A Man With Shortness Of Breath

1. HKCEM College Tutorial A Man With Shortness Of Breath Author Dr. Lau chuleung, terry Nov., 2013

2. A Man With Shortness Of Breath… • Issue(s) identified? • HT • Tachypnea • Tachycardia • DDx of SOB? • COPD • CHF • Asthma • APO • Pneumothoax • Upper airway obstruction • Any red flags of headache? • 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

3. 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?

4. Revise your DDx? • Facial Swelling • Nephrotic syndrome • Cellulitis • Angioedema • Myxedema • Moon face (chronic steroids) • Superior vena cava obstruction • Melkersson-Rosenthal Syndrome - orofacial edema • 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

5. 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, UL • Thin walled  extremely susceptible to extrinsic compression • Immediately life-threatening oncologic emergency if airway compromise or CNS symptoms are present

6. 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 sclerosingaortitis • Fibrosingmediastinitis • Radiation • Arteriosclerotic • Infection - TB mediastinitis, luetic(syphilitic) aneurysm, histoplasmosis

7. If suspected SVCO…. • Early symptoms • Edema of face, neck, UL • SOB • Venous distension of upper chest, neck and face • Ruddy complexion (Plethora) • Dysphagia • Chest pain • Late symptoms • Severe respiratory distress • Cyanosis • Headache • Visual disturbances • Coma • Convulsions • Death • 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 • Papilledema • Confusion, coma

8. If venous dilatation over abdomen…significant?

9. 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 • Substernalgoitre

10. 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

11. 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 • Mediastinaltumour • Superior Mediastinal Syndrome – SVCO + tracheal compression

12. SVCO – Intravenous Access • Should be considered in lower limbs in the case of complete SVC obstruction • 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 watchword • Diuretics must be used judiciously to avoid hypovolemia

13. 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 radiation-induced oedema • Reduce tumor burden in lymphoma & thymoma  reduce obstruction • Risk • Obscuring the tissue diagnosis, especially if lymphoma is suspected • Steroid-induced acute tumourlysis syndrome

14. 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

15. 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 – substernalgoitre • Superior mediastinal widening • Hilar mass - bronchogenic carcinoma • Anterior mediastinal mass – lymphoma • Calcification – Histoplasmosis • Pleural/pericardial effusion

16. SVCO - CXR • Small-cell lung cancer

17. SVCO - CT (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

18. SVCO – CT Venogram • 4-cm thrombus in the SVC

19. 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

20. Kishi Scoring System

21. 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

22. SVC stenting • Complications 3-7% • Stent migration • Bleeding • Infection • 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 tumourlysis syndrome • Late complications • Bleeding (1-14%), death (1-2%)

23. 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

24. SVCO – Management Options

25. SVCO (Malignancy) - Management • Urgent treatment with radiotherapy and corticosteroids should be used only for life-threatening situations • Stridor, hypotension, collapse • Stenting is becoming increasingly used • Useful procedure for patients with severe symptoms such as respiratory distress that require urgent intervention • 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

26. 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

27. 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

28. 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

29. 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

30. 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

31. SVCO - Complications • Superior mediastinal syndrome • Rubin Syndrome – SVCO + spinal cord compression • Steroid-induced acute tumourlysis 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

32. References • 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 AnaesthAnalg 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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