Pierre Robin’s sequence
Overview
Pierre Robin syndrome is a condition present at birth in which an infant has a very small lower jaw, a tongue that tends to fall back and downward, and a soft cleft palate. The syndrome is also called Pierre Robin complex or sequence.
Symptoms
* Cleft soft palate * High-arched palate * Jaw that is very small with significant receding chin * Jaw placed unusually far back in the throat * Large-appearing tongue in relation to jaw * Natal teeth (teeth appearing when the baby is born) * Recurrent ear infections * Small opening in the roof of the mouth, which causes choking
Causes
The specific causes of Pierre Robin syndrome are unknown. It may be part of many genetic syndromes. The lower jaw develops slowly over the first few months of life before birth, but speeds up during the first year after birth. The falling back of the tongue may cause choking episodes and feeding and breathing difficulties, especially when the child sleeps.
Prevention
There is no known prevention. Treatment may reduce the number of episodes of breathing problems and choking.
Diagnosis
Prenatal ultrasonic examination may show findings to indicate the possibility of Pierre-Robin sequence alerting the physician to be prepared at birth for the possibility of the baby having breathing and feeding problems.
Prognosis
The prognosis for individuals with Pierre-Robin sequence varies with the severity of symptoms and if it is associated with other congenital abnormalities. The more severe the symptoms and associated congenital abnormalities, the greater the risk of complications. The rate at which the lower jaw starts to catch up in growth depends on the cause of Pierre-Robin sequence. The majority of children with the isolated type will achieve near normal jaw size within a few years of birth. If Pierre-Robin sequence is part of a syndrome that has a small jaw, the jaw may remain small throughout life.
Treatment
Infants with this condition should NOT be put on their back, so that the tongue does not fall back into the airway. Problems associated with this syndrome tend to get better over the first few years as the lower jaw grows to a more normal size. In moderate cases, the patient requires placement of a tube through the nose and into the airways to avoid airways blockage. In severe cases, surgery is needed to prevent upper airways obstruction. A tracheostomy (surgery to make a hole in the windpipe) is sometimes required. Feeding must be done very carefully to avoid choking and breathing liquids into the airways.