20.02 SVCO - Hong Kong College of Emergency Medicine

HKCEM College Tutorial
A Man With
Shortness Of
NOV., 2013
A Man With Shortness Of Breath…
▪ M/65 Chronic smoker
▪ SOB for 2 days
▪ Increased when lying supine
▪ Headache, facial swelling
▪ BP 178/84 mmHg
▪ Pulse 124 bpm
▪ RR 20 /min, SpO2 97% RA
▪ T - 37.3 ºC
▪ Issue(s) identified?
▪ HT
▪ Tachypnea
▪ Tachycardia
▪ DDx of SOB?
Upper airway obstruction
▪ Any red flags of headache?
What are your immediate management?
▪ ABC - secure airway if necessary
▪ Oxygen
▪ Set intravenous access
▪ Monitoring – BP/P, SpO2, cardiac monitor
▪ While you get further history from patient, you notice…
▪ What are the DDx of SOB with dilated neck veins?
Revise your DDx?
▪ SOB + Dilated neck veins
Congestive heart failure
Right ventricular infarct
Superior vena cava obstruction
Cardiac tamponade
Constrictive pericarditis
Tension pneumothorax
Massive haemothorax
Massive pulmonary embolism
▪ Facial Swelling
Nephrotic syndrome
Moon face (chronic steroids)
Superior vena cava obstruction
Melkersson-Rosenthal Syndrome orofacial edema
What is Superior Vena Cava Syndrome?
▪ Conglomeration of s/s that results
from compression or occlusion of
the SVC
▪ SVC receives venous drainage from H&N,
▪ Thin walled  extremely susceptible to
extrinsic compression
▪ Immediately life-threatening
oncologic emergency if airway
compromise or CNS symptoms are
SVCO – When to suspect? Common causes?
▪ Dilatation of the two external jugular veins
▪ Increasing symptoms when the patient is in a horizontal position
▪ Malignant (90%)
▪ Ca bronchus
▪ Small-cell lung cancer (SCLC)
▪ Non-small-cell cancer (NSCLC)
▪ Lymphoma
▪ Metastatic disease
▪ Germ-cell cancer
▪ Thymoma
▪ Mesothelioma
▪ Benign (10%) - compression, infiltration, thrombosis
Indwelling central venous catheters
Thoracic aortic aneurysm (ascending)
Substernal goiter
Constrictive pericarditis
Primary thrombosis
Idiopathic sclerosing aortitis
Fibrosing mediastinitis
Infection - TB mediastinitis, luetic (syphilitic) aneurysm, histoplasmosis
If suspected SVCO….
▪ What are the common presentations?
▪ Physical signs?
▪ Facial edema, plethora
▪ Jugular venous distention
▪ Prominent superficial vascularity
▪ Neck & upper chest
Stokes sign – tightness of shirt collar
Edema of larynx or pharynx
Hoarseness, stridor
Cerebral edema, increased ICP
Confusion, coma
▪ Early symptoms
Edema of face, neck, UL
Venous distension of upper chest, neck and face
Ruddy complexion (Plethora)
Chest pain
▪ Late symptoms
Severe respiratory distress
Visual disturbances
If venous dilatation over abdomen…significant?
Any specific physical sign?
▪ Pemberton Sign
▪ Exaggeration of edema and flushing with placement of the patient’s arms overhead
▪ Indicates compression of vascular structures in the thoracic inlet
▪ Highly indicative of SVCO
▪ Substernal goitre
SVCO – Management Aims
▪ Recognition of life-threatening symptoms - airway compromise
and/or cerebral edema
▪ Confirmation of the presence of venous obstruction
▪ Imaging +/- interventions to establish the etiology
▪ Relief obstruction
▪ Treatment of the underlying cause
SVCO – ED Management
▪ Revise your Mx? Any precautions?
