Multiple Myeloma

Clinical Average age is 60-70 Men much more common than women Most have an elevated serum protein with 80-90% in the globulin fraction, especially IgG Bence-Jones protein in 40-60% of patients (light chains) X-ray findings Osteoporosis is most common skeletal abnormality in this disease Lesions are usually multiple and found in vertebrae, ribs, skull, pelvis, and femur Over 50% of solitary lesions are found in vertebrae Mandible involved in 1/3 of patients with diffuse involvement Widespread lucencies in bone Discrete, “punched-out” lesions Uniform in size Distinctive to MM are the lucent, elliptical, subcortical shadows, especially in long bones=endosteal scalloping Due to buttressing since MM is usually a slower process than mets In spine, MM destroys body and spares pedicle DDX: mets and disuse osteoporosis MM is more widespread More discrete holes in MM Large foci of coalescence more often due to mets Severe disuse osteoporosis may simulate bone changes of MM Sclerosis is usually seen only with treatment or fracture Bone scans may typically be negative and many hot  areas on scan may be healing fractures Most believe that almost all patients with a solitary plasmacytoma will develop multiple myeloma Solitary plasmacytoma produces “soap-bubbly” expansile, septated lesion, when characteristic

 

Pneumothorax Tension Pneumothorax

Presence of air in the pleural space Anatomy Visceral pleura is adherent to lung surface There is no air in the pleural space normally The introduction of air into the pleural space separates the visceral from the parietal pleura In contradistinction, the visceral and parietal pleura usually do not separate from each other in obstructive atelectasis Pathophysiology Either from disruption of visceral pleura Or, trauma to parietal pleura Clinical findings Acute onset of Pleuritic chest pain Dyspnea (in 80-90%) Cough Back or shoulder pain Etiologies Penetrating trauma Blunt trauma May be due to rib fracture May be caused by increased intrathoracic pressure May lead to bronchial rupture “Fallen lung sign” (ptotic lung sign) --  hilum of lung is below expected level within chest cavity Persistent pneumothorax with functioning chest tube Iatrogenic Tracheostomy Central venous catheter attempt or insertion Mechanical ventilation May occur in up to 25% of patients maintained on PEEP May be bilateral or under tension Thoracic irradiation Spontaneous pneumothorax Most common etiology Cause Rupture of subpleural blebs in apical region of lung Age 20-40 years M:F = 8:1 Especially in patients who are tall and thin Smokers Red arrows point to thin white visceral pleural line which is the single best sign for a pneumothorax Prognosis Recurrence in 30% on same side Recurrence in 10% on contralateral side Treatment Simple aspiration (success in >50%) Tube thoracostomy (effective in 90%) Other causes of a pneumothorax Neonatal disease Meconium aspiration Respirator therapy for hyaline membrane disease Malignancy Primary lung cancer Lung metastases, especially from osteosarcoma Also pancreas, adrenal, Wilms tumor Pulmonary infections Tuberculosis Necrotizing pneumonia Coccidioidomycosis Hydatid disease Pertussis Acute bacterial pneumonia Staphylococcal septicemia AIDS (Pneumocystis carinii, Mycobacterium tuberculosis, atypical mycobacteria) Complication of pulmonary fibrosis Histiocytosis X Idiopathic Cystic fibrosis Sarcoidosis Scleroderma Eosinophilic granuloma Interstitial pneumonitis Rheumatoid lung Idiopathic pulmonary hemosiderosis Pulmonary alveolar proteinosis Biliary cirrhosis Asthma or emphysema Produce a second peak incidence of pneumothorax from 45-65 years of age Due to rupture of peripheral emphysematous areas “Catamenial pneumothorax” is a recurrent spontaneous pneumothorax that occurs during menstruation and is associated with endometriosis of the diaphragm R >> L Marfan’s syndrome Ehlers-Danlos syndrome Pulmonary infarction Lymphangiomyomatosis and tuberous sclerosis Incidence of pneumothorax is particularly high in lymphangiomyomatosis and histiocytosis X Types of pneumothorax Closed pneumothorax = intact thoracic cage Open pneumothorax = "sucking" chest wound Tension pneumothorax Accumulation of air within pleural space due to free ingress and limited egress of air Pathophysiology: Intrapleural pressure exceeds atmospheric pressure in lung during expiration (check-valve mechanism) Frequency In 3-5% of patients with spontaneous pneumothorax Higher in barotrauma (mechanical ventilation) Tension pneumothorax on left (blue arrow) is displacing the heart and mediastinal structures to the right (red arrow); this case also shows a deep sulcus sign on the left (yellow arrow). There is underlying hyaline membrane disease. Simple pneumothorax –no shift of the heart or mediastinal structures Imaging findings in pneumothorax Must see the visceral pleural white line Very thin white line that differs from a skin fold by its thickness Absence of lung markings distal or peripheral to the visceral pleural white line Not evidence enough to say there is a pneumothorax only if there are no lung markings seen No lung markings will be seen with bullous disease Bullae have a concave surface facing the chest wall Pneumothorax almost always has a convex surface facing the chest wall Displacement of mediastinum and/or anterior junction line Deep sulcus sign On frontal view, larger lateral costodiaphragmatic recess than on opposite side Diaphragm may be inverted on side with deep sulcus Total / subtotal lung collapse This is passive or compressive atelectasis Collapse of SVC or IVC due to decreased systemic venous return and decreased cardiac output Tension hydropneumothorax Sharp delineation of visceral pleural by dense pleural space Mediastinal shift to opposite side Air-fluid level in pleural space on erect chest radiograph Radiographic signs in upright position White margin of visceral pleura separated from parietal pleura Usually seen in the apex of the lung Absence of vascular markings beyond visceral pleural margin May be accentuated by an expiratory film in which lung volume is reduced while amount of air in pneumothorax remains constant so that relative size of pneumothorax appears to increase Radiographic signs in supine position Anteromedial pneumothorax (earliest location) Outline of medial diaphragm under cardiac silhouette Deep sulcus sign Decubitus views of the chest may demonstrate a pneumothorax on the side that is non-dependent Left lateral decubitus view for right-sided pneumothorax Right lateral decubitus view for left-sided pneumothorax Subpulmonic pneumothorax (second most common location) Hyperlucent upper abdominal quadrant Deep lateral costophrenic sulcus Sharply outlined diaphragm in spite of parenchymal disease Visualization of anterior costophrenic sulcus Visualization of inferior surface of lung Apicolateral pneumothorax (least common location) Visualization of visceral pleural line Posteromedial pneumothorax (in presence of lower lobe collapse) Lucent triangle with vertex at hilum V-shaped base delineating costovertebral sulcus Pneumothorax outlines pulmonary ligament Pitfalls in diagnosis Skin fold Thicker than the thin visceral pleural white line Air trapped between chest wall and arm Will be seen as a lucency rather than a visceral pleural white line Edge of scapula Follow contour of scapula to make sure it does not project over chest Overlying sheets Usually will extend beyond the confines of the lung Hair braids Prognosis Resorption of pneumothorax occurs at a rate of 1.25% per day (accelerated by increasing inspired oxygen concentrations)

