Diaphragm pacing in infants and children.

Since 1976 we have implanted bilateral diaphragm pacers in 34 infants and children: 26 with central hypoventilation syndrome (CHS), three with myelomeningocele, and five with quadriplegia. Compared to adults, several modifications have been necessary to achieve effective ventilation in infants and younger children. In all instances, a tracheostomy has been necessary due to impaired neuromuscular control of upper airway patency during pacing. Bilateral pacing has been necessary to achieve adequate ventilation; in the CHS children with normal awake ventilation, bilateral pacing during sleep eliminates the need for positive pressure ventilation. For the remaining children, adequate awake ventilation is achieved with bilateral pacing, thus permitting substantially greater mobility and limiting use of the ventilator to sleep time. Our longest survivor has now been paced for 10.7 years, and in no instance has phrenic nerve damage occurred secondary to electrical stimulation. Our current pacing regime is characterized by moderate respiratory rates (21 breaths/min), long interpulse intervals (95 ms), and short inspiratory times (0.6 sec), resulting in 50%-75% fewer stimuli/min compared to our previous regime. For all infants and children requiring 24-hour ventilatory assistance, our recent successes in maintaining ventilation using significantly fewer stimuli suggest that long-term continuous pacing is a realistic future goal.