Swyer-James-MacLeod Syndrome: A case report in an adult and review of the literature

Abstract

Swyer-James-MacLeod Syndrome (SJMS) is a rare, unilateral lung disease represented by radiographic translucency of the lung parenchyma secondary to the diminution of the pulmonary vasculature and to the overdistention of the alveoli. It is an uncommon sequela of post-infectious bronchiolitis obliterans (BO) in childhood. Patients with SJMS are often diagnosed in childhood and typically present with recurrent respiratory tract infections. Symptoms during childhood can be mild or absent, leading to a delayed diagnosis in adulthood. SJMS is characterized by the destruction of the small bronchioles and agenesis or hypoplasia of the pulmonary arteries leading to hypoperfusion of the pulmonary parenchyma, resulting in characteristic chest imaging findings of unilateral hyperlucency or translucence.Swyer and James first described this syndrome in 1953. It is a rare disease that can be can be caused by an infection with adenoviruses (types 3, 7, or 21) or Bordetella pertussis, a foreign body in the airway and hydrocarbon inhalation. We present a case of SJMS in whom the adult patient had been misdiagnosed with chronic obstructive pulmonary disease (COPD). She was eventually diagnosed with SJMS based on chest x-ray and chest CT findings of unilateral lung hyperlucency, as well as with scintigraphic findings showing virtually absent perfusion to the left lower lobe of the lung.