Pierre Robin

 

Pierre Robin sequence means a baby born with a short lower jaw (micrognathia), which in turn makes the tongue sit high in the mouth causing a cleft palate. The prominent tongue can cause breathing problems as it blocks the back of the throat. If this is not severe, nothing need be done, as most children will have catch up growth of the jaw, and grow out of the problem within a year or two. If there are breathing problems, a sleep study may show the severity, and whether intervention is required. 

 

Some children have a less severe form with no cleft palate. However they may also have very compromised breathing. Children with Pierre Robin may be very difficult to intubate. If the baby's breathing is very blocked a tracheotomy is usually needed. Other treatments are rarely effective. The tracheotomy can usually be removed once the jaw has grown. In a few children the jaw does not grow sufficiently, and an operation (mandibular distraction) is needed to lenghthen the jaw.

 

Children with Pierre Robin are more prone to have problems at the tracheostomy site and tracheomalacia, which may delay removal of the tracheotomy tube. The cleft palate is usually repaired before one year of age.