 cases by in situ hybridization, 2 of which were positive (18.2%), and the 2 positive cases were cHL. CONCLUSIONS: MTHLs occur predominantly in adolescents and young adults, mainly present as superior vena cava syndrome and anterior mediasinal masses. cHL, PMBCL, T-LBL were the most common MTHLs.GZLs mainly occur in young adults, those whose morphology reminiscent of cHL, immunohistochemistry reminiscent of PMBCL, and vice versa. Thoracoscopy, frozen section and a suitable panel of antibodies were practical approaches to MTHL.

Isolated Myeloid Sarcoma and Intracardiac Thrombus Resulting in Superior Mediastinal Syndrome.

Superior mediastinal syndrome (SMS) is a relatively common emergency in the practice of Pediatric Oncology. It typically results from the compression of large airways and superior vena cava by a swiftly growing mass. T-lineage acute lymphoblastic leukemia or lymphoma, neuroblastoma, and germ cell tumor are the common etiologies of SMS in children. Occasionally, SMS can be an unexpected presentation of less common childhood cancers and a surprise for the diagnostic and treating teams. The present paper reports the diagnostic and therapeutic challenge of managing a 9-y-old boy with SMS resulting from mediastinal myeloid sarcoma. The presence of a sizeable intracardiac thrombus, in addition, contributed to the SMS. The initial pleural fluid cytology and image-guided fine-needle aspiration cytology of the mediastinal mass were nondiagnostic. A thoracotomy was subsequently performed to debulk the tumor for symptomatic relief and obtain tissue for diagnosis.