Classical Imaging Triad in a Very Young Child with Swyer-James Syndrome

Abstract

Swyer-James syndrome (SJS) or Swyer-JamesMacLeod syndrome is a rare disorder characterized by roentgenographical hyperlucency of one lung, lobe, or part of a lobe. As originally described, it shows a unilateral small lung with hyperlucency and air trapping on radiographs. It is postinfectious obliterative bronchiolitis due to childhood respiratory infection. In SJS, the involved lung or portion of the lung does not grow normally. The characteristic radiologic appearance is that of pulmonary hyperlucency on chest X-ray/CT caused by over-distention of the alveoli in conjunction with diminished arterial flow detected on Tc-99 m MAA pulmonary perfusion scan. Radionuclide pulmonary ventilation studies are considered difficult to perform in very young children as patient co-operation is crucial to achieve adequate tracer entry into the lungs. We present here the case of a female child aged 2 years, who presented with chronic productive cough and fever off and on for 1.5 years. She had classical features on imaging and markedly diminished ventilation on Tc-99 m DTPA aerosol study. Our case highlights the typical findings on ventilationperfusion scan (V/Q scan) and suggests that it is possible to conduct a satisfactory ventilation study with Tc-99 m DTPA aerosol even in very young children.

Multimodal imaging findings in an adult case of Swyer‐James‐MacLeod syndrome

“…In SJMS, the involved lung or portion of the lung does not grow normally. Fibrosis and obstruction of the terminal and respiratory bronchioles, likely caused by respiratory infections in early childhood, prevent the normal development of the alveolar bud .…”

Swyer-James-Macleod syndrome: a rare finding and important differential in the ED setting

“…Swyer-James-Macleod syndrome is late sequela of infectious bronchiolitis that occurs during childhood. Causative organisms involved include Mycoplasma pneumonia, influenza A, mycobacteria, Bordetella pertussis, adenovirus, paramyxovirus, and measles [4]. The infectious agent results in an inflammatory response, which causes obliteration of peripheral airways in the infected lung.…”

An uncommon obliterative lung disease: Swyer–James–MacLeod syndrome

“…This syndrome is most often seen in children, but can rarely present in adults with a past medical history of recurrent respiratory infections. SJMS can be caused by numerous microbes including adenovirus types 3,7, and 21, paramyxovirus morbillivirus, Bordetella pertussis, Mycoplasma pneumoniae, Mycobacterium tuberculosis, and influenza A [2,3]. These infections can cause post-infectious bronchiolitis obliterans, and may also cause hypoplasia or agenesis of the pulmonary vasculature.…”

“…These infections can cause post-infectious bronchiolitis obliterans, and may also cause hypoplasia or agenesis of the pulmonary vasculature. In the latter scenario, hypoperfusion of the pulmonary parenchyma will result in unilateral hyperlucency of the lung on radiographic images [2,3]. The symptoms of SJMS can vary from asymptomatic to chronic or recurring lung infections, dyspnea, or hemoptysis [1].…”

“…Diagnosis is made following radiographic evidence (plain film and CT) of the classic SJMS triad: which includes a unilateral hyperlucent lung, diffusely decreased ventilation, and matching decreased perfusion in the affected lung [3]. Unilateral lung hypoplasia, destruction of the pulmonary parenchyma and air trapping (bronchiectasis) are also likely to be visualized on CT imaging [1,2].…”