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]

• 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]

• 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]

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]

1. Dirweesh A, Alvarez C, Khan M, Shah N. A unilateral hyperlucent lung - Swyer-James syndrome: A case report and literature review. Respir Med Case Rep. 2017;20:104-106.
2. Abba AA, Al-Mobeireek AF. Clinical spectrum of Swyer-JamesMacleod syndrome in adults. Saudi Med J. 2003;24(2):195–198.
3. Mehra S, Basnayake T, Falhammar H, Heraganahally S, Tripathi S. Swyer–James–MacLeod syndrome—a rare diagnosis presented through two adult patients. Respirol Case Rep. 2017;5(5):e00245.
4. Capela C, Gouveia P, Sousa M, et al. Adult diagnosis of Swyer‐James‐MacLeod syndrome: a case report. J Med Case Rep. 2011;5:2.
5. Sen HS, Taylan M, Abakay O, Sezgi C, Cetincakmak MG. Adult diagnosis of Swyer-James-Macleod syndrome: retrospective analysis of four cases. Respir Care. 2014;59(4):e51-54.
6. Chong B, Xiao-Lian S, Hui S, Xiao-Peng Y, Qiang L. Swyer-James syndrome with peculiar course and ipsilateral pulmonary vein defect. Intern Med. 2011;50(17):1829-1833.
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.