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].

• Short Stature

  He had the typical craniofacial appearance and short stature of Noonan syndrome; he had mild mental retardation, atrophic testis, mild funnel chest and kyphosis. cardiovascular abnormalities included asymmetric hypertrophic cardiomyopathy and a significantly [ncbi.nlm.nih.gov]

• 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]

  Identified reports of patients undergoing surgery were reviewed to identify perioperative complications. wyer-James syndrome (SJS) is a rare acquired pulmonary disorder that develops secondary to infectious etiologies in early childhood.1 The condition [kundoc.com]

• Hyperresonance

  The physical examination may reveal hyperresonance over the affected area, restricted chest expansion on one side, or occasionally rales on auscultation. [ncbi.nlm.nih.gov]

  Recurrent lung infections and bronchiectasis are also present in a large number of patients, which may adversely affect prognosis.[4] Physical examination may reveal decreased chest expansion and breath sounds, hyperresonance, crackles, or wheeze.[5] [journals.lww.com]

• Fear

  Over a span of few months, she would remain fearful most of the day. On being asked, she reported camera being fitted in her room to spy on her. She also reported hearing voices of her neighbor abusing her and making plans to harm her. [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]

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].

• 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]

Bronchodilators and steroid treatment had no effect. [ncbi.nlm.nih.gov]

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]

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]

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

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]

Although neurodevelopmental model of schizophrenia has long been accepted, recently infectious (especially viral) etiology has gained popularity. [ijpm.info]

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]

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]

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]

These pathophysiologic changes lead to air trapping and hypoperfusion in the affected segment, thus creating radiographic hyperlucent or translucent findings [4]. Clinically, most patients are asymptomatic. [hindawi.com]

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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7. Mori J, Kaneda D, Fujiki A, Isoda K, Kotani T, Ushijima Y. Swyer-James Syndrome in a 7-Year-Old Female. Pediatr Rep. 2016;8(3):6643.
8. Chang AB, Masel JP, Masters B. Post‐infectious bronchiolitis obliterans: clinical, radiological and pulmonary function sequelae. Pediatr Radiol. 1998;28(1):23–29.
9. Singal KK, Bhatti KP, Gupta A, et al. Swyer-James-MacLeod syndrome: a rare case. Bangladesh J Med Sci. 2015;14(4):402.
10. Soni R, Branes D. Macleod's syndrome presenting with spontaneous pneumothorax. Respirology. 1999; 4(3):275-277.
11. Moore AD, Godwin JD, Dietrich PA, et al. Swyer-James syndrome: CT findings in eight patients. AJR Am J Roentgenol. 1992;158 (6):1211-1215.
12. Khalil KF, Saeed W. Swyer-James-MacLeod Syndrome. J Coll Physicians Surg Pak. 2008;18(3):190-192.
13. Gopinath A, Strigun D, Banyopadhyay T. Swyer-James syndrome. Conn Med. 2005;69(6):325-327.

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.