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Swyer-James Syndrome

Syndrome Swyer James

Swyer-James syndrome, also known as unilateral hyperlucent lung syndrome, refers to the manifestation of postinfectious obliterative bronchiolitis. Most patients do not develop symptoms while the remaining exhibit a productive cough, hemoptysis, exertional dyspnea, and decreased exercise tolerance. The diagnosis is based on a thorough history, physical exam, and imaging studies that characteristically reveal hyperlucency, hypovascularity, and a smaller lung.


Presentation

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]
Cyanotic Congenital Heart Disease
  • The relevant polycythemia was most probably secondary to cyanotic congenital heart disease associated with the newly diagnosed PDA with minimal right-to-left shunt.[jmedicalcasereports.biomedcentral.com]
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]
Constipation
  • For 15 years Ann's been suffering from Bronchietasis with symptoms like stiffness, difficulty in breathing, constipation, fatigue, chronic headaches and muscle aches. She needed to be dependent on 6 portable oxygen cylinders each month.[steadyhealth.com]
  • The patient showed sedation and constipation while building up dose to 300 mg, but her blood parameters were within normal range. The patient started showing a response on positive symptom on similar 300 mg doses of clozapine.[ijpm.info]
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]
  • This patient presented with primary amenorrhea. Clinical characteristics showed a typical stigmata of gonadal dysgenesis: primary amenorrhea, sexual infantilism, a small uterus and bilateral streak gonads.[lymphedemapeople.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]
  • This patient presented with primary amenorrhea. Clinical characteristics showed a typical stigmata of gonadal dysgenesis: primary amenorrhea, sexual infantilism, a small uterus and bilateral streak gonads.[lymphedemapeople.com]

Workup

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]
  • pleural effusion, accompanied by a Mycoplasma complement-fixation titer increasing to 1:16,384.[jhu.pure.elsevier.com]

Treatment

  • Bronchodilators and steroid treatment had no effect.[ncbi.nlm.nih.gov]
  • She is under supportive treatment and her respiratory symptoms have been improved.[ncbi.nlm.nih.gov]
  • This treatment improved his pulmonary function, and he is active as a bicycle racer now.[ncbi.nlm.nih.gov]
  • Treatment of this syndrome is intended to reduce the pulmonary lesions and improve the patient's quality of life.[ncbi.nlm.nih.gov]
  • Treatment of this condition consists of antibiotics, mucolytics, bronch-spasmolytis, and physiotherapy. Surgery is rarely indicated.[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • With current pharmaceutical developments, the prognosis is good for sufferers of the illness to lead normal and healthy lives.[checkorphan.org]
  • Reports on long-term follow-up and prognosis of Swyer–James–MacLeod syndrome are lacking in the literature; however, one case report describes a normal pregnancy outcome in a patient with the condition [ 9 ].[breathe.ersjournals.com]
  • […] therapies with a favorable impact on prognosis.[medichub.ro]

Etiology

  • […] is to present the existing literature accumulated from 1955 to 1992 pertaining to 46,XY pure gonadal dysgenesis (Swyer-James syndrome), add the findings of a personally observed patient, and highlight the complexities in attempting to determine the etiology[ncbi.nlm.nih.gov]
  • 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]
  • Regarding the etiology, Swyer-James syndrome is thought to be acquired following infections that occurred during infancy or childhood.[symptoma.com]
  • […] review is to present the exiting literature accumulated from 1955 to 1992 pertaining to 46,XY pure gonadal dysgenesis (Swyer-James syndrome), add the findings of a personally observed patient, and highlight the complexties in attempting to determine the etiology[journals.lww.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.com]

Epidemiology

  • 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]
  • BO affects mainly male children during the first year of life. 1 - 3 Despite 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 , 5 There are[scielo.br]
  • 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]
  • 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]
Sex distribution
Age distribution

Pathophysiology

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

Prevention

  • 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]
  • Management of the syndrome is typically conservative, with prevention and early treatment of pulmonary disease and, occasionally, resection for recalcitrant disease.[ncbi.nlm.nih.gov]
  • 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]
  • Prevention - Swyer James McLeod Syndrome Not supplied. Diagnosis - Swyer James McLeod Syndrome Not supplied. Prognosis - Swyer James McLeod Syndrome Not supplied.[checkorphan.org]

References

Article

  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.

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Last updated: 2019-07-11 21:12