▪ Propped up position
▪ Elevate patient's head -  hydrostatic pressure (edema)
▪ Potential difficult airway
▪ Cannot lie flat
▪ Edematous epiglottis and vocal cords and narrowed glottic opening
▪ Mediastinal tumour
▪ Superior Mediastinal Syndrome – SVCO + tracheal compression
SVCO – Intravenous Access
▪ Should be considered in lower limbs in the case of complete SVC
▪ With partial obstruction, upper limb access is acceptable
▪ UL iv access  delays in resuscitation fluids and drugs reaching the
central circulation
▪ Induction time will be prolonged
▪ Overdose is a potential risk
▪ In the absence of major bleeding / hypotension, fluid restriction is the
▪ Diuretics must be used judiciously to avoid hypovolemia
SVCO – Any role of steroid?
▪ Glucocorticoid therapy (dexamethasone, iv 4 mg Q6H)
▪ Work mainly by reducing tumour and airway oedema
▪ Benefits documented only in case studies
▪ Generally used in conjunction with radiotherapy because of concern about radiationinduced oedema
▪ Reduce tumor burden in lymphoma & thymoma  reduce obstruction
▪ Risk
▪ Obscuring the tissue diagnosis, especially if lymphoma is suspected
▪ Steroid-induced acute tumour lysis syndrome
SVCO - Imaging
▪ Confirming the diagnosis of SVCO
▪ Identify the site and extent of the occlusion
▪ Presence of intravascular thrombus and collateral circulations
▪ Presence of collateral vessels is highly suggestive of SVCO
▪ Sensitivity of 96% and a specificity of 92%
▪ Identify its underlying cause
▪ Planning treatment
▪ Information on the length of the lesion
▪ Any involvement of the brachiocephalic veins
SVCO – CXR signs
▪ Signs of the development of collateral circulation
▪ Opacity above the right stem bronchus  dilation of the arch of the azygos
▪ Sub-aortic opacity or ‘‘aortic nipple’’ sign  dilation of the left superior intercostal vein
▪ Neck mass – substernal goitre
▪ Superior mediastinal widening
▪ Hilar mass - bronchogenic carcinoma
▪ Anterior mediastinal mass – lymphoma
▪ Calcification – Histoplasmosis
▪ Pleural/pericardial effusion
▪ Small-cell lung cancer
(a) Axial CT - Large right hilar mass obstructing SVC
Multiple chest wall collateral vessels
(b) Coronal CT - Compression of SVC distally (arrow)
Thrombosis of proximal SVC and brachiocephalic veins
(c) 3D CT - appearance of multiple collaterals of chest wall
SVCO – CT Venogram
▪ 4-cm thrombus in the SVC
SVCO - Venogram
▪ Invasive venography - gold standard
▪ Carried out prior to stenting to delineate the presence of an SVC stenosis or occlusion, and to identify
the extent of the obstruction
▪ Cannot be performed in isolation, as it cannot identify the cause of the obstruction
▪ Simultaneous bilateral arm venogram
▪ Defines obstruction and collateral circulation
▪ Identifies thrombus
▪ Figure
▪ severe compression of both the right and left subclavian veins (RSV and LSV)
▪ a thrombus in the left subclavian vein
▪ multiple venous collaterals
Kishi Scoring System
SVC stenting
▪ Advantages
▪ Rapid relief of the symptoms of venous congestion
▪ Relief can be immediate, but in most series, it is reported within 24 to 72 hours following the procedure
▪ Allowing treatment of underlying pathology to be initiated
▪ Stent can be placed before a tissue diagnosis is available
▪ Allows early cisplatin based chemotherapy to commence (requires hydration)
▪ Prevent the risk of death due to laryngeal or bronchial oedema
▪ Indications
▪ Symptomatic malignant SVCO
▪ Symptomatic benign SVCO
▪ known chemotherapy and radiation-resistant tumors
▪ No absolute contraindications to SVC stenting
▪ Relative contraindications
▪ Patient cannot lie flat or semisupine on the table
▪ Patient with malignancy with a very good chance of cure or remission
SVC stenting
▪ Complications 3-7%
Stent migration
Thrombosis (Figure: Reocclusion of the stent by thrombus on an (a) axial CT and (b) coronal CT)
SVC rupture
Pericardial tamponade
Hematoma at insertion site
Acute tumour lysis syndrome
Late complications
▪ Bleeding (1-14%), death (1-2%)
