Swyer-James syndrome is a rare pulmonary disease characterized by the presence of a unilateral hyperlucent lung [1]. The disorder is the manifestation of postinfectious obliterative bronchiolitis of the lung or a part of the lung [2]. Regarding the etiology, Swyer-James syndrome is thought to be acquired following infections that occurred during infancy or childhood [3]. The most common pathogens include adenoviruses types 3, 7, and 21, while others such as paramyxovirus, Bordetella pertussis, Mycobacterium tuberculosis, Mycoplasma pneumoniae, Streptococcus pneumoniae, and influenza virus have also been implicated [4] [5].

The majority of patients are asymptomatic while some report a productive cough, hemoptysis, dyspnea on exertion, wheezing, and reduced tolerance to exercise [1] [3] [6]. Children may present with asthma-like features that do not respond to typical asthma therapy [1] [7]. Asymptomatic individuals are likely to be diagnosed in adulthood [7].

Acute cases can result in complications such as respiratory distress and atelectasis while long-term consequences may include chronic and recurrent episodes of pulmonary infections such as bronchiolitis and pneumonitis [6] [7] [8]. Additionally, patients are at risk for developing sequelae such as bronchiectasis, which has prognostic implications [1] [9]. Specifically, saccular bronchiectases are associated with more severe manifestations [9]. Other complications of Swyer-James syndrome include lung abscesses and spontaneous pneumothorax [10].

On lung auscultation, the patient may exhibit diminished breath sounds on the involved side. Vital signs and the remainder of the exam may be unremarkable [1].

• Pulmonary Disorder

  OBJECTIVE: The aim of this study was to describe the anesthetic management and perioperative outcomes in patients with Swyer-James syndrome (SJS), a rare acquired pulmonary disorder caused by postinfectious bronchiolitis obliterans resulting in airway [ncbi.nlm.nih.gov]

  Although rare, aspergillosis can occur in immunocompetent adults with a pre-existing lung disease other than chronic obstructive pulmonary disorder. [jmedicalcasereports.biomedcentral.com]

  4,9 The pulmonary changes associated with this disorder could pose challenges for anesthetic management. [kundoc.com]

  Swyer–James–MacLeod syndrome, or unilateral hyperlucent lung syndrome, is a rare pulmonary disorder. [jpeds.com]

Patients with the above-described clinical presentation warrant a thorough workup. Diagnosis is achieved through a detailed history (which mainly confirms frequent childhood lung infections), physical exam, and the appropriate studies. Of importance, Swyer-James syndrome should be considered in individuals with an asthma-like presentation who lack a response to standard treatment [1].

Chest radiography will characteristically reveal unilateral hyperlucency of the lung or portion of the lung resulting from alveolar overdistension and decreased arterial flow [11] [12] [13]. Other features on this modality include hypovascularity, a mediastinal shift towards the affected lung, a small hilar shadow, and air trapping with expiration [9] [11].

Computed tomography (CT) scanning demonstrates greater sensitivity in depicting hyperlucency and the severity of the disease [11]. This study also detects diminished vascularity, a smaller lung, and other pathologies such as bronchiectasis, atelectasis, scarring, etc [3] [9]. Additionally, the bronchi of the affected lung reflect a pruned appearance while an air trapping, associated with an expiration, is also visible.

Magnetic resonance angiography (MRA) in these patients will display an underdeveloped pulmonary vasculature. Moreover, quantitative ventilation/perfusion lung scan reveals a decrease in both variables in the affected lung [14].

Finally, although it has limitations, pulmonary angiography demonstrates hypoplasia of the affected pulmonary artery [1].

