Micrognathia

Pierre Robin Sequence consists of micrognathia usually accompanied by a high arched or midline cleft palate.

The tongue is usually of normal size, but the floor of the mouth is foreshortened.

Obstruction of the air passages may occur, particularly on inspiration & usually requires treatment to prevent suffocation.

The infant should be maintained in a prone or partially prone position so that the tongue falls forward to relieve respiratory obstruction.

Some patient may require endotracheal intubation or tracheostomy. Often the growth of the mandible will achieve a normal profile within 4 to 6 years.

Feeding of infants can usually be accomplished without resorting to gavage.

A specialized feeder (Breck) facilitates the oral feeding but sometimes a gastrostomy will be necessary.

Dental anomalies require individualized treatment.

30 to 50% of children with Pierre Robin Syndrome have Stickler syndrome, an AD condition that includes other findings such as early arthritis & ocular problems.