Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
SUPERIOR VENA CAVA SYNDROME (SVCS) DEFINITION clinical expression of obstruction of blood flow through the SVC developing quickly or gradually in case of pathological process in the superior mediastinum causing compression, invasion or thrombosis ANATOMY AND PATHOPHYSIOLOGY Thin – walled Compliant Easily compressible Vulnerable to any space occupying process in its vicinity Extensive collateral system (azygos venous system, internal mammary veins, lateral thoracic veins, paraspinous veins, esophageal venous network, subcutaneous veins) Maintains blood at a low pressure ETIOLOGY Malignant conditions – 85% Lung cancer – underlying process in 70% Small cell lung cancer – the most common histologic subtype Lymphoma involving the mediastinum (8%) Other primary mediastinal malignancies: thymoma, germ cell tumors Metastatic mediastinal tumors (breast cancer, testis cancer) ETIOLOGY Nonmalignant conditions Thrombosis ( central vein catheters, pacemakers) Mediastinal fibrosis (e.g. due to radiotherapy or histoplasmosis) ETIOLOGY IN CHILDREN Mainly iatrogenic (70%) – secondary to cardiovascular surgery for congenital heart diseases, ventriculoatrial shunt for hydrocephalus, SVC catheterization for parenteral nutrition Congenital anomalies of the cardiovascular system Mediastinal tumors (lymphomas in 75%) SYMPTOMS Dyspnea Facial swelling or 63% 50% Head fullness Cough Arm swelling Chest pain Dysphagia Hoarseness 24% 18% 15% 9% PHYSICAL FINDINGS Venous distention of neck Venous distention of chest wall Facial edema Cyanosis Edema of arms Exophtalmus 66% 54% 46% 20% 14% DIAGNOSTIC PROCEDURES Chest film (superior mediastinal widening, pleural effusion, right hilar mass, bilateral diffuse infiltrates, cardiomegaly, calcified paratracheal nodes, mediastinal (anterior) mass) CT (more detailed information about the SVC, its tributaries and other critical structures such as bronchi and the cord) MRI Contrast venography (valuable if surgical bypass is considered for the obstructed vena cava); an alternative – radionuclide technetium-99m venography DIAGNOSTIC PROCEDURES Procedures that help to establish the histologic diagnosis are the priority! Sputum cytology Thoracocentesis (if there is pleural effusion) Supraclavicular lymph node biopsy Bronchoscopy (brushing, washing, biopsy samples) Percutaneous transthoracic fine-needle biopsy under CT or fluoroscopic guidance Mediastinoscopy Bone marrow biopsy Thoracotomy MANAGEMENT The treatment should be selected according to the histologic disorder and stage of the primary process! Goals: relieve symptoms attempt cure of the primary malignant process METHODS Radiotherapy Chemotherapy Thrombolytic therapy Anticoagulants Transluminal angioplasty and endoprosthesis insertion Surgery General measures RADIATION THERAPY Radiosensitive cancers (non-small cell lung cancer) Contraindications to chemotherapy Combination therapy: chemo- and radiotherapy (lymphomas, small cell lung cancer) Unknown histologic diagnosis in case of the deteriorating patient clinical status CHEMOTHERAPY Provides both local and systemic therapeutic activity Chemosensitive carcinomas Lymphomas Small cell lung cancer * Alone or in conjunction with radiotherapy INVASIVE METHODS Percutaneus transluminal angioplasty using baloon technique Insertion of expandable wire stents With or without thrombolytic therapy Successful in opening catheter induced SVC obstructions SURGERY Bypass grafts In oncologic patients - considered only after other therapeutic methods have been exhausted TROMBOLYTIC THERAPY Streptokinase Urokinase Recombinant tissue-type plasminogen activator *May cause lysis of the thrombus early in its formation Anticoagulants Heparin Oral anticoagulants * May reduce the extent of the thrombus and prevent progression GENERAL MEASURES Bed rest with the head elevated Oxygen Steroids PROGNOSIS Strongly correlates with the prognosis for the underlying disease Average survival for cancer patients: 7-8 months 5 months for lung cancer patients Average survival in case of primary benign process: 9 years