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. J Pediatr Surg 35:1499-1502. Copyright © 2000 by W.B. Saunders Company.