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DISEASES OF THE DIAPHRAGM, CHEST WALL, PLEURA, AND MEDIASTINUM 1 Cecil Medicine, 23rd ed. Chapter 100 BOOK REVIEW CONFERENCE 2 A. THE DIAPHRAGM The Diaphragm 3 m/i respiratory muscle separate the thoracic and abd cavities 2 components (Central noncontractile tendon and Muscle fibers) Controlled by phrenic n Respiratory function Diaphragmatic contraction -> abd contents downward, ribs outward -> negative intrathoracic pressure Nonrespiratory function (speech, defecation, and parturition) - THE DIAPHRAGM Dysfunction and Fatigue 4 TABLE Diaphragmatic 100-1 -- THERAPEUTIC dysfunction MODALITIES TO IMPROVE DIAPHRAGMATIC FUNCTION - Lung MECHANICAL hyperinflation (asthma REDUCE LOAD or COPD) -> shorten and flatten the diaphragm -> not generate the normal expanding action on the thorax -> coupled with ↑airway resistance Decrease resistance-> (administer bronchodilators, treatdemand infection, decrease and ↓ airway lung compliance ↑ work of breathing -> energy outstript the inflammation) supply -> Reduce hyperinflation muscle fatigue, ventilation fail Decrease ventilatory requirement (administer oxygen, control fever, avoid caloric loads) IMPROVE RESPIRATORY Diaphragmatic fatigueMUSCLE CONTRACTILITY AND ENDURANCE - Rapid and shallow breathing, respiratory alternans, and abdominal paradox Administer oxygen therapy Improve nutrition and acidosis -> noninvasive or invasive mechanical ventilation - Hypercapnea Improve cardiovascular performance Correct electrolytes (sodium, potassium, calcium, phosphorus) Administer drugs that improve contractility (β2-agonist, caffeine) Check for hypothyroidism or drugs that impair contractility (aminoglycosides) Provide ventilatory muscle training IMPROVE RESPIRATORY MUSCLE COORDINATION AND ENERGY CONSERVATION REHABILITATION RESPIRATORY MUSCLE RESTING THE DIAPHRAGM Disorders of Diaphragmatic motion 5 Diaphragmatic paralysis - Unilateral paralysis 1. Usually secondary to phrenic n involvement by tumor, neurologic diseases or idiopathic 2. Elevated diaphragmatic leaflet on chest radiograph ->paradoxical diaphragmatic motion with sniffing and coughing on fluoroscope 4. Usually asymptomatic * Irreversible symptomatic -> surgical plication of the affected hemidiaphragm - Bilateral paralysis 1. High cervical trauma, neuropathies, or myopathies 2. Symptomatic early, dyspnea (worsened by the supine position) 3. Inspiratory abdominal paradoxical retraction -> transdiaphragmatic pressure and/or electromyographic recording 4. Ventilatory failure -> intermittent mechanical ventilation * Permanent paralysis with intact muscle function -> diaphragmatic pacing THE DIAPHRAGM Disorders of Diaphragmatic motion 6 Diaphragmatic hernias - Congenitally week or incompletely fused area, esophageal hiatus(>70%), or traumatic rupture of muscle - Anterior hernia (foramina of Morgagni, obese, Rt cardiophrenic angle), Posterior hernia (foramina of Bochdalek, infants, Lt >Rt) - Severity 1) extension of abdominal contents 2) strangulation - Chest radiograph -> CT scans, gastrointestinal contrast films, radioisotope scan of the liver - Infants, large -> immediate surgical correction Asymptomatic, adult with previous evidence of a hernia->observation Surgery for diagnosis or relief strangulation THE DIAPHRAGM Disorders of Diaphragmatic motion 7 Hiccup - Produced by spasm of the diaphragm followed by closure of the glottis during - an inspiratory effort Self-limited, may for days or weeks Most unknown cause, occasionally sign of disease ( CNS disorder, uremia, herpes zoster, diaphragmatic irritation), and psychogenic Subside