Tension Pneumothorax. Radiograph of the chest shows a large left-sided pneumothorax (white arrows) which is under tension as manifest as displacement of the heart to the right (black arrow) and depression of the left hemidiaphragm (yellow arrow).

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Coarctation Of the Aorta
 

l    General

o       2X more common in males

l    Common classification

o       Infantile or preductal form

o       Adult or juxtaductal form

l Adult Form

o       Adult or juxtaductal (postductal) form is more common

o       Usually localized

o       Area of coarctation is just beyond the origin of LSCA at level of ductus

l Infantile Form

o       Infantile, preductal form = diffuse type

o       Long, tubular segment of narrowed aorta

§ From just distal to innominate to level of ductus

o       Intracardiac defects (VSD,ASD, deformed mitral valve) present in 50% of diffuse type

§  Also patent ductus arteriosis

l Other Classifications

o       More complicated classifications take following into account:

§  Location and length of coarct

§  Patency of ductus arteriosis

§  Relationship of coarct to ductus

l Associated Defects

o       Bicuspid aortic valve (most common associated defect seen in 75-80%)

o       VSD

o       ASD

o       Transposition

o       Found in 25% of patients with Turner’s Syndrome

l Shone Syndrome

o       Coarctation

o       Aortic stenosis

o       Parachute mitral valve

o       Supravalvular mitral ring

l Imaging findings

Coarctation of the aorta.
See photo below for labeled close-up of upper thorax.
Click here or on photo to enlarge.

o       Rib Notching

§         Single best sign

§         Older the person, more likely to have rib notching

§         Majority have it over 20 years of age

§         Rib notching occurs in the high pressure circuit

§         Most often involves 4th-8th rib

·  Sometimes may involve 3rd and 9th

·  Does not involve 1st and 2nd ribs

·  Intercostals come off costocervical trunk and do not supply collateral flow to descending aorta

§         4th-8th do anastomose with internal mammary to form collaterals for descending aorta

§         Rib Notching–Unilateral

·         Isolated Right sided notching occurs when LSCA is involved in actual coarctation

·         Isolated Left sided notching can occur if there is an aberrant RSCA which arises from below coarct

o       Figure 3 Sign

§         Caused by (in order) either a dilated LSCA or aortic knob,  “tuck” of coarct itself, and poststenotic dilatation

