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]

• Nasal Flaring

  The initial physical examination revealed tachypnea (respiratory rate, 40/min) with intercostal and subcostal retractions, nasal flaring, and a lowered wishbone. [scielo.br]

• Sialorrhea

  Possible reasons for the increased predisposition to infections include agranulocytosis and impaired swallowing along with sialorrhea leading to aspiration pneumonia. [ijpm.info]

• Primary Amenorrhea

  Diagnosis typically occurs in adolescence due to absence of primary amenorrhea, at which point patients usually begin hormone replacement therapy to induce menstruation and the development of secondary sex characteristics. [visualdx.com]

• Amenorrhea

  Diagnosis typically occurs in adolescence due to absence of primary amenorrhea, at which point patients usually begin hormone replacement therapy to induce menstruation and the development of secondary sex characteristics. [visualdx.com]

• Burning Micturition

  Case 2 An Australian Indigenous 40‐year‐old male presented acutely to the emergency department with cough, fever, and burning micturition. [onlinelibrary.wiley.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].

• Pulmonary Infiltrate

  Right-sided pulmonary infiltrates c. Left-sided pneumothorax d. Right-sided pneumothorax e. Elevated hemidiaphragm on the right Figure 1 Chest radiograph upon presentation to the emergency department. Answer 1 c. [breathe.ersjournals.com]

  Diagnostic criteria for allergic bronchopulmonary aspergillosis include the presence of bronchial asthma, immediate skin test reactivity to Aspergillus fumigatus, elevated serum immunoglobulin E levels, pulmonary infiltrates, central bronchiectasis, peripheral [pubs.rsna.org]

• Left Pleural Effusion

  An 11-year-old girl contracted pneumonia with consolidation of the left lower lobe, infiltrates in the lingula and left upper lobe, and a large left pleural effusion, accompanied by a Mycoplasma complement-fixation titer increasing to 1:16,384. [ncbi.nlm.nih.gov]

However, our patient responded well to the treatment with clozapine without significant worsening of recurrent infections. [ijpm.info]

She is under supportive treatment and her respiratory symptoms have been improved. [ncbi.nlm.nih.gov]

Treatment is most often prolonged or combined [ 9, 10 ]. The choice of the anti-fungal was based on the normal immune status and non-severity of the infection, which allows for oral treatment. [jmedicalcasereports.biomedcentral.com]

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 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]

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]

With current pharmaceutical developments, the prognosis is good for sufferers of the illness to lead normal and healthy lives. [checkorphan.org]

With current pharmaceutical developments, the prognosis is good for sufferers of the illness to lead normal and healthy lives. References[edit] ^ Macleod, W. M. (1954). "Abnormal transradiancy of one lung" (PDF). [en.wikipedia.org]

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]

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.com]

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]

Regarding the etiology, Swyer-James syndrome is thought to be acquired following infections that occurred during infancy or childhood. [symptoma.com]

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]

Prospective epidemiological evaluation of seasonal influenza in all elementary schoolchildren in Matsumoto City, Japan, in 2014/2015. Jpn J Infect Dis,in press 2016(Aug.) [soar-rd.shinshu-u.ac.jp]

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]

Multimodal imaging was helpful for noninvasively diagnosing and understanding the pathophysiology. 1. Thieme SF, Johnson TR, Lee C, McWilliams J, Becker CR, Reiser MF, et al. [atsjournals.org]

Pertinent findings will be highlighted, and the relevant pathophysiology discussed; see example content as part of the attached PowerPoint. [cf.arrs.org]

University of Zagreb School of Medicine Resident - Anesthesiology General Hospital, Split, Croatia MSc University of Rijeka School of Medicine Internship Department of Medicine, Medical Military Academy MSc - Postgraduate Study of Clinical Pathophysiology [mayo.edu]

To prevent the recurrence of pneumothorax related to Swyer-James syndrome, some kind of procedure, such as extensive pleurodesis, is necessary. [ncbi.nlm.nih.gov]

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

This early insult results in damage to the terminal and respiratory bronchioles, preventing normal development of alveolar buds. Radiographic manifestations of SJS include pulmonary hyperlucency and pruning of vessels. [roentgenrayreader.blogspot.com]

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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.