The SVC syndrome or superior vena cava syndrome- Oncological emergencies

Oncological emergencies - SVC syndrome or superior vena cava syndrome

Superior vena cava syndrome or superior vena cava obstruction prevent or restrict the venous return from the upper body to the heart which results in dilation of the arm and neck veis, dusky blue discoloration of the face, arms and upper chest, headache and swelling or odema of the face and neck. Superior vena cava syndrome or SVC syndrome is caused by compression of the vessel by mediastinal mass with or without any intraluminal thrombus. To compensate the matter, azygous vein will form a collateral circulation to provide drainage and after a few weeks collateral circulation will be formed in the chest. The blood will flow in these collateral veins from above downwards to the inferior vena cava.

The patient who suffer from superior vena cava syndrome or svc syndrome may present with headache, shortness of breath, sensation of choking, chest pain and swelling of the face and arms. Loss of venous pulsation on the distended neck vein , facial swelling/odema, plethora, cyanosis are the most important physical signs. The rate of obstruction and the development of the compensatory collateral circulation will determine the severity of the SVC syndrome. If the patient bends or lying flat the symptoms will get worse or deteriorate as it will further compromised the obstructed venous return.

In term of history the patient will suffer from minor symptoms of SVC for a long period.Most cases of SVC syndrome is associated with lung cancer such as small cell lung cancer, metastases breast cancer, lymphoma and germ cell tumors. The management of SVC syndrome includes relieving the symptoms and treat the underlying causes. It also depends on the severity, cause and the prognosis. SVC syndrome is one of the oncological emergencies which will compromise the airway. Delay in treatment will adversely affect the outcome. In this case, the patient is treated empirically with steroids and radiotherapy. If the patient suffer from minor symptoms of SVC syndrome for a long time, it is possible to delay the treatment to obtain the histological report so that the diagnosis of the condition is establish and optimum treatment can be given.

The procedure requires to diagnose SVC syndrome include plain chest x ray, sputum culture, thoracoscopy, bronchoscopy, mediastinoscopy, CT scan, MRI scan and venography. A biopsy of palpable lymph node may also be performed.

Patient with SVC syndrome is required to sit upright with oxygen therapy while corticosteroid is administered intravenously. The most appopriate treatment modality for the majority of the cases is radiotherapy that able to relieve the symptoms in fortnight in 90% of the cases. Chemotherapy is the optimal initial treatment if the SVC syndrome is associated with small cell lung cancer, lymphoma and germ cell tumor.

Insertion of expandable wire stents under radiological guidance is suitable and effective for patient who suffer from recurrent SVC syndrome or unsuitable for other treatment modalities. Recent medical studies shows that this technique provide good response rate and excellent symptomatic relief. However this technique is performed for patient with benign disorder. If the SVC syndrome is associated with thrombosis, then the central venous catheter line need to be removed and anticoagulation is prescribed to the patient. The low dose of warfarin ( anti coagulation ) has been shown to reduce the incident of formation of thrombus within the central venous catheter.

The non malignant cause of SVC syndrome includes:

Mediastinal fibrosis such as tuberculosis, idiopathic, histoplasmosis and actinomycosis.

Benign mediastinal tumor such as sarcoidosis, dermoid tumor, cystic hygroma, retrosternal goitre and aortic aneurysm. Vena cava thrombosis such as long term venous catheter, shunts, pacemaker, idiopathic, paroxysmal nocturnal haemoglobinuria and polycythemia vera.

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