Disorders of Chest wall Hesham Atef Abdel Halim
Disorders of Chest wall Hesham Atef Abdel. Halim, MD Professor of Pulmonary Medicine Faculty of Medicine, Ain Shams University
Thoracic Cage The chest wall, an integral part of the respiratory pump, consists of the rib cage, rib cage muscles, diaphragm, and abdomen. Like the respiratory muscles, the nonmuscular structures of the chest wall (i. e. , thoracic spine, ribs) are also essential for normal respiratory function.
Rib abnormalities Congenital abnormalities of the chest wall Bifid ribs are common, particularly in the upper six ribs, and if not appreciated on the chest radiograph may give rise to confusion in interpretation. Fused and absent ribs may also occur but are only of radiological interest.
Rib abnormalities Congenital abnormalities of the chest wall Bifid ribs are common, particularly in the upper six ribs, and if not appreciated on the chest radiograph may give rise to confusion in interpretation. Fused and absent ribs may also occur but are only of radiological interest.
Cervical ribs: Occur in 0. 5% of the population, Usually arising from the seventh cervical vertebra; They may vary greatly in size and shape, and are bilateral in 80% of cases Usually asymptomatic. May give rise to the thoracic outlet syndrome due to compression of subclavian vessels or nerves in the root of the neck. Symptoms due to compression are more prevalent in Females and more common on the left. The production of symptoms bears no relation to the size of the rib; a small cervical rib with a fibrous attachment may cause severe symptoms whereas a large cervical rib may be asymptomatic.
Rib notching Erosion of the inferior border of ribs or rib notching is most seen in patients with coarctation of the aorta, when it is due to the enlarged intercostal arteries acting as collateral vessels. It is present in 75% of adult patients. The first two and last three ribs are never involved.
Pectus carinatum or pigeon chest In this condition the sternum is prominent, forming an anterior ridge like the keel of a ship with the ribs falling away steeply on either side, sometimes with a vertical groove on each side of the sternum. It may be associated with other congenital anomalies, especially cardiac lesions and coarctation of the aorta. Aetiology Pectus carinatum may result from obliteration of all the sternal sutures at an early age, possibly due to inadequate segmentation during fetal life, with a resulting synostosis. An alternative explanation, which has been confirmed at postmortem, is that the deformity results from malattachment of the anterior portion of the diaphragm to the posterior portion of the rectus sheath rather than to the xiphoid process, with consequent distorting mechanical effects.
Management There is no associated functional defect and correction is not normally indicated. However, corrective procedures for both types of pectus carinatum have been described and may be required for cosmetic reasons. Such patients are almost invariably happier with the large scar than with the deformity, though this effect of the operation should be discussed with them before referral to a thoracic surgeon.
Pectus excavatum or funnel chest In this condition the manubrium is normal, but the body of the sternum is angled backwards towards the spine from the manubriosternal joint downwards with maximum recession at the xiphoid, below which the costal margin bends forwards. The condition is usually symmetrical but occasionally the sternal recession may be greater on one side than the other. As with pectus carinatum, there may be ass ociated congenital anomalies and Marfan’s syndrome may be present.
Aetiology Pectus excavatum probably results from an imbalance of forces counteracting the inward pull of the diaphragm on the xiphisternum. Biopsies have shown that the anterior portion of the diaphragm is replaced by fibrous tissue. In the normal person the muscle in this region contracts on inspiration and its attachments to the lower chest wall on either side of the xiphisternum and lower sternum prevent these being dragged backwards as the diaphragm descends. When this muscle is lacking, the unopposed action of the posterior diaphragmatic muscle displaces the sternum and xiphisternum backwards as the diaphragm descends. This movement occurs in affected newborn infants only on inspiration but after the age of 3 months the deformity progresses, with resulting secondary changes in the sternum and costal cartilages.
It has been suggested that an additional factor is a congenital laxity of the pericardium that allows the heart to fall to the left, removing its support from the sternum. and it has been recommended therefore that at operation the pericardial sac should be sutured into a central position. It has also been suggested that upper airway obstruction, whether due to enlarged tonsils and adenoids or segmental bronchomalacia, appears to predispose to pectus excavatum, which may resolve if the obstruction is treated early enough. An association between pulmonary sequestration and pectus excavatum has been described.
Pulmonary function • Minor decreases in VC, TLC and MVV. • Normal or increased RV. • Normal FEV 1 • Even in the presence of normal pulmonary mechanics and lung volumes, it may be possible to demonstrate abnormal findings during exercise in symptomatic patients.
