Spectrum of manifestations of Swyer-James-MacLeod syndrome

Abstract

Purpose: The clinical and imaging findings of children with Swyer-James-MacLeod syndrome (SJMS) were reviewed to determine the incidence and type of bronchiectasis and analyze whether the clinical course of patients with bronchiectasis differed from that of patients without bronchiectasis.

Method: Our study population consisted of 13 patients. All had inspiratory/ expiratory chest X-ray films, chest CT, and lung scans. Eight underwent pulmonary function test. The results of these studies at diagnosis and during follow-up were analyzed and compared with the clinical features.

Results: Bronchiectasis was demonstrated in nine patients, being saccular in five and cylindrical in four. Expiratory slices were helpful for demonstrating bilateral lung involvement that had not been suspected on inspiratory CT scans or conventional radiographs. The clinical features of the five patients with saccular bronchiectasis resembled those of patients with classic postviral bronchiectasis who suffered recurrent pulmonary infections; three of them underwent lobectomy. The remaining patients presented mild respiratory symptoms, with a spontaneous tendency toward improvement.

Conclusion: SJMS should be considered as a spectrum disease. Bronchiectasis is not a universal finding. The presence and type of bronchiectasis will influence clinical manifestations and prognosis. Patients without bronchiectasis or with cylindrical bronchiectasis had a lower incidence of pneumonia episodes than those with saccular bronchiectasis.