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Pediatric Cleft and Craniofacial Center
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 fall back towards the throat, and obstruct the airway (glossoptosis). Most infants will also have a cleft palate.
What causes Pierre Robin Sequence/Complex?
The basic cause appears to be the failure of the lower jaw to develop normally before birth. At about 7-10 weeks of pregnancy, the lower jaw develops rapidly, allowing the tongue to fall from 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, possibly causing breathing difficulty. For some patients, these physical characteristics may result from another syndrome or chromosomal condition.
What problems can be expected with Pierre ?
Robin Sequence/Complex, like most birth defects, varies in severity from child to child. Problems with breathing and feeding in early infancy are the most common. Parents will be taught how to position the infant in order to minimize problems. Most infants are placed on their stomach to facilitate adequate breathing. For severely affected children, position changes may not be enough. Some infants require a tracheostomy and/or a g-tube to protect their airway and facilitate feeding.
How is the condition 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 approximately four to six years of age, their small jaw appears normal. Children who do not experience this "catch-up" growth may require surgery on their jaws. If a child is born with a cleft palate this will be surgically repaired at approximately 12-18 months depending on the child’s health and growth. Because children with cleft palate are at higher risk for delayed or abnormal speech development, they should be monitored by a speech pathologist throughout early childhood. They will also be followed by our Cleft and Craniofacial Team on an annual basis to monitor their growth and development.