Swyer-James-MacLeod syndrome presenting as spontaneous pneumothorax in an adult: Case report and review of literature.

ABSTRACT

Swyer-James-MacLeod Syndrome (SJMS) is an uncommon, emphysematous disease characterized by obliteration of the small bronchioles, hypoplasia/or absence of pulmonary artery and peripheral vascular bed. It is most commonly diagnosed in childhood. Patients are often asymptomatic or they could suffer from symptoms of recurrent pulmonary infections. Spontaneous pneumothorax is a rare presentation of this syndrome. We report a case of a 42-year old female patient presented at our Emergency Department with complaints of dyspnea and pleuritic chest pain with a diagnosis of spontaneous pneumothorax in emphysematous disease. The diagnosis of SJMS is usually based on imaging and clinical findings rather than on the results of pathologic examination; indeed, asymptomatic adult patients with SJMS are often diagnosed after a chest radiograph obtained for another reason. High-resolution computed tomography (HRCT) seems to be the most appropriate technique for the diagnosis. SJMS can be associated with spontaneous pneumothorax which represent an emergency condition due to the underlying pathological disease. The surgical treatment of the affected lung should be considered when conservative approach is ineffective.