Radiology • On the posteroanterior chest film the heart is often displaced to the left and anterior ribs may show marked obliquity. • The depressed sternum is obvious on the lateral chest film. • Paradoxical cardiac enlargement during inspiration in children with pectus excavatum has been reported and is due to reduction of the anteroposterior diameter of the thorax caused by diaphragmatic descent.
Treatment Surgery is rarely required because of cardiorespiratory symptoms, More often, surgical repair is sought for cosmetic reasons. Early cosmetic and functional results are usually good, with a satisfactory long-term result. Unsatisfactory results have been recorded in patients with severe and asymmetrical deformities and with Marfan’s syndrome.
Fractures Acquired abnormalities of the chest wall • Traumatic fractures. • Fatigue fractures. • Cough fractures. • Pathological fractures.
Radiology Unless there is displacement of a fracture it may not be visible on the radiograph even in the acute stage, and radiologically visible callus formation does not occur for several weeks. The diagnosis is therefore frequently clinical. Pneumothorax and/or subcutaneous emphysema may be seen with traumatic lesions, while the primary lesion may be visible in the case of pathological fractures. Sometimes the film may show linear shadows 2– 3 cm long underlying a traumatically fractured rib, possibly due to bruising of the lung.
Treatment with adequate analgesia is usually all that is required, although occasionally it may be necessary to infiltrate with local anaesthesia. Treatment Lower respiratory tract infection, particularly on the affected side, may develop in patients with chronic airways obstruction and require intervention with antibiotics. Chest wall strapping is contraindicated since it predisposes to the same complication.
FLAIL CHEST Flail chest can occur in up to 25 % of adults who have blunt chest wall trauma. It is a condition in which fractures of the ribs produce a segment of the rib cage that deforms markedly during breathing. Pulmonary complications such as pulmonary contusion, hemothorax, and pneumothorax can occur in up to 60 % of patients with flail chest. Thus, the mortality from flail chest may be high. Symptoms consist of chest tightness, chest pain, dyspnea, and limitation of ability to exercise. The disordered movement of the flail segment is related to changes in pleural pressure during the breathing cycle unopposed subatmospheric intrapleural pressure causes the flail segment to move inward during inspiration. During expiration, pleural pressure becomes more positive and the flail segment moves outward. This paradoxical motion of the flail segment is amplified by anything that further lowers pleural pressure, such as pulmonary contusion, which reduces lung compliance or an increase in airway secretions, which increases airways resistance. Flail chest may severely reduce VC and FRC to as much as 50 % of predicted.
Treatment The mainstay is pain control to reduce splinting, improve tidal volume, and minimizes areas of atelectasis. It can be accomplished by use of oral or intravenous narcotics, intercostal nerve blocks, or epidural anesthesia. Supplemental oxygen, improving tracheal bronchial toilet are used. Mechanical ventilation with positive pressure breathing has been shown to stabilize the flail segment by eliminating subatmospheric changes of pleural pressure during inspiration. However, complications of mechanical ventilation often supervened and increased morbidity and mortality. Consequently, mechanical ventilation is no longer recommended as a primary means of stabilizing the chest wall; instead, it is recommended when there is respiratory failure , or other indications. Positive pressure ventilation delivered by noninvasive techniques may provide an alternative means by preventing subatmospheric changes in pleural pressure during inspiration. Noninvasive ventilation to selected patients who are breathing spontaneously in conjunction with regional anesthesia can improve gas exchange and enable physiotherapy and early patient mobilization.
KYPHOSCOL IOSIS Kyphoscoliosis refers to a group of spinal disorders characterized by curvature of the spine in the lateral direction (scoliosis), sagittal plane (kyphosis) as well as rotation of the spine itself.
Kyphoscoliosis may be: Congenital. Secondary to other disorders. or Idiopathic. Diagnosis and Etiology
Aetiological classification of scoliosis Structural Non-structural
Structural Idiopathic Adolescent kyphosis Osteopathic Congenital scoliosis Klippel–Feil syndrome Spondylolisthesis Myopathic Duchenne muscular dystrophy Faciohumeroscapula r dystrophy • Neuropathic • • • Poliomyelitis Cerebral palsy Syringomyelia Neurofibromatosis Friedreich’s ataxia • Hereditary syndromes • Dominant • Marfan’s syndrome • Ehlers–Danlos syndrome • Recessive • Homocystinuria • Morquio’s syndrome • Sex-linked • Turner’s syndrome • Miscellaneous • Long-standing unilateral lung fibrosis • Burns • Irradiation • Thecoperitone al shunts • Empyema • Thoracoplasty • Hiatus hernia
Non-structural Postural compensatory Sciatic Inflammatory Hysterical
• Most cases of scoliosis nowadays are idiopathic in origin. • Idiopathic scoliosis may be seen at all ages during growth and at all spinal sites. • Thoracic scoliosis to the right is the most common presentation. • The condition is more prevalent in girls. • The higher in the spine the structural curve, the worse the prognosis.
