|Year : 2021 | Volume
| Issue : 1 | Page : 41-44
Swyer-James-Macleod Syndrome: A Rare Finding in a Case of Haemoptysis
Neeraj Sharma1, Preeti Gupta2, Aseem Yadav3
1 Department of Respiratory Medicine, Command Hospital, Kolkata, West Bengal, India
2 Department of Radiology, Command Hospital, Kolkata, West Bengal, India
3 Department of Respiratory Medicine, MH, Namkum, Ranchi, Jharkhand, India
|Date of Submission||17-Apr-2020|
|Date of Decision||30-Jun-2020|
|Date of Acceptance||31-Jul-2020|
|Date of Web Publication||15-Feb-2021|
Dr. Neeraj Sharma
Pulmonologist, Department of Respiratory Medicine, Command Hospital Eastern Command, Near Visa house, Alipore, Kolkata, West Bengal 700027
Source of Support: None, Conflict of Interest: None
Swyer-James-MacLeod Syndrome (SJMS) is a rare cause of a hyperlucent unilateral hemithorax. It is usually found in childhood and is a long-term sequalae of postinfectious bronchiolitis obliterans. It can be an incidental finding in adults. Most patients are asymptomatic. However, some patients can present with recurrent pulmonary infections, haemoptysis or dyspnoea. The diagnosis of SJMS is made radiographically by chest x-ray and CT scan. Majority of symptomatic patients will require conservative management. It is also important to rule out other diseases which have similar radiological picture as inaccurate diagnoses can lead to inappropriate therapy. Here we report a case of 63-year-old woman who presented with haemoptysis and progressive dyspnoea and was subsequently diagnosed as a case of SJMS.
Keywords: Haemoptysis, hyperlucent hemithorax, Swyer-James-Macleod Syndrome
|How to cite this article:|
Sharma N, Gupta P, Yadav A. Swyer-James-Macleod Syndrome: A Rare Finding in a Case of Haemoptysis. J Assoc Chest Physicians 2021;9:41-4
| Introduction|| |
Swyer-James-MacLeod Syndrome (SJMS) is rare cause of a hyperlucent unilateral hemithorax. It is a long-term sequalae of postinfectious bronchiolitis obliterans that occurs during childhood. It can be an incidental finding in adults. It is characterized by pulmonary artery hypoplasia and/or agenesis which results in hypoperfusion of the pulmonary parenchyma. Radiographic or computed tomography (CT) imaging is required for the diagnosis. The characteristic radiological feature is a hyperlucent unilateral hemithorax with decreased bronchovascular markings. Most patients are asymptomatic. However, some patients can present with recurrent pulmonary infections, haemoptysis or dyspnoea. Here we report a case of 63-year-old woman who presented with haemoptysis and progressive dyspnoea and was subsequently diagnosed as a case of SJMS.
| Case presentation|| |
A 63-year-old woman presented to the emergency department with a one-month history of productive cough with intermittent haemoptysis and progressive dyspnoea on exertion. There was no h/o fever, anorexia and weight loss. She denied h/o smoking and biomass fuel exposure. Her past medical history revealed recurrent pulmonary infections in childhood. She was not on any medical follow up and treatment for same. On general examination she had pallor and was normotensive with pulse rate of 90 beats per minute. She had respiratory rate of 24 breaths per minute with an oxygen saturation of 90% while breathing room air. Respiratory examination revealed use of accessory muscles of respiration and reduced movement of left hemithorax. On chest auscultation there was diminished vesicular breath sounds over left hemithorax. On evaluation she had a haemoglobin of 8 g/dl with normal total leucocyte count. Serum electrolytes and other biochemical parameters were with in normal limits. Her sputum for AFB was negative. Her chest X-ray [Figure 1] showed hyperlucent left hemithorax. She was subjected to CT chest [Figure 2] which showed mosaic perfusion of right lung, hyperlucent left lung with bronchiectatic changes and hypoplastic left pulmonary artery. She also underwent CT pulmonary angiography [Figure 3] which showed hypoplastic left pulmonary artery and its branches with differential attenuation of both lungs, findings consistent with Swyer-James-McLeod syndrome. There was a mixed defect (mild obstruction and restriction) noted on spirometry. She was managed with oral antibiotics, nebulisation with bronchodilators and steroids and other supportive therapy. She responded well to the therapy and was later discharged on inhaled bronchodilators and steroids. She was advised and influenza and pneumococcal vaccinations.