SVCO – Further Management
▪ In the absence of a need for urgent intervention, the management should focus initially on
establishing the correct diagnosis
▪ Treatment is directed at the underlying pathological process
▪ When malignancy is suspected without known primary cancer  tissue biopsy
Sputum cytology
Pleural fluid analysis
Excisional LN biopsy
Bone marrow
Bronchoscopy with transbronchial needle aspiration
SVCO – Management Options
SVCO (Malignancy) - Management
▪ Urgent treatment with radiotherapy and corticosteroids should be used only for lifethreatening situations
▪ Stridor, hypotension, collapse
▪ Stenting is becoming increasingly used
▪ Useful procedure for patients with severe symptoms such as respiratory distress that require urgent
▪ No evidence to support routine anticoagulation in patients with malignant SVCO in
the absence of thrombosis
▪ After a tissue diagnosis has been obtained and the extent of the disease has been
determined, a decision should be made to address control of the malignant process
in either a curative fashion or palliatively
▪ Radiation, chemotherapy, or stent placement, or a combination of these modalities
SVCO (Malignancy) - Chemotherapy
▪ Chemotherapy responsive tumour
▪ Non-Hodgkin lymphomas, small cell lung cancer, and germ cell tumors are widely regarded as
chemotherapysensitive tumors
▪ Good prognosis - high rates of response and quick onset of tumor shrinkage
▪ Less responsive tumours - non-small cell lung cancer, B-cell lymphoma
▪ Stents or RT/chemotherapy
SVCO (Malignancy) - Radiotherapy
▪ Relative contraindications
▪ Previous treatment with radiation in the same region
▪ Certain connective tissue disorders - scleroderma
▪ Known radioresistant tumor types – sarcoma
▪ Majority of tumor types are sensitive
▪ Improvement is often apparent within 72 hours
SVCO (Malignancy) – Surgical Management
▪ Thymomas are relatively resistant to chemotherapy and radiation  Surgery
▪ Bypass grafting using an autologous vein graft or a synthetic tube
▪ Good patency rates (80–90%)
▪ Major surgical procedure that requires careful patient selection
▪ High morbidity and 5% mortality rate
SVCO (Benign) - Management
▪ More insidious course  development of adequate collaterals
▪ Treatment is usually directed at the underlying cause
▪ Medical management with diuretics and steroids  NOT useful
▪ If symptoms caused by thrombus formation
▪ Thrombolysis followed by anticoagulation with heparin or warfarin
▪ Less effective in chronic thrombosis (with onset of symptoms more than 10 days previously)
▪ If symptoms develop rapidly
▪ SVC bypass surgery
▪ Endovascular stenting
SVCO – Iatrogenic / thrombotic
▪ Result from indwelling vascular hardware
▪ No evidence that removing the catheter in the ED provides any benefit
▪ Anticoagulation
▪ Percutaneous transluminal angioplasty +/- metallic stent
▪ SVCO may coexist with pulmonary embolism
SVCO - Complications
▪ Superior mediastinal syndrome
▪ Rubin Syndrome – SVCO + spinal cord compression
▪ Steroid-induced acute tumour lysis syndrome
▪ ‘‘Overload syndrome’’
▪ Opening of a SVC stenosis inducing a fast cardiac return of the third compartment
(oedema) may generate an ‘‘overload syndrome’’ with pre-capillary pulmonary
hypertension and pulmonary oedema
▪ Increased intracranial pressure
▪ Spontaneous intracranial hemorrhage
▪ Postgrad Med J 2013;89(1050):224–30
▪ Journal of Clinical Neuroscience 2013;20:1040–1
▪ Q J Med 2013;106:283–4
▪ Rosen’s Emergency Medicine 8th ed.
▪ Journal of Emergency Medicine 2012;43(6):1079–80
▪ South Afr J Anaesth Analg 2012;18(1):20-4
▪ BMJ 2011;343:d4466
▪ Visual Diagnosis in Emergency and Critical Care Medicine (2011)
▪ Ann Emerg Med. 2010;56:305
▪ Emerg Med Clin N Am 2009;27:243–55
▪ Irwin and Rippe’s Intensive Care Medicine (2008)
▪ NEJM 2007;356(18):1862-9
▪ Critical Care – Just the facts (2007)
▪ NEJM 2006;354 (8): e7
▪ Can J Emerg Med 2005;7(4):273-7
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