spontaneously or improve initiating disease Chronic or debilitating -> local anesthesia or phrenic n crushing THE DIAPHRAGM 8 B. THE CHEST WALL The Chest Wall 9 Consists of the bony thoracic cage (ribs, sternum, and vertebrae) and the repiratory muscles Thoracic cage (determinant of ventilation and static and dynamic lung volumes) -> diseases of the bony thoracic cage-> alter the ventilation and ventilationperfusion relationship -> hypoxemia or hypercapnia TABLE 100-2 -- MOST IMPORTANT RIB CAGE DERANGEMENTS SPINE Scoliosis (idiopathic, congenital, paralytic) Kyphosis Ankylosing spondylitis STERNUM, RIBS, OR PLEURA Pectus excavatum Thoracoplasty Fibrothorax THE CHEST WALL Derangements of the Chest Wall 10 1. Kyphoscoliosis(m/c) Scoliosis (lateral angulation of the spine, Rt >Lt, convexity of the curvature) Kyphosis (less important, anteroposterior angulation of the spine) Cobb's angle >70◦(respiratory dysfunction), >120◦ (dyspnea, respiratory failure) Ribs over the convex side separated and rotated posteriorly & Ribs over the concave side crowded, displaced anteriorly(decreased thoracic height) >forward bulging of the anterior wall and kyphoscoliotic hump Usually idiopathic, begin in child, ventilatory failure in the 4th to 6th decade Static lung volume↓, lung compliance↓, hypoxemia, hypercapnia Therapeutic approaches -Surgical correction(cosmetic effects, minimal improvement in puImonary function) -Oxygen, Intermittent positive pressure ventilation, nighttime ventilatory assistance, stop smoking, aggressive treatment of bronchospasm and respiratory infection, weight loss THE CHEST WALL Derangements of the Chest Wall 11 2. Ankylosing Spondylitis fusion of the costotransverse and vertebral joints Inspiratory relative fixation of the rib cage Ventilatory failure are rare 3. Pectus Excavatum Congenital deformity of the lower portion of the sternum -> symmetrical bowing of the anterior ribs Heart, mediastinal structures laterally displaced ->some fail to increase cardiac output normally during exercise THE CHEST WALL Derangements of the Chest Wall 12 4. Fibrothorax Pleural diseases (hemothorax or asbestosis) Treatment is similar to that for kyphoscoliosis, pleurectomy (pleural fibrosis) 5. Flail Chest Double fractures of ≥3 adjacent ribs or combined sternal and rib fractures Flail segment paradoxically moves inward during inspiration -> inefficient ventilation -> ↑work of breathing m/c accidental chest trauma or after CPR supportive care with oxygenation, clear airways, and prevention of infection, artificial ventilation(ventilatory failure), and chest fixation (large flail chest ) THE CHEST WALL 13 C. THE PLERA Definition 14 Parietal Pleura Visceral Pleura Cover surfaces chest wall, diaphragm, and mediastinum Lungs, including interlobar fissures Blood supply systemic circulation pulmonary circulation Sensory nerves yes no Consists of a layer of mesothelial cells, smooth semitransparent Supported by network of connective and fibroelastic tissue, lymphatics, and vv Rich microvilli of mesothelial cells -> deliver glycoproteins (hyaluronic acid) -> ↓ friction between the lung and chest wall THE PLEURA Physiology 15 5 ~ 10 mL pleural fluid in cavity facilitates lung expansion and helps maintain lung inflation Pleural fluid -> low protein concentration(<2 g/dL), similar pH andOFglucose value of blood TABLE 3 MECHANISMS THAT LEAD TO ACCUMULATION PLEURAL FLUID -> form from parietal pleura ↑ hydrostatic pressure in theonmicrovascular (heart failure) -> turnover depends the Starlingcirculation forces and gravity gradient *hydrostatic : parietal pleura 30 (severe cm H2O, visceral pleura 