Swyer-James/MacLeod syndrome is an uncommon disease with characteristic radiological feature of unilateral hyperlucency due to loss of pulmonary vasculature and air trapping. Typically, this disorder is diagnosed in childhood during evaluations for recurrent respiratory infections. Here, we report a case in a 30-year-old adult female who presented with dyspnoea, cough with expectoration and recurrent hemoptysis due to associated bronchiectasis. This case highlights the importance of computed tomography in the diagnostic workup of recurrent hemoptysis in pulmonary tuberculosis epidemic countries like India

Ghulam Hassan

Nautiyal RG

Rashmi Upadhyay

S Saheer

Santosh Kumar

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 GJMEDPH, Vol 1(3) May- June 2012  Page 24    Global Journal of Medicine and Public Health     www.gjmedph.org   Swyer- James -MacLeod syndrome presenting as hemoptysis in an adult  Santosh Kumar 1, S Saheer2, Nautiyal RG3, Ghulam Hassan4, Rashmi Upadhyay 5.  1: Professor, 2: Junior Resident, 3. Associate Professor, 4: Post-doctoral Trainee, 5. Junior Resident, Department of Pulmonary Medicine, CSM Medical University (Erstwhile KGMC) and Sushila Tiwari Memorial Medical College UP, Lucknow, India A B S T R A C T Swyer-James/MacLeod  syndrome  is  an  uncommon  disease  with  characteristic  radiological  feature  of  unilateral hyperlucency  due  to  loss  of  pulmonary  vasculature  and  air  trapping.  Typically,  this  disorder  is  diagnosed  in childhood during evaluations for recurrent respiratory infections. Here,  we report a case in a 30-year-old adult female  who  presented  with  dyspnoea,  cough  with  expectoration  and  recurrent  hemoptysis  due  to  associated bronchiectasis. This case highlights the importance of computed tomography in the diagnostic workup of recurrent hemoptysis in pulmonary tuberculosis epidemic countries like India. Key-words:  Swyer-James/MacLeod syndrome, unilateral hyperlucent lung, hyperinflation, bronchiolitis obliterans, pulmonary artery abnormalities.  Corresponding Author: Santosh Kumar, Professor, Department of Pulmonary Medicine C.S.M. Medical University, U.P., Lucknow (U.P.) 226003, India E-mail – skumarchest@yahoo.com                   Funding: None         Conflict of interest: None     Introduction: Swyer-James/MacLeod syndrome was first described by Swyer and James1 in 1953 and further detailed by Macleod  in  1954.2  It  is  a  rare  acquired  disease occurring secondary to postinfectious bronchiolitis in childhood.3  Bronchiolitis  obliterans  leads  to  reflex vasoconstriction  and  hypoperfusion  causing hypoplasia  of  pulmonary  vasculature  leading  the development of hyperlucent pathologic lung. Usually this  disorder  is  diagnosed  in  childhood  after  an evaluation  for  recurrent  respiratory  tract  infections but  sometimes  their  diagnosis  may  be  missed  until adulthood  if  the  patient  has  no  or  little  sequelae bronchiectasis. The rarity of the disorder and clinical curiosity prompts us to report this case.   Case History: A  30-year-old  female  presented  to  our  outpatient department with complaints of dyspnoea, cough with expectoration  for  last  twenty  years  and  recurrent hemoptysis for the last three years. She was taking the treatment for asthma diagnosed on the basis of clinical  and  spirometry  for  last  eighteen  years.  For the  complaint  of  hemoptysis  she  was  prescribed multiple courses of emperical antitubercular drugs in the past without sputum smear examination. She had normal haematological and biochemical parameters. On examination her vital signs were within normal limits.  Respiratory  examination  revealed  reduced chest  movements  and  breath  sounds  on  the  left hemithorax with coarse crackles in the left lower lung fields  and  bilateral  rhonchi.  Once  hemoptysis  was controlled her sputum for acid fast bacilli was done twice which turned out to be negative. Her admission chest radiograph showed left sided hyperlucent lung with  small  ipsilateral  hilar  shadow  [Figure  1].  A diagnostic bronchoscopy was done, but there was no endobronchial growth or bleeding points. As clinical and  radiologic  findings  were  unable  to  explain  the cause of hemoptysis computed tomography (CT) of thorax  was done which revealed small left lung with decreased  vascularity  and  bronchiectactic  segments in the left lower lobe with airtrapping on expiration [Figure  2a  and  2b].  As  CT  was  suggestive  of  the  GJMEDPH, Vol 1(3) May- June 2012  Page 25  disorder,  a  pulmonary  angiogram  was  done  to confirm the diagnosis which revealed hypoplasia of left pulmonary artery and vein [Figure 3]. Therefore the  patient  was  diagnosed  to  have  Swyer-James-MacLeod syndrome and the cause of hemoptysis was ascertained to be due to bronchiectasis of left lower lobe as confirmed by CT. Ventilation perfusion scan and  surgical  removal  of  bronchiectactic  segments were  advised  to  the  patient,  both  of  which  were refused  by  the  patient.  