• Prognosis is also a function of the angle of curvature, • Lines are drawn parallel to the upper border of the upper vertebral body and to the lower border of the lowest vertebra of the structural curve. • Perpendiculars are then erected from these lines to cross each other, • The angle between the perpendiculars being the angle of curvature or Cobb’s angle.
Physiology Lung volumes are reduced, with a restrictive pattern even in asymptomatic adolescents with scoliosis. VC, TLC, MVV, FRC and PEF are all reduced. There is a direct relationship between the angle of curvature and the degree of reduction of these indices. The higher the curve in the dorsal spine, the more severe its effect on function. RV may be maintained, resulting in an increased RV/TLC ratio. The FEV 1/FVC ratio is normal unless obstructive disease such as asthma supervenes. Dlco is reduced in proportion to the reduction in lung volumes, giving normal values for Kco.
Lung compliance in scoliosis is commonly low. impaired cardiorespiratory response to exercise, Maximal oxygen uptake is reduced. Regional perfusion and ventilation are often normal. Pa. O 2 is diminished even in asymptomatic adolescents with scoliosis, and with severe disease carbon dioxide retention occurs. As a consequence of increasing hypoxia, pulmonary artery hypertension occurs, the pressure being inversely proportional to Pao 2 In patients with severe scoliosis (with an angle ≥ 100°), severe desaturation may occur during sleep with episodes of central or obstructive apnoea and hypopnoea, particularly during REM sleep. Diminution in the maximum inspiratory and maximum transdiaphragmatic pressures.
Treatment Surgery Although surgical intervention, for example by spinal fusion, may ameliorate the angle of curvature and halt the progression of scoliosis, remarkably little benefit has been documented in terms of pulmonary function tests apart from an improvement in submaximal exercise ventilation.
Ankylosing spondylitis A form of spinal arthritis, chiefly affecting young males, that eventually causes ankylosis of vertebral and sacroiliac joints. The aetiology of ankylosing spondylitis is unknown but the strong association with HLA-B 27 is now well recognized. The disease is much commoner in young adult males than in females.
Pathophysiology • Fixation of the thoracic cage occurs in ankylosing spondylitis as a result of ankylosis of costotransverse and costovertebral joints. Sternomanubrial and sternoclavicular Joints may also be affected.
In severe disease a restrictive pattern of abnormality is seen, with a reduction in VC due to reduced inspiratory capacity and a reduction in TLC. RV and FRC are normal or slightly increased. Gas exchange is normal. Thoracic and total respiratory compliance are decreased but lung compliance is normal. Normal perfusion but reduced apical ventilation in patients, although later studies suggested that apical underventilation is only seen in the presence of fibrosis.
Clinical features Patients typically complain of low back pain and stiffness beginning in late adolescence or early adulthood. Onset of the disease after the age of 45 is rare. Symptoms are worse in the morning or after rest. Chest pain due to inflammation of manubriosternal junction and/or the sternoclavicular joints and inability to fully expand the chest on inspiration are infrequent complaints. Physical examination There may be tenderness of the anterior chest wall, or over the costochondral region or the manubriosternal junction. Exercise intolerance and dyspnea are uncommon.
Treatment Medical treatment to relief symptoms (e. g. analgesic for chest pain) Physiotherapy is regarded as an essential element of the overall management in AS. In occasional patients with severe disease, surgical attempts have been made to mobilize the chest by resection of the posterior parts of several ribs or by other procedures, some of which appear to have improved respiratory function. The recent introduction of antagonists of tumor necrosis factor (TNF) shown remarkable improvements in all aspects of the disease, including rib cage expansion and quality of life.
Osteomyelitis of ribs Infections of the chest wall Relatively uncommon and is more frequent in children than adults. It often occurs after trauma and affects one of the upper three ribs, most commonly the first. Local osteomyelitis of a rib may be secondary to the drainage of an empyema, with infection spreading from the drainage site into the bone. The most common Staphylococcus aureus. offending Microorganism is Management with appropriate antibiotics leading to a resolution in most cases.
Osteomyelitis of the sternum is very uncommon. It presents with fever and a tender swelling. Radiographic evidence of bony erosion and sclerosis. Staph. aureus and Pseudomonas aeruginosa have been reported as causative organisms. Surgical drainage and curettage may be required in addition to antibiotic therapy.