This report shows that although rarely indicated, surgical treatment can improve quality of life and pulmonary function in SJMS. [jpedsurg.org]

Treatment and prognosis Treatment is conservative and preventative, focused primarily on controlling pulmonary infections. Inhaled corticosteroids may have a limited role in treatment 9. [radiopaedia.org]

Currently there are no established treatment recommendations for Swyer-James syndrome to guide care. However treatment is usually described as "conservative." Also, regular monitoring and prompt treatment of infection is important. [rareguru.com]

The cornerstone of treatment is conservative management. [spandidos-publications.com]

TREATMENT AND PROGNOSIS The prognosis is generally good. Most patients have an asymptotic course unless the opposite lung becomes diseased. The treatment is generally symptomatic. [wits.ac.za]

Physicians should be aware of this condition and its frequent association with bronchiectasis, to manage patients appropriately, prevent recurrent pulmonary infections, and achieve a better prognosis. [journals.lww.com]

Treatment and prognosis Treatment is conservative and preventative, focused primarily on controlling pulmonary infections. Inhaled corticosteroids may have a limited role in treatment 9. [radiopaedia.org]

The clinical manifestation and prognosis is affected by the presence and type of bronchiectasis, with worst prognosis associated with saccular form 6. [onlinelibrary.wiley.com]

Etiology It is postulated that the infectious acute bronchiolitis progresses to fibrous obliteration of the airway lumen, namely damaging the terminal and respiratory bronchioles, preventing normal development of their alveolar buds. [vcuthoracicimaging.vcu.edu]

This case outlines the need for emergency and trauma physicians to be cognizant of other etiologies of abnormal chest X-ray studies and to follow appropriate clinical pathways when working-up patients for chest trauma. [ncbi.nlm.nih.gov]

Swyer-James-Macleod syndrome (SJMS) is a rare etiology of a unilateral hyperlucent hemithorax but an important one, which should be considered in any individual with such findings. [experts.umn.edu]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Deterrence and Patient Education Enhancing Healthcare [pubmed.ncbi.nlm.nih.gov]

Among more than 40 different respiratory diseases the highest global epidemiological bur den is associated with: upper and lower respiratory infections, asthma, chronic obstructive pulmonary disease, lung cancer and tuberculosis. [dlib.si]

Using resources from the Rochester Epidemiology Project and the Mayo Clinic Study of Aging and in collaboration with the Mayo Clinic Department of Neurology, they are examining the role of anesthetic exposure on subsequent development of mild cognitive [mayo.edu]

BO affects mainly male children during the first year of life.1-3Despite the lack of available epidemiology, there is a high prevalence in countries within the southern hemisphere, such as Argentina, Brazil, Chile and New Zealand.4,5There are several [scielo.br]

Multimodal imaging was helpful for noninvasively diagnosing and understanding the pathophysiology. Figure 1. (A) Chest radiograph showed unilateral hyperlucency in the left lung. [atsjournals.org]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Deterrence and Patient Education Enhancing Healthcare [pubmed.ncbi.nlm.nih.gov]

Pathophysiology and surgical management.Ann Thorac Surg. 1974; 18: 172-185 Morita K. Shimizu J. Kamesui T. et al. A case of surgical treatment of Swyer-James syndrome.Nippon Kyobu Geka Gakkai Zasshi. 1994; 42: 1949-1952 Koyama T. Osada H. [jpedsurg.org]

Etiology/Pathophysiology: Felt to be due to adenovirus infections that are severe. Pathology: Necrotizing bronchiolitis with obliteration of terminal bronchioles and maintenance of peripheral alveoli. [virtualpediatrichospital.org]

The patient started inhaled corticosteroids and long acting beta agonist, and vaccines to prevent pulmonary infections were administered. [journals.lww.com]

Investigation of these cases with chest CT may prevent misdiagnosis or a delay in diagnosis, especially in patients who do not respond to conventional therapy, in order to achieve better prognosis and to prevent inappropriate management. [cureus.com]

Almost all the reported cases of SJMS were managed by conservative therapy, comprising prevention and treatment of recurrent pulmonary infections. [casereports.bmj.com]

Prevention and prompt treatment of recurrent respiratory infections: Primary prevention such as pneumococcal and influenza vaccination is necessary.[10] Mucolytics may be helpful in patients with bronchiectasis. [ncbi.nlm.nih.gov]

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14. Arslan N, Ilgan S, Ozkan M, et al. Utility of ventilation and perfusion scan in the diagnosis of young military recruits with an incidental finding of hyperlucent lung. Nucl Med Commun. 2001;22(5):525-530.