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Swyer-James-MacLeod syndrome (SJMS), or unilateral hyperlucent lung syndrome, is a rare disease thought to be associated with post-infectious bronchiolitis oblite- rans (1). This disease was first described in 1953 by Swyer and James in a 6-year-old child who had been treated with pneumonectomy (2). MacLeod presented a unilateral hyperlucency series one year later. From this date on, the disease was defined as Swyer-James- MacLeod syndrome (SJMS) (3). This disorder is diag- nosed in children with recurrent pulmonary infection or in asymptomatic adult cases during radiological exami- nations. The characteristic radiological findings include unilateral air trapping, hyperlucency signs, small or nor- mal sized lungs, and a decrease in the number and di- mensions of the pulmonary vessels (3).
A 22-year-old female patient was admitted to our hos- pital with recurrent cough, wheezing and fever. The me- dical history of the patient included frequent recurrent attacks of bronchiolitis and particularly, productive co- ugh triggered by infections. The physical examination of the patient revealed decreased respiratory sounds in the right lung and rare crepitant rales in the middle and lo- wer zones, while examinations of the other systems we- re normal. PA chest radiography and contrast-enhanced thoracic multi-slice computerize tomography (MSCT)
examinations were performed. The chest X-ray reve- aled hyperlucency and diminished peripheral vascular shadowing in the right lung (Figure 1). MSCT examina- tion revealed peribronchial thickening in the right midd- le and lower lobe, sequelae fibrotic changes, and areas of linear and cystic bronchiectasis, which were more prominent in the lower lobe (Figure 2). The right pulmo-
A case of Swyer-James-MacLeod syndrome diagnosed in adulthood
Nurefşan BOYACI1, Ömer KARAKAŞ1, Ekrem KARAKAŞ1, Funda YALÇIN2, Sema YILDIZ1, Hasan ÇEÇE1
1Harran Üniversitesi Tıp Fakültesi, Radyoloji Anabilim Dalı, Şanlıurfa,
2Harran Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Şanlıurfa.
Tuberk Toraks 2013; 61(4): 355-356• doi: 10.5578/tt.6131
* Bu olgu, 33. Ulusal Radyoloji Kongresi (2012, Antalya)’nde poster bildirisi olarak sunulmuştur
Yazışma Adresi (Address for Correspondence):
Dr. Nurefşan BOYACI, Harran Üniversitesi Tıp Fakültesi, Radyoloji Anabilim Dalı, ŞANLIURFA - TURKEY
e-mail: drnurefsan@yahoo.com
Figure 1. PA chest radiography showing hyperlucency and diminished peripheral vascular shadowing in the right lung.
EDİTÖRE MEKTUP/LETTER TO THE EDITOR
Tuberk Toraks 2013; 61(4): 355-356 Geliş Tarihi/Received: 12/08/2013 - Kabul Ediliş Tarihi/Accepted: 07/10/2013A case of swyer- james -macleod syndrome diagnosed in adulthood
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Tuberk Toraks 2013; 61(4): 355-356nary artery was hypoplastic (Figure 3). The findings of the bronchoscopy, performed to rule out lesions that may cause endobronchial obstruction and to investiga- te the presence of additional anatomical anomalies, we- re within normal limits. SJMS was diagnosed on the ba- sis of clinical findings and radiological studies.
SJMS is a type of obliterative bronchiolitis that occurs due to damage to developing lungs. In this syndrome, small bronchi and bronchioles are affected. There is an increase in aeration of the lung areas distal to these structures due to collateral air flow.
There is hypoplasia of the lung tissue and decrease in the diameter and the number of pulmonary arteries and their branches. The decrease in perfusion and air trapping results in the finding of unilateral hyperlucent lung. The hilum of the affected lung is small, while the lung volume is normal or slightly increased. Bronchi- ectasis, atelectasis, and focal scars may also be obser- ved (4).
The patients are typically asymptomatic and the disor- der is frequently diagnosed accidentally on chest radi- ography. Unilateral air trapping on expiratory chest X- rays is the key finding.
However, the fact that unilateral hyperlucency is the most frequent finding on chest radiography is due to the rotati- on of the patient and it is observed with a rate of 1% on chest X-rays (5). This lucency is caused by an increase in X-ray transmission on the side to which the patient turns. This can be understood by determining if the dis- tances between the medial ends of the clavicles and the spinous processes are equal. Moreover, unilateral hyper- lucency may be caused when X-rays are not centralized and in the presence of scoliosis in the spinal column.
SJMS should always be considered in adult cases with unilateral hyperlucency on chest X-ray and a history of recurrent pulmonary infection. Evaluation of these ca- ses by thoracic CT may prevent misdiagnosis or dela- yed diagnosis.
CONFLICT of INTEREST None declared.
REFERENCES
1. Mason RJ, Murray JF, Broaddus, VC, Nadel JA. Textbook of Respiratory Medicine. 4thed. Philadelphia: Elsevier-Sa- unders, 2005.
2. Swyer PR, James GCA. Case of unilateral pulmonary emphysema. Thorax 1953; 8: 133-6.
3. Kaplanoglu H, Kaplanoglu V, Toprak U, Dilli A, Hekimog- lu B. A case of Swyer-James-Macleod syndrome associ- ated with middle lobe hypoplasia and arteriovenous mal- formation. Case Rep Med 2012. doi: 10.1155/2012/
959153.
4. Lucaya J, Gartner S, García-Peña P, Cobos N, Roca I, Li- ñan S. Spectrum of manifestations of Swyer-James-Mac- Leod syndrome. J Comput Assist Tomogr 1998; 22: 592-7.
5. Dillman JR, Sanchez R, Ladino-Torres MF, Yarram SG, Strouse PJ, Lucaya J. Expanding upon the unilateral hyperlucent hemithorax in children. Radiographics 2011;
31: 723-41.
Figure 3. Axial MSCT image on the mediastinal window sho- wing hypoplasia of the right pulmonary artery.
Figure 2A-B. MSCT; axial image on the parenchymal window (a) and coronal MPR image (b) showing peribronchial thickening in the middle and lower lobes and areas of tubular and cystic bronchiectasis in the lower lobe.
A B