10 cm H2O ↓oncotic pressurepressure in the microvascular circulation hypoalbuminemia) *oncotic pressure : 25 cm H2O ↓pressure in the pleural spaceover (lungthe collapse) -> drainage to stomas parietal surface of the low mediastinum, low chest wall, and diaphragm seem to empty into the subpleural lymphatics ↑permeability of the microvascular circulation (pneumonia) Accumulation of pleural fluid Impaired lymphatic drainage from the pleural space (malignant effusion) ↑hydrostatic force or ↓oncotic pressure ->low-protein transudates ↑outpouring capillaries or cells or (ascites) blocking of lymphatics -> high-protein Movement of fluidby from the peritoneal space exudates THE PLEURA Clinical Manifestations 16 Pain, dyspnea, or cough -> not sensitive and specific (absent in some large effusions and in critically ill patients) Pain (unilateral, sharp, worsens with inspiration or cough and radiate to the shoulder, neck, or abdomen) Dyspnea (compression of lung tissue and mechanical alterations in the respiratory muscles as the fluid changes) Physical examination decreased breath sounds dullness with absent tactile fremitus THE PLEURA Diagnosis 17 A. Radiologic Examination Effusion ->blunting, medial displacement of the sharp costophrenic angle Subpulmonic effusion -> elevation of the hemidiaphragm or widening of the shadow between the gas-containing stomach and the lower left lung margin 300 mL fluid may fail to be seen on PA chest radiograph 150 mL fluid may be seen on a lateral decubitus view Supine film may obscure the diagnosis because the fluid layers posteriorly THE PLEURA Diagnosis 18 A. Radiologic Examination pseudotumor - fluid loculation in an interlobar fissure(m/c minor fissure) -> mass-like - diagnosis is the presence of pleural fluid elsewhere and a biconvex lenticular configuration Hydropneumothorax(horizontal concave margins) Pneumothorax (contrast between the water density of the visceral pleura centrally and the gas radiolucency without vascular markings laterally, expiratory film may help outline small pneumothorax) THE PLEURA Diagnosis 19 A. Radiologic Examination US and CT provide better definition of pleural and parenchymal abnormalities FIGURE 100-1 Ultrasound image of the left hemithorax FIGURE 100-2 Computed tomography of the patient shown in Figure 100-1 THE PLEURA Diagnosis 20 B. Diagnostic procedures 1. Thoracentesis and pleural fluid analysis 2. Percutaneous pleural biopsy 3. Exploration of the pleura 1. Thoracentesis and pleural fluid analysis Diagnostic thoracentesis - diagnostic in approximately 75%, help exclude other important Dx(empyema) * As a rule, newly discovered effusions should be tapped !!! - no absolute contraindications - relative contraindications -> bleeding diathesis, anticoagulation, small volume, mechanical ventilation, and a low benefit-to-risk ratio Therapeutic thoracentesis - no ≥1000 to 1500 mL at one time because of the re-expansion pulmonary edema) THE PLEURA Diagnosis 21 1. Thoracentesis and pleural fluid analysis Differentiate “transudate” or “exudate” - Not absolute, but helpful in suggesting further evaluation and possible Dx - Exudates defined by least 1 (1)pleural fluid–serum protein ratio >0.5 (2) pleural fluid–serum LDH ratio >0.6 (3) pleural fluid LDH concentration > 200 IU/L TABLE 100-4 -- CHARACTERISTICS OF PLEURAL FLUID TRANSUDATES Absolute Value Pleural Fluid/Serum Value Protein <3 g/dL <0.5 Lactate dehydrogenase <200IU/L <0.6 Glucose >60 mg/dL 1.0 White blood cell count Cholesterol <1000/mm3 <45 mg/dL — THE PLEURA Diagnosis 22 Malignancy, empyema (pus), tuberculosis (positive AFB in smears or culture), fungal