Septic arthritis Sternoclavicular joint infection is an unusual cause of chest pain. The joint is usually swollen and tender, with fever and systemic evidence of infection. The condition is commoner in heroin users in whom infection with both Ps. aeruginosa and Staph. aureus has been reported. Treatment is with antibiotics and aspiration as required.
Tuberculosis of the thoracic cage Costal tuberculosis is rare but may give rise to local pain, swelling and sinus formation. Radiologically there is an initial a small area of bone destruction that may progress to periosteal elevation and soft tissue swelling. There is usually no evidence of pulmonary tuberculosis. Similar disease clavicular joints. may arise in sterno-clavicular or acromio- Multiple lesions of the costovertebral portion of the ribs may accompany Pott’s disease of the spine with paravertebral abscess formation.
Cold abscess of the chest wall, which originates in tuberculosis of the intercostal lymph glands, can be divided into two main groups: The first lies at the angle of the ribs, from which the pus may track either backwards with the posterior primary division of the intercostal nerve, presenting near the erector spinae muscles, or forwards with the anterior division to present in the lateral chest wall. The second may arise from nodes in the region of the internal mammary artery and present near the costal cartilages. Cold abscess usually presents as a painless fluctuant swelling that may be mistaken for a lipoma. Diagnosis is made by aspiration of pus or during surgical exploration. It responds well to conventional antituberculous chemotherapy.
Syphilitic gumma of chest wall. Arising in the anterior mediastinum and giving rise to a hard, fixed lump in the intercostal space that may later soften and form a punched-out ulcer. Radiologically, the lesion may resemble a tumor of the anterior mediastinum. The true nature may be suspected if a punched-out ulcer has developed or as a result of positive serology. Actinomycosis. may spread from the lungs into the chest wall and give rise to destructive lesions with irregularity of the rib margins caused by periosteitis. Echinococcosis. Costal echinococcosis is very rare and usually presents as a multiloculated osteolytic lesion in rib. Fungal infections Blastomycosis, coccidioidomycosis and cryptococcosis can cause osteolytic lesions of ribs similar to those described for tuberculosis.
THORACOPLASTY Thoracoplasty consists of different combinations of rib removal, rib fractures, phrenic nerve resection, or compression of underlying lung by filling the pleural space with foreign material. These individuals commonly developed dyspnea, severe restrictive dysfunction, and chronic respiratory failure as they aged. The severity of the restrictive pattern was related to several factors including: The number of ribs removed, the presence of fibrothorax, progressive lung fibrosis due to underlying granulomatous disease, previous lung resection, or phrenic nerve damage. Often, surgery on the rib cage was followed by progressive scoliosis with aging and further deterioration of respiratory function. The severity of restriction and stiffening of the chest wall was like that seen with kyphoscoliosis leading to an increase in the oxygen cost of breathing, limited exercise tolerance, and impairment in gas exchange
Neuromuscular conditions affecting respiration
Levels of Respiratory System Dysfunction Induced by Neuromuscular Diseases and Conditions Level Disease or Condition Upper motor neuron Cerebral Vascular accidents Cerebellar atrophy Spinal cord Trauma Tumor Syringomyelia Multiple sclerosis Lower motor neuron Anterior horn cells Poliomyelitis Spinal muscle atrophy Amyotrophic lateral sclerosis Motor nerves Cardiac surgery Charcot-Marie-Tooth disease Diabetes Polyneuropathy Toxins Guillain-Barr ´e syndrome Neuralgia amyotrophy Critical illness polyneuropathy
Neuromuscular Muscle Myasthenia gravis junction Eaton-Lambert syndrome Botulism Organophosphate poisoning Drugs Dystrophy Acid maltase deficiency Malnutrition Corticosteroids Polymyositis
Principles of Management Principles in the management of respiratory dysfunction in patients with neuromuscular disease include: 1. Preventive therapies designed to minimize the impact of impaired secretion clearance and alveolar hypoventilation on gas exchange and lower respiratory tract infections. 2. Stabilization of patients who develop acute or chronic respiratory failure. Preventive Therapies : Intermittent Positive Pressure Breathing Respiratory Muscle Training Mechanical Ventilation.
Tumours of the chest wall Benign Malignant Soft tissue Lipoma Haemangioma Cystic hygroma Neurofibroma Bony cage Osteochondroma Chondroma Simple cyst Osteoid osteoma Fibrous dysplasia Fibrosarcoma Liposarcoma Leiomyosarcoma Synovioma Neuroectodermal tumours Chondrosarcoma Ewing’s tumour Fibrosarcoma Osteogenic sarcoma Myeloma
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