HKCEM College Tutorial

A Man With Shortness Of Breath

AUTHOR DR. LAU CHU LEUNG, TERRY 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?

COPD CHF Asthma APO Pneumothoax 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 Cellulitis Angioedema Myxedema 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, UL Thin walled  extremely susceptible to extrinsic compression ▪ Immediately life-threatening oncologic emergency if airway compromise or CNS symptoms are present

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 Radiation Arteriosclerotic 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 Papilledema Confusion, coma ▪ ▪ ▪ ▪ ▪ ▪ ▪ 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

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 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 watchword Diuretics must be used judiciously to avoid hypovolemia restriction is the

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 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

SVCO - CXR

▪ Small-cell lung cancer

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

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 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 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 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

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  ▪ Bypass grafting using an autologous vein graft or a synthetic tube Surgery ▪ ▪ ▪ 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

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 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

The end