Misdiagnosis of pulmonary embolism
Department of Respiratory Medicine, Norfolk and Norwich University Hospital, Colney Lane, Norwich NR4 7UY, UK. email: dkclee{at}doctors.org.uk
A 43-year-old lady complained of shortness of breath following breast surgery, and was hypoxic on air. A chest radiograph (left) showed increased radiolucency of the left lung, which was thought to be consistent with oligaemia due to a large acute pulmonary embolism (Westermark's sign), and appropriate treatment was administered. Subsequent radioisotope lung ventilation perfusion scanning showed a significant matched defect of the left lung with a more severe ventilatory disturbance. Computer tomography pulmonary angiography of the thorax (right) did not show any pulmonary emboli, but demonstrated reduced density in the left lung, particularly the left lower lobe, with slightly dilated and thick walled bronchi consistent with a diagnosis of Macleod (Swyer-James) syndrome (MSJS).
It is thought that severe respiratory tract infections acquired in childhood lead to an arrest in lung growth, and this in turn culminates in the development of MSJS. Indeed this is often reflected in the chest radiograph as hypoplasia of the pulmonary artery, typical of the arrested development in MSJS. This is in contrast to the features found in pulmonary embolism, where there is enlargement and truncation of the proximal pulmonary artery. Lung ventilation and perfusion are both reduced in MSJS, whereas only the latter tends to be affected in pulmonary embolism. The clinical presentation of MSJS is remarkably heterogeneous, ranging from incidental finding on chest radiography (as in this case) to that of severe chronic lung disease, and treatment options tend to vary accordingly.
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