infection (positive potassium hydroxide stains or culture), pleuritis of SLE (LE cells), chylothorax (↑ TG or chylomicrons), urinothorax (pleural fluid–serum creatinine ratio >1), and esophageal rupture (↑amylase and ↓pH) Corrrelation of pleural fluid exudate findings and causative disease PMNs (bacterial infection), lymphocytes (tuberculosis, lymphoma, leukemia), eosinophils (non-specific, long-standing fluid or air, previous thoracentesis) - bloody effusion (trauma, malignancy, pulmonary infarction), white effusion(chyle, cholesterol, lymphoma), black fluid (aspergillosis), yellow-green (rheumatoid pleurisy - putrid odor (anaerobic empyema), ammonia odor(urinothorax) - ADA (tuberculosis) (see later text) - Complications of thoracentesis (pain, bleeding, pneumothorax, infection, and spleen or liver puncture THE PLEURA Diagnosis 23 TABLE 100-5 -- CORRELATION OF PLEURAL FLUID EXUDATE FINDINGS AND CAUSATIVE DISEASE Tests Diseases pH <7.2 Empyema, malignancy, esophageal rupture, rheumatoid, lupus, and tuberculous pleuritis Infection, rheumatoid pleurisy, tuberculous and lupus effusions, esophageal rupture Pancreatic disease, esophageal rupture, malignancy, ruptured ectopic pregnancy Collagen vascular disease Glucose (<60 mg/dL) Amylase (>200 μg/dL) RF, ANA, LE cells Complement (decreased) SLE, rheumatoid arthritis RBCs (>5000/μL) Trauma, malignancy, pulmonary embolus TG>110 mg/dL Tuberculosis, violation of the thoracic duct Biopsy (+) Malignancy ADA (>40 μg/L) Tuberculosis THE PLEURA Diagnosis 24 2. Percutaneous Pleural Biopsy undiagnosed exudative effusion (particularly those with lymphocytic predominance, most frequently malignancy or tuberculosis) under local anesthesia with a hook-type needle Contraindications (small or loculated pleural effusion, uncooperative, and anticoagulation or a bleeding diathesis) Multiple samples are needed (pleural seeding not be uniform) diagnostic 60% for malignancy and 75% for tuberculosis 3. Exploration of the Pleura 5 to 10% of patients with undiagnosed effusion, the effusion itself disappears spontaneously or the cause becomes evident and necessary to make a diagnose VATS under local anesthesia, high yield (>85%) Open pleural biopsy under general anesthesia, larger specimens and concomitant lung tissue. THE PLEURA Differential Diagnosis 25 A. Transudative Effusion Biventricular failure with venous hypertension (m/c cause) often bilateral, usually Rt>Lt, vascular congestion and cardiomegaly Thoracentesis is indicated if febrile, large and unilateral effusions, pain/unexplained hypoxemia liver cirrhosis (5~10% ) - movement of ascitic fluid, Rt>Lt - radioactive tracer injected in the ascitic fluid appears in the chest - ascites control, Occasionally chemical pleurodesis for symp, recurrent effusions nephrotic syndrome (20%) - ↓oncotic pressure (hypoalbuminemia) and ↑ hydrostatic forces, peritoneal dialysis, or atelectasis - Bilateral - correct the protein-losing nephropathy THE PLEURA Differential Diagnosis 26 B. Exudative Effusions 1. Infections Parapneumonic effusion (m/c) - Uncomplicated & complicated - Uncomplicated effusions -> resolve with antibiotics, moderate PMNs, a glucose value similar to that of blood, pH >7.30, and an LDH <500 U/L Complicated effusions -> drainage, large numbers of PMNs(>100,000/mm3), pH < 7.20, glucose <40 g/dL, and LDH >1000 U/L - Purulent and bacteria -> immediate drainage - Persistent fever for 48 to 72 hours with complicated effusions -> drainage is inadequate ?