Abstract
Swyer-James-Macleod syndrome (SJMS) is a rare clinical entity acquired during childhood due to a respiratory infection leading to bronchiolitis obliterans. This inciting event is hypothesized to cause structural and functional changes of the developing alveoli, terminal bronchioles, and the corresponding pulmonary vasculature, resulting in emphysematous changes and a matched ventilation-perfusion defect. We present a 67-year-old male patient with hypercapnic respiratory failure requiring invasive mechanical ventilation, who had typical features of SJMS undiagnosed before this admission. He was extubated successfully, discharged home, and continues to be stable at a 90-day follow-up period. This marks one of the rare accounts where a patient with SJMS was given ventilatory support emergently, and one of the oldest patients reported. SJMS is under-reported due to its indolent clinical course and misdiagnosed as some other pulmonary abnormality. The clinical course progression and prognosis are unclear and variable in many affected patients due to this condition's rarity.
Highlights
Swyer-James-Macleod syndrome (SJMS) was named after the three physicians who described them simultaneously in the 1950s [1,2]
We present an elderly male with co-morbidities and undiagnosed SJMS presenting with acute hypercapnic respiratory failure
Computed tomography (CT) without contrast (Figure 2) and CT angiography of the chest done during a prior hospitalization (Figure 3) revealed features typical of SJMS
Summary
Swyer-James-Macleod syndrome (SJMS) was named after the three physicians who described them simultaneously in the 1950s [1,2]. We present an elderly male with co-morbidities and undiagnosed SJMS presenting with acute hypercapnic respiratory failure. The patient was managed as an acute exacerbation of obstructive lung disease and acute exacerbation of diastolic heart failure. He failed bilevel positive airway pressure and required mechanical ventilation. Posteroanterior view of portable chest radiograph of the patient on presentation showing the signature finding of SJMS, right upper lobe hyperlucency. Computed tomography of the chest without contrast demonstrating right upper lobe air trapping and volume loss of left hemithorax. CT angiography of the patient showing small-calibre pulmonary vasculature of right upper lobe consistent with SJMS with associated pulmonary changes mainly paucity of broncho-alveolar structures
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