Lung aplasia: anatomy, history, diagnosis and surgical management.

PURPOSE: The aim of the study was to define the details of the history and clarify the cause for respiratory distress, justify the need for surgical correction and suggest a rational operative technique for patients with lung aplasia.

METHODS

Our experience with the management of 9 patients with lung aplasia and 1 patient with lung agenesis in the period from 1985 to 2004 is presented. All 10 patients were referred for respiratory distress. A detailed study of clinical symptoms and the data from X-ray, computed tomography (CT), tracheobronchoscopy and digital subtraction angiography (DSA) suggested that all patients had different degrees of mediastinal shift and heart rotation, tracheal kinking and compression of the aortic arch and innominate artery or emphysema of a single lung. Two of the patients required operation.

RESULTS

We performed ipsilateral cephalad translocation of the diaphragm which resulted in complete recovery from respiratory distress and a significant improvement in tolerance of physical exercise in both patients. The good results in these patients were followed up for 8 and 2 years, respectively.

CONCLUSIONS

We suggest that ipsilateral translocation of the diaphragm could be a useful alternative to unsuccessful symptomatic treatment in patients with lung aplasia.