Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 12  |  Issue : 2  |  Page : 273-275

Unilateral lung hyperlucency in an adult: an unusual cause


All India Institute of Medical Sciences, New Delhi, India

Date of Submission07-Aug-2017
Date of Acceptance12-Nov-2017
Date of Web Publication23-May-2018

Correspondence Address:
Animesh Ray
46 C, Masjid Moth, Phase 1 New Delhi 1100048
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ejb.ejb_72_17

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  Abstract 

We report a case of young adult with unilateral lung hyperlucency. His radiological features were suggestive of Swyer–James syndrome. The condition is uncommon in adults. We report this rare case with discussion on clinical presentation, diagnosis, and management of Swyer–James syndrome. The report also throws light on various other causes of unilateral lung hyperlucency and its approach.

Keywords: bronchiectasis, pulmonary artery hypoplasia, unilateral lung hyperlucency


How to cite this article:
Kodan P, Das A, Vyas S, Ray A, Sharma SK. Unilateral lung hyperlucency in an adult: an unusual cause. Egypt J Bronchol 2018;12:273-5

How to cite this URL:
Kodan P, Das A, Vyas S, Ray A, Sharma SK. Unilateral lung hyperlucency in an adult: an unusual cause. Egypt J Bronchol [serial online] 2018 [cited 2018 Aug 14];12:273-5. Available from: http://www.ejbronchology.eg.net/text.asp?2018/12/2/273/233047


  Case report Top


A 21-year-old male patient presented with exertional, nonprogressive shortness of breath since childhood, modified medical research council dyspnea scale − grade 2, nonprogressive without associated wheeze/stridor. On examining, he revealed history of multiple prolonged respiratory infections in childhood. However, no previous medical records were available. On examination, breath sounds were mildly reduced on left side. Other systemic examination finding was normal. Chest radiographs showed diffuse hyperlucency in the left hemithorax ([Figure 1]). High-resolution computed tomography (CT) of the thorax also showed left-sided hyperlucency with presence of bronchiectasis ([Figure 2]). There was also evidence of air trapping in the left side on an expiratory scan. CT pulmonary angiography revealed narrowed left branch of pulmonary artery ([Figure 3] and [Figure 4]). This combination led us to the diagnosis of Swyer–James syndrome [1].
Figure 1 Chest X-ray Showing lung hyperlucency.

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Figure 2 CT Scan showing left sided hyperlucency with presence of bronchiectasis.

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Figure 3 CT pulmonary angiography showing narrowed left branch of pulmonary artery.

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Figure 4 Virtual bronchoscopy showing slight narrowing of the left main bronchus without any luminal compromise.

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Virtual bronchoscopy done showed slight narrowing of the left main bronchus without any luminal compromise. Virtual bronchoscopy did not show any significant compromise of the bronchial lumen.

Spirometry showed that forced expiratory volume in 1s was reduced (1.5 l/65% of predicted) and forced vital capacity was reduced (1.8 l), with a normal forced expiratory volume 1/forced vital capacity ratio of 0.83. However, body plethysmography showed normal total lung capacity (6.1 l) with an increased residual volume (4.23 l), suggestive of air trapping. This nonspecific pattern of ventilatory defect as seen in our case has been described in association with obstructive lung diseases.

The patient was managed with bronchodilators, and regular follow-up was done for timely and prompt management of any infection. The patient was also advised pneumococcal and annual influenza vaccinations. The patient improved with conservative management and is on regular follow-up.


  Discussion Top


Swyer–James syndrome or Mcleod or Bret syndrome is a unilateral hyperlucent lung syndrome which was first described by Swyer, James, and Mcleod in Canada and independently by Dr Bret from France [1]. A childhood history of infections in the form of viral bronchiolitis or pneumonitis can usually be traced in these patients [2]. The etiology of this disorder has been linked to childhood infection leading to abnormality of the bronchial architecture leading to secondary pulmonary artery hypoplasia. Alternatively, it is thought that primary pulmonary arterial abnormality predisposes the patient to respiratory tract infection and subsequent airway/alveolar changes. Pathologically, bronchiolitis obliterans can be appreciated in the affected lung.

Patient may be asymptomatic or present with dyspnea on exertion, cough, or history of repeated chest infections. A plain radiograph usually reveals single lung hyperlucency with a normal or decreased hemithorax with a small unilateral hilum. CT scan is the diagnostic modality of choice to clinch the diagnosis, and it reveals hyperlucent lung with diminished vascularity [3].

All cases of unilateral hyperlucency should be systematically evaluated (Flowchart 1 and [Table 1] and [Table 2]). Any unilateral hyperlucency should prompt the physician to look for the accurate cause relevant to the history. Unilateral hyperlucency can be attributed to just a technical factor like malrotation, to decreased chest wall thickness in relevant history of mastectomy or in Poland syndrome, or to any other etiology such as obstructive causes like Swyer–James syndrome, as in our case. Swyer–James syndrome should be considered as one of the differential factors in any case of obstructive hyperinflation. In Swyer–James syndrome, expiratory scan will reveal features of air trapping. Bronchiectasis is usually present in long-standing cases but is not a universal finding. CT pulmonary angiography will show evidence of narrowed pulmonary artery of the affected side [4]. Pulmonary function studies may show features of obstructive ventilatory defect. Pulmonary ventilation and perfusion scan usually demonstrates matched ventilation and perfusion defects. Management of Swyer–James syndrome includes the early control of lung infections, bronchodilators, and preventive vaccinations. In severe cases, even lung resection has been attempted as a last resort [5].
Table 1 Pathophysiologically unilateral transradiancy of the hemithorax can be caused by a number of factors

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Table 2 The differentiating points between the different causes of lung hyperlucency

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  Conclusion Top


Swyer–James syndrome should be considered as a differential diagnosis in case of unilateral lung hyperlucency. Diminished lung vascularity and bronchiectasis along with features of unilateral air trapping on the affected side can help us clinch the diagnosis [1],[4]. Prevention of respiratory infections and symptomatic care should remain the key management strategy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Capela C, Gouveia P, Sousa M, Regadas MJ. Adult diagnosis of Swyer-James-MacLeod syndrome: a case report. J Med Case Reports 2011;5:2.  Back to cited text no. 1
    
2.
Fregonese L, Girosi D, Battistini E, Fregonese B, Risso FM, Bava GL et al. Clinical, physiologic, and roentgenographic changes after pneumonectomy in a boy with Macleod/Swyer–James syndrome and bronchiectasis. Pediatr Pulmonol 2002; 34:412–416.  Back to cited text no. 2
    
3.
Moore AD, Godwin JD, Dietrich PA, Verschakelen JA, Henderson WR Jr. Swyer-James syndrome: CT findings in eight patients. Am J Roentgenol 1992; 158:1211–1215.  Back to cited text no. 3
    
4.
Marti-Bonmati L, Ruiz Perales F, Catala F, Mata JM, Calonge E. CT findings in Swyer–James syndrome. Radiology 1989; 172:477–480.  Back to cited text no. 4
    
5.
Koyama T, KOsada H, Kitanaka Y, Funaki S, Hiekata T. Surgically treated Swyer–James syndrome. Jpn J Thorac Cardiovasc Surg 2001; 49:671–674.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2]



 

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