This case series called attention to certain differences between our group of patients and those reported in the international literature. Patients were predominantly female and were diagnosed between 1 and 20 months of life. All were asymptomatic at the time of the study. Half of the patients had a history of respiratory disease and all patients had with pulmonary hypertension. Surgical management was required in on-third of the patient group.
Pulmonary aplasia is an uncommon pathology. Due to great variability in clinical presentation, there must be a high index of suspicion in the presence of a fully radiopaque hemithorax. Among the diagnostic methods, X-rays, tomography and lung scan are useful. Bronchoscopy is required for diagnostic confirmation. Surgical removal of the stump and translocation of the diaphragm have been proposed as surgical options.
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