Tracheomalacia in an infant with multiplex congenita (Larsen's) syndrome

An infant born with multiplex congenita (Larsen's) syndrome developed respiratory distress 30 days following tracheostomy for relief of upper airway obstruction. The infant had structural and functional abnormalities of the thoracic cage. Tracheobronchoscopy revealed excessive compliance of the trachea with a tendency for collapse of the tracheal rings and obliteration of the tracheal lumen. Continuous positive airway pressure in the range of 20-25 cm H2O was used to maintain patency of the tracheal lumen and assure adequate ventilation. Hemodynamic and pulmonary barometric complications often observed when high levels of positive airway pressure are utilized in infants were not observed.