She  was  prescribed conservative  management  for  hemoptysis  and  is under our regular follow up.  Table 1.Causes of unilateral hypertranslucency of the lung 7. Normal Increased  density  of  contralateral  lung,  e.g.  pleural effusion/thickening, consolidation  Technical Rotation, scoliosis  Soft tissue  Mastectomy Congenital absence of pectoralis muscle Poliomyelitis  Emphysema Compensatory: lobar collapse, lobectomy Obstructive:  foreign  body,  tumour,  Macleod's syndrome, congenital lobar emphysema Bullous   Vascular Absent/hypoplastic pulmonary artery Obstructed  pulmonary  artery,  e.g.  by  tumour, embolus Macleod's syndrome  Pneumothorax  Discussion:  This  disorder  may  present  with  various  clinical features and the reported prevalence of this disease is 0.01%  in  17,450  survey  chest  radiograph.4  The patient  can  be  completely  asymptomatic  with hyperlucent lung field as an incidental finding on the chest  radiograph  taken  for  other  indications. Recurrent  respiratory  infections,  productive  cough, shortness  of  breath  and  dyspnoea  on  exertion  and rarely hemoptysis as in our case may be the alternate      presentations.5  If  there  is  no  bronchiectatic component, the prognosis is usually favourable. The classic  chest  radiographic  finding  is  a  pronounced one  sided  hyperlucency  due  to  the  oligemia  of  the involved  segments  of  the  lung.6  Expiratory radiograph  may  demonstrate  air  trapping  or mediastinal  shift  towards  the  opposite  side.  The various  causes  of  unilateral  hyperlucent  lung  are given below [Table 1].7 CT is the imaging technique of  choice  despite  characteristic  findings  in  chest radiograph.5 It not only show hyperlucent areas, but also helps out in ruling out other causes of unilateral hyperlcent  lung,  endobronchial  tumour  partially obstructing the lumen of a lobe and in assessing the type and extend of bronchiectasis. This modality of investigation  have  a  valuable  role  in  countries  like India  were  antitubercular  drugs  are  first  prescribed drugs  for  the  management  of  hemoptysis  without exactly  knowing  the  etiology.  Spirometry  shows mixed  pattern  with  reversible  obstructive  airway disease. Ventilation perfusion (V/Q) scanning of the lungs  shows  matched  V/Q  defects  and  a  marked trapping  on  the  washout  phase  on  133  Xe scintigraphy.8  In  our  case  despite  doing  various investigations the cause of hemoptysis in our patient was ascertained only until we did a CT.  In conclusion, this case reaffirms the utility of CT in diagnostic  workup  of  recurrent  hemoptysis  in countries like India where tuberculosis is an epidemic.  References: 1.  Swyer  PR,  James  GCW.  A  case  of  unilateral pulmonary emphysema. Thorax 1953; 8:133-136.      2.  Macleod  WM.  Abnormal  transradiancy  of  one lung. Thorax 1954; 9:147-153 3.  Fragonese  L,  Girosi  D,  Battistini  E.  Clinical, physiologic  and  roentgenographic  changes  after pneumonectomy  in  a  boy  with  Macloed/Swyer-James  Syndrome  and  bronchiectasis.  Pediatric Pulmonology 2002; 34:412-416. 4.  Yiu MWC, Tsang KWT, Wong Y, Ooi GC. Focal area  of  hyperlucency  on  a  chest  radiograph. Respiration 2001; 68:545-7. 5.  Lucaya  J,  Gartner  S,  Garcia-Pena  P,  Cobos  N, Roca I, Linan S. Spectrum  of  manifestations of Swyer-James-MacLeod  syndrome.  J  Comput Assist Tomogr. 1998; 22:592-7. 6.  Chalmers  JH  Jr.  Swyer-James  syndrome.  Semin Respir Infect 1999; 14:295–297. 7.  Janet M, Philip JA Robinson, Richard W. Whitehouse, Andrew R Wright, Jeremy PR Jenkins. The normal chest: methods of Investigation and differential  Diagnosis.  In:  David  Sutton,  editor.  Text Book  of  Radiology   and  Imaging.  7th  ed. Edinburgh: Churchill Livingstone; 2003. p. 38. 8.  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.    GJMEDPH, Vol 1(3) May- June 2012  Page 24  Figure 1.Admission chest radiograph (PA view) showing hyperlucent left lung(arrow) with small ipsilateral hilar shadow.    Figure 2:Computed tomography of chest (a)  in mediastinal window showing small left pulmonary artery[blue arrow] and coronal CT image (b)  showing hyperlucent areas in left lung field(Thick blue arrow)  and bronchiectactic segments(Thin blue arrow) in left lower lobe.        GJMEDPH, Vol 1(3) May- June 2012  Page 25  Figure 3: CT Pulmonary angiogram showing a small left pulmonary artery (arrow heads) with reduced size and number of branches with diminished peripheral vasculature in the left lung.                   Access This Article Online Quick Response Code:   Website: www.gjmedph.org   

Swyer- James -MacLeod syndrome presenting as hemoptysis in an adult

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