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.

Aplasia of the right lung in a 4-year-old child: surgical stabilization of the mediastinum by diaphragm translocation leading to complete recovery from respiratory distress syndrome.

Lung aplasia is defined as unilateral absence of the lung with preservation of main bronchus remnant at the tracheal bifurcation. Patients usually die soon after birth and there is no specific therapy for this condition, as evidenced by the literature. The authors present a case of an infant that was asymptomatic with this malformation until 3 months of age, when the child had respiratory distress syndrome. Subsequently, lung aplasia was diagnosed. The authors performed an extrapleural dissection and cephalad translocation of diaphragm to reduce the mediastinal shift and heart rotation, to relieve a kink and compression of the trachea by the aortal arch and truncus arteriosus, as well as to relieve hyperinflation of lung parenchyma and provide recovery from respiratory distress syndrome. This new approach resulted in complete recovery from respiratory distress syndrome and full tolerance of physical exercise. The child underwent follow-up for 4 years. Diaphragmatic translocation may be useful in treatment of respiratory disorders associated with lung aplasia.