Pierre Robin Sequence

 

What is Pierre Robin Sequence/Complex?

Pierre Robin Sequence or Complex is the name given to a birth condition that involves the lower jaw being either small in size (micrognathia) or set back from the upper jaw (retrognathia).  As a result, the tongue tends to be displaced back towards the throat, where it can fall back and obstruct the airway.  Most infants, but not all, will also have a cleft palate.

What causes Pierre Robin Sequence?

• The basic cause is the failure of the lower jaw to develop normally before birth. At about 7-10 weeks into a pregnancy, the lower jaw grows rapidly, allowing the tongue to descend from between the two halves of the palate.  If, for some reason, the lower jaw does not grow properly, the tongue can prevent the palate from closing, resulting in a cleft palate.
• The small or displaced lower jaw also causes the tongue to be positioned at the back of the mouth, causing breathing difficulty at birth. This “sequencing” of events is the reason why the condition has been classified as a deformation sequence. For some patients, these physical characteristics may result from another syndrome or chromosomal condition, however.

How common is this condition?

Robin Sequence is uncommon. Frequency estimates range from 1 in 2,000 to 30,000 births, based on how strictly the condition is defined. In contrast, cleft lip and/or palate occurs once in every 700 live births.

What problems can be expected with this condition?

• Robin Sequence varies in severity from child to child. Some children may have more problems than others. Problems with breathing and feeding in early infancy are the most common. Parents need to know how to position the infant in order to minimize problems (i.e. not placing the infant on his or her back).  
• For severely affected children, positioning alone may not be sufficient, and the pediatrician may recommend specifically-designed devices to protect the airway and facilitate feeding. Some children who have severe breathing problems may require a surgical procedure to make satisfactory breathing possible.
• The pediatrician and ear, nose, and throat specialist will also carefully monitor the baby for ear disease. Virtually all children with cleft palate are prone to build-up of fluid behind the eardrum. The placement of ventilation tubes in the eardrums may be recommended to reduce fluid build-up. Since ear infections can cause temporary hearing loss that can affect speech and language development, the infant’s hearing should also be monitored from early infancy by an audiologist.

How is Pierre Robin Sequence treated?

In many patients, the lower jaw grows rapidly during the first year of life. In some children, the jaw may grow so quickly that by the time the child is 4 to 6 years of age, the profile looks normal. Children who do not experience this “catch-up” growth may require surgery on their jaws.  The cleft palate, if present, needs to be surgically closed.  The timing of the surgery depends on the child’s growth and development, but ideally is performed before 12 months of age and prior to speech development. Because children with cleft palate are at higher risk for delayed or defective speech development, they should be monitored by a speech pathologist throughout early childhood.