| Discussion|| |
SJMS is considered a fairly rare condition characterized by unilateral hyperlucency of some or all of the lung. It was first described in the year 1953 by Swyer and James. It is considered to be a disease which is acquired secondary to viral bronchiolitis and pneumonitis in childhood. The causative organisms associated with it are Paramyxovirus morbillivirus, Bordetella pertussis, Mycobacterium tuberculosis, Mycoplasma pneumoniae, Influenza A and Adenovirus types 3, 7 and 21. Regardless of this association, it is fairly uncommon occurring in around 3.8%–4.3% of patients with bronchiolitis obliterans.The infectious agent causes an inflammatory response, which leads to obliteration of peripheral airways in the infected lung. In most cases, both the bronchioles and small bronchi are affected. There is submucosal fibrosis of the airways leading to luminal occlusion and irregularity. This inflammation also results in obliteration of the vascularity of the lung and impeded lung development. In some cases, the lung distal to diseased bronchioles becomes hyperinflated with pan-acinar emphysematous changes.
Most patients are clinically asymptomatic. Symptomatic patients typically have persistent cough, exertional dyspnoea and occasionally haemoptysis. Some patients will give history of repeated pulmonary infections in childhood. The patients who have little or no associated sequelae bronchiectasis will be asymptomatic or have minimal symptoms.
The diagnosis of SJMS is made radiographically by chest x-ray and CT scan. The characteristic radiographic findings include a unilateral hyperlucent lung along with decreased broncho-vascular markings, slight displacement of the mediastinum to the affected side and a small hilar shadow. The hyperlucency can sometimes be limited to one lobe rather than entire lung. In detecting hyperlucent lung regions, CT chest is more sensitive than chest radiograph, and is also superior in determining the severity and distribution of the disease. CT scan findings may also include bronchiolectasis, bronchiectasis, scarring and atelectasis. Around 30% of patients will have bronchiectatic changes on CT chest and these patients usually have more severe exacerbations as compared to those who do not have bronchiectasis. The presence of bronchiectasis also explains the obstructive-restrictive pattern (mixed pattern) typically seen on spirometry. Pulmonary angiography is not an important investigation for the diagnosis of SJMS although it can show hypoplasia and diminished size of the affected pulmonary artery.
Treatment of SMJS is usually individualized depending on the symptoms of the patient. It ranges from conservative management to surgical intervention. Majority of symptomatic patients will require conservative management which includes chest physiotherapy, inhaled bronchodilators and low-dose inhaled corticosteroids. Patients should also be given pneumococcal and Influenza vaccinations. Long-term oxygen therapy may be appropriate in cases with severe disease and respiratory failure. Surgical intervention is usually considered for patients who have severe disease, repeated pulmonary infections and are not responding to optimal medical management. The surgical procedures carried out are pneumectomy, lobectomy or segmentectomy depending on extent of disease and the other co-morbidities of the patient. Prognosis is generally dependent on the presence or absence of bronchiectasis.
There are several important differential diagnoses that should be kept in mind when assessing any individual with unilateral pulmonary hyperlucency. It includes pneumothorax, congenital lobar emphysema, pulmonary artery hypoplasia and asymmetric emphysema. Another rare cause of a unilateral hyperlucent hemithorax is Poland syndrome. It is a congenital anomaly characterised by unilateral absence of the pectoralis major and minor muscles, hypoplasia of the breast and nipple and scarcity of subcutaneous tissue.
| Conclusion|| |
Swyer-James-Macleod syndrome is a rare disease that should be considered in individuals with recurrent pulmonary infections, dyspnoea and haemoptysis. The diagnosis is made radiographically by chest x-ray and CT scan which shows unilateral hyperlucent hemithorax with decreased bronchovascular markings. Majority of symptomatic patients will require conservative management. It is also important to rule out other diseases which have similar radiological picture as inaccurate diagnoses can lead to inappropriate therapy.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]