(loculated) -> VATS, intrapleural streptokinase -> antibiotic is inappropriate? -> diagnosis is wrong? THE PLEURA . Differential Diagnosis 27 2. Tuberculosis Small, moderate effusion on chest radiograph,1/3 parenchymal dis Protein(>4g/dl), WBC about 5000 cell/mm3, lymphocyte dominant( PMN dominant for the first few days), low glucose, pH 7~ 7.3( pH > 7.4 vitually excluding tuberculosis), ADA( >40ug/L), mycobacterial antigen (rapid diagnosis) Multiple samples from closed pleural biopsy (50~80 % positive), cultures(30~70 % positive) 3. Immunologic causes of pleural effusion RA(5%) low glucose(<30ng/dl), low pH, high LDH, low complement, high RA factor SLE(5%) normal pH and glucose, hemolytic complement(C3,C4), LE cells ANA ratio >1:160 THE PLEURA Differential Diagnosis 28 4. Malignancy Lung ca invasion(m/c), breast ca, ovarian ca, gastric ca Abentant RBCs(30,000 to 50,000 /mL), Lymphocyte dominant, occasionally transudate(5 to 10%), positive cytology(60%), positive biopsy(70%, repeated thoracentesis) 5. Hemothorax Pleural blood (Hct>20%) Trauma, hematologic disorders, pulmonary infarction, pleural malignancy 6. Malignant mesothelioma 80 to 90 % asbestos exposure Dispend, cough, Wt loss, pain Massive effusion, often bloody, 70% pH <7.3 Elevated hyaluronic acid, special stain, electron microscopy of biopsy tissue 29 7. Pneumothorax (1) perforation of visceral pleura and gas from the lung (2) penetration of the chest wall, diaphragm, mediastinum, esophagus (3) gas generated microorganisms in empyema Simple spontaneous pneumothorax healthy, 20 to 40 yrs, spontaneous rupture of subpleural blebs at the apex Rt>Lt, frequent recurrence, acute pain, dyspnea, cough, decreased breath sounds, tactile fremitus, ipsilateral hyperresonance visceral pleural line on chest radiograph small(<20%), asyptomatic -> observation -> reabsorbed in 7 to 14 days large(>50%), symptomatic, tension pneumothorax -> chest tube Secondary or complicated pneumothorax Trauma, pulmonary diseases(emphysema ,m/c) chest tube 30 D. THE MEDIASTINUM Definition 31 Mediastinum - bounded by the thoracic inlet, diaphram, sternum, vertebral bodies, and pleura - contain many vital structures Anatomic compartments Anterior From the sternum to the pericardium, ascending aorta, and brachiocephalic vessels contains the thymus, thyroid gl, parathyroid gl, blood vv, pericardium, and LNs Middle Figure 100-4. Anatomic Compartment of the Mediastinum to the posterior pericardium contains the heart, great vv, trachea, main bronchi, LNs, phrenic n, and vagus n Posterior to the dorsal chest wall contains the vertebrae, descending aorta, esophagus, thoracic duct, azygous and hemizygous veins, vagus, sympathetic chains, and LNs THE MEDIASTINUM Clinical Manifestations 32 Most mediastinal masses are asymptomatic m/c symptoms are chest pain, cough, hoarseness, and dyspnea - stridor, dysphagia, and Horner's syndrome infrequently - Syndromes associated with primary mediastinal lesion Myasthenia gravis in thymoma - Hypoglycemia in mesothelioma, fibrosarcoma, and teratoma - Hypercalcemia in parathyroid tumors - Neurologic symptoms in neurogenic tumors - Physical examination is usually nonspecific - superior vena cava obstruction (facial edema, dilated veins, and arm edema) THE MEDIASTINUM Diagnosis 33 Chest CT - - - initial procedure of choice Other radiologic evaluation angiography, esophagography, and MRI Asymptomatic and benign by CT careful follow-up Histologic diagnosis mediastinoscopy or mediastinotomy for a nterior and middle compartment lesions Thoracotomy for middle and posterior co mpartment lesions or when surgery is th e treatment of choice for the suspected l esion CT-guided needle aspiration has become the procedure of choice in many centers Figure 100-5,6,7. PA and