What Is Swyer Syndrome?
Swyer-James Syndrome (SJS) (S-Jan syndrome), a 6-year-old child was first reported by Swyer and James in 1953. In 1954, Macleod reported 9 cases successively, so it was also called Swyer-James-Macleod syndrome or Macleod syndrome. Also known as unilateral transparent lung, unilateral translucent lung or unilateral acquired lobar emphysema.
Swyer-James syndrome
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- Swyer-James Syndrome (SJS) (S-Jan syndrome), a 6-year-old child was first reported by Swyer and James in 1953. In 1954, Macleod reported 9 cases successively, so it was also called Swyer-James-Macleod syndrome or Macleod syndrome. Also known as unilateral transparent lung, unilateral translucent lung or unilateral acquired lobar emphysema.
- X-ray features Increased lateral lung brightness, reduced pulmonary vascular texture, reduced or normal lung volume, and exhaled gas retention
- The etiology of this disease was originally thought to be related to congenital development, but it was soon rejected because its classification of the trachea and the number of branches of the pulmonary vessels were not abnormal. SJS is now considered to be essentially an occlusive bronchitis due to an infection. Severe pulmonary infections in infancy are the cause of the disease. Adenovirus, measles, mycoplasma, pertussis, tuberculosis, influenza virus, etc. are its pathogens.
- Obstructive bronchitis caused by air retention and hypoperfusion in the affected lobe is the cause of transparent lungs in SJS radiological manifestations
- SJS is more common in children and women. There are more left lungs than right ones. Generally, only unilateral or unilobed lungs are involved. Multilobed, multisegmented, and contralateral lungs are also affected. Other interstitial lesions may be involved. The possible reason is that the same infection in infancy leads to different results, such as SJS caused by occlusive bronchiolitis in this part and bronchiectasis and atelectasis in another part.
- The main clinical manifestations of SJS are repeated cough, expectoration, hemoptysis, wheezing, and dyspnea. Most of the symptoms are related to whether there is a combined expansion. Symptoms were significantly less in patients without combined branch expansion or columnar branch expansion than those with cystic branch expansion.
- A confirmed radiographic examination of SJS. Typical manifestations are: the lung has increased transparency and reduced texture (but does not disappear), and most of them are associated with reduced lung volume; exhaled gas retention; affected pulmonary arteries become smaller; can be combined with dilatation, atelectasis, cavities, Interstitial lesions such as subpleural infiltration.
- SJS bronchoscopy showed no abnormal airway structure and no obstruction in the airway. Ventilation and perfusion scans showed reduced ventilation on the affected side and significantly reduced perfusion (indicating smaller pulmonary arteries). Pulmonary angiogram showing small pulmonary artery trunks and branches
- Differential diagnosis:
- Congenital lobar emphysema develops within 6 months, and progressive dyspnea and cyanosis appear shortly after birth.The image shows abnormally increased transmittance of the lung field on the affected side, excessive expansion of the lung lobe, and shift of the mediastinal heart shadow to the healthy side. Position, mediastinal pulmonary hernia can be seen, the adjacent lung tissue is significantly compressed, and the affected side is slender and scattered.
- Congenital one-sided pulmonary dysplasia is similar to that of unilateral transparent lung, but the disease has no history of repeated pulmonary infections, lack of bronchial pulmonary inflammation changes and signs of alveolar gas retention, and angiography shows pulmonary artery dysplasia or associated cardiovascular malformations.
- Bronchial foreign body
- Treatment and prognosis:
- Prevent and control recurrent respiratory infections, use antibiotics to control infections; those who have obvious airway obstruction and sputum orally or nebulized tracheal dilator
- The prognosis of SJS is related to whether to merge and expand. The prognosis of patients with no branch expansion or column strong branch expansion is good. Without branch expansion, there is only a slight tendency for spontaneous improvement in respiratory symptoms.
- The indications for surgery are limited to localized branch expansion with uncontrolled hemoptysis; repeated hemoptysis; repeated pulmonary infections; other disabling symptoms.