§         Occurs in 1/3–1/2 of patients with coarct

§         Matched by “reverse 3” or “E” on barium-filled esophagus

o       Convexity of left side of mediastinum just above aortic knob 2° to dilated LSCA

o       Convexity of ascending aorta in 1/3

Close up of upper thorax in a patient with Coarctation of the Aorta.
The red arrows point to rib notching caused by the dilated intercostal arteries.
The yellow arrow points to the aortic knob, the blue arrow to the actual coarctation
and the green arrow to the post-stenotic dilation of the descending aorta.
Click here for same photo without labels.

l Clinical Findings–Infancy

o       Severe CHF most common from  2nd to 6th week of life

o       Weak or absent leg pulses

o       Lower BP in the legs than in the arms

o       EKG

§         RV hypertrophy because RV assumes most of the cardiac output during fetal life in these patients

l Clinical Findings-Children and Adults

o       Differential pulses in arms (bounding) and legs (weak)

o       EKG

§         LVH

l Echocardiographic Findings

o       2D echo can demonstrate coarcts from suprasternal notch

o       Echo is most helpful in excluding associated hypoplastic left heart syndrome

l Complications

o       Heart failure in neonate

o       Subarachnoid bleeds from ruptured Berry aneurysms

o       Dissection of aorta

o       Infective endocarditis

o       Mycotic aneurysm

Tuberculosis   Primary Pulmonary Tuberculosis Parenchyma §       Upper lobes affected slightly more than lower §       Alveolar infiltrate §       Cavitation is rare §       Lobar pneumonia is almost always associated with lymphadenopathy—therefore, lobar pneumonia associated with hilar or mediastinal adenopathy at any age should strongly suggest TB Lymph node §       Mostly unilateral hilar and/or paratracheal, usually  right sided, rarely bilateral §       Differentiates primary from postprimary TB—it does not occur in postprimary TB §       Much more common in children ·       Airway ·       Atelectasis classically affects the anterior segments of the upper lobes or the medial segment of the RML ·       Pleura §       Pleural effusion as a manifestation of primary TB occurs more often in adults than children §       With appropriate treatment, it carries the best prognosis of all patterns of TB and is the least likely to develop complications §       The fluid accumulates slowly and painlessly—therefore, patients with TB are seldom seen with a small amount of pleural fluid §       Parenchymal disease will almost never be present with a pleural effusion although lymphadenopathy may §       Apical pleural scarring is rarely tuberculous in origin Calcification in the primary complex is relatively rare. Very few patients with primary TB have clinical manifestations Postprimary Tuberculosis  (“Reactivation TB”) Most cases in adults occur as reactivation of a primary focus of infection acquired in childhood Limited mainly to the apical and posterior segments of the upper lobes and the superior segments of the lower lobe Caseous necrosis and the tubercle (accumulations of mononuclear macrophages, Langerhan's giant cells surrounded by lymphocytes and fibroblasts) are the pathologic hallmarks of postprimary TB Healing occurs with fibrosis and contraction; calcification is rarer than in    primary Patterns of distribution §       Almost always affect the apical or posterior segments of the upper lobes or the superior segments of the lower lobes—bilateral upper lobe disease is very common §       May present as pneumonia §       Cavitation may result: the cavity is usually thin-walled, smooth on the inner margin with no air-fluid level §       Transbronchial spread may occur—from one upper lobe to opposite lower or to another lobe §       Miliary spread (below) §       Bronchiectasis—usually asymptomatic §       Bronchostenosis due to fibrosis and stricture: fibrosis may cause distortion of a bronchus and atelectasis many years after the initial      infection—“middle lobe syndrome” §       Solitary pulmonary nodule—the tuberculoma—may occur in either primary or postprimary disease; round or oval lesions with small, discrete shadows in the immediate vicinity of the lesion—the “satellite” lesion Formation of a pleural effusion in postprimary TB almost always means direct spread of the disease into the pleural cavity and should be regarded as an empyema—this carries a graver prognosis than the pleural effusion of the primary form Direct extension into the ribs or sternoclavicular joints is uncommon Miliary Tuberculosis Older men, Blacks and pregnant women are sus ceptible Onset is insidious Fever, chills, night sweats are common Takes weeks between the time of dissemination and the radiographic appearance of disease Considered to be a manifestation of primary TB–although clinical appearance of miliary TB may not occur for many years after initial infection When first visible, they measure about 1 mm in size; they can grow to 2-3mm if left untreated When treated, clearing is rapid—miliary TB seldom, if ever, produces calcification TB and Other Diseases There is an association between TB and silicosis, TB and HIV There may be an association between TB and sarcoid There is no association between TB and bronchogenic carcinoma HIV and TB No matter what form of TB the patient has, it tends to look like 1° TB Hilar and mediastinal adenopathy are common Cavitation is less common There is no predilection for the apices MAI (mycobacterium avium-intracellulare) is more common in HIV than TB