lateral chest radiograph of patient with a mass in the anterior mediastinum. The mass proved to be a dermoid cyst in CT scan. THE MEDIASTINUM Diagnosis 34 Tumors m/c cause of a mediastinal mass in older pts is a metastatic carcinoma (m/c bronchogenic carcinoma) - In young adults, primary mediastinal pathology is more frequent - Anterior Middle Posterior Thymoma Lymphoma Neurogenic tumors Lymphoma Cancer Enteric cysts Teratogenic tumors Cysts Esophageal lesions Thyroid aneurysms Aneurysms Diaphragmatic hernias (Bochdalek) Parathyroid aneurysms Hernia (Morgagni) Table 100-6. Most Frequent Causes of Mediastinal Masses THE MEDIASTINUM Diagnosis 35 Tumors 1. Neurogenic tumors - M/c(20%) posterior mediastinal mass, most benign - Nonspecific chest pain, nonproductive cough - Nerve sheath (neurilemoma, neurofibroma) or sympathetic ganglion cells (ganglioneuroma) - Pheochromocytomas may occasionally arise in the mediastinum - Treatment resection * Neuroblastomas (malignant tumor) require postoperative radiation therapy 2. Thymomas (20%) - Anterior mediastinum - 2/3 malignant - 40% Myasthenia gravis, paraneoplastic syndromes (Cushing's syndrome, refractory anemia, and hypogammaglobulinemia) - Treatment regarded as malignant, and surgical resection followed by radiation therapy THE MEDIASTINUM Diagnosis 36 3. Lymphatic tumors (17%) - Anterior mediastinum - Hodgkin's lymphoma (most frequent and best prognosis), Non-Hodgkin's lymphoma, plasmacytomas, and angiomatous lymphoid hamartomas (worse prognosis) 4. Teratomatous tumors (10%) - anterior compartment - 1/3 malignant, more frequent cystic teratomas - embryologically and histologically linked to the thymus - Contain squamous cells, hair follicles, sweat gl, cartilage, and calcifications 5. intrathoracic goiter (10%) - Anterior mediatinum - usually benign nodular or follicular enlargement of the thyroid gl - 3/4 stridor, cough, and dyspnea - occasionally cause superior vena cava syndrome THE MEDIASTINUM Diagnosis 37 6. Benign cysts - Asymptomatic - Bronchogenic cysts ; middle and posterior compartments (around the paratrachea or carina), lined with respiratory epithelium and cartilage (not communicate with the tracheobronchial tree) - Pericardial cysts ; anterior compartment (cardiophrenic angle), endothelial or mesothelial lining - Enteric cysts ; posterior mediastinum, lined by gastric or intestinal epithelium, may become infected, bleed, or rupture 7. Vascular tumors - Vascular hamartomas, lymphangiomas, and hemangiomas -> benign - hemangiopericytomas -> malignant - 8. Hernias through the diaphragm - foramen of Morgagni, foramen of Bochdalek, esophageal hiatus(m/c) THE MEDIASTINUM Diagnosis 38 Pneumomediastinum - Tears in the esophagus or tracheobronchial tree ( -> traumatic) or alveolar - rupture ( -> spontaneously or complication of artificial ventilation) Subcutaneous emphysema or pneumothorax, or both. Retrosternal pain and dyspnea, classic crepitus of subcutaneous emphysema (Hamman's sign) Lateral chest radiograph usually diagnostic Simple, spontaneous -> generally resolves without treatment Severe or organ rupture -> surgical drainage and repair Superior Vena Cava Syndrome - Obstruction of blood flow through the superior vena cava - Causes dilation of the collateral veins of the upper thorax and neck and edema - and congestion of the face Headache, dyspnea, dysphagia, and wheezes Malignancy m/c cause ( m/c bronchogenic carcinoma, 2nd lymphoma) invasive procedures are contraindicated by tumor, Irradiation, chemotherapy, or stent placement should be initiated before attempts are made to obtain mediastinal tissue THE MEDIASTINUM