Surgical treatment of airway obstruction in infants with Pierre Robin sequence

ABSTRACT

The deformities of micrognathia and glossoptosis in the newborn are frequently associated with a cleft palate, which is known as Pierre Robin sequence. Upper airway obstruction is the most serious problem in these patients. Treatment of Pierre Robin sequence includes either positional or surgical intervention. Mild cases are often managed in the prone position. However, when the patient fails to thrive due to chronic upper airway obstruction, or severe respiratory distress ensures despite positional treatment, surgical intervention is mandatory to relieve the obstruction. We experienced three infants with Pierre Robin sequence who showed a symptom triad of micrognathia, glossoptosis, and cleft palate. Intermittent cyanosis, depression of the chest, respiratory difficulty and feeding problems were also observed. To relieve severe upper airway obstruction caused by micrognathia and glossoptosis, we simultaneously performed modified tongue lip adhesion (TLA) and a subperiosteal release of the floor of the mouth (SRFM). Respiratory and feeding difficulties were relieved, the tongue positioned anteriorly, body weight increased, and mandibular growth improved. Simultaneous TLA and SRFM may constitute a simple and reliable method for surgical treatment of airway obstruction in patients with Pierre Robin sequence.