Pierre Robin Syndrome

My eight-year-old son was born with Pierre Robin Syndrome. He had the cleft of the soft palate repaired at 10 months. He has had an extra tooth removed from the roof of his mouth. He is just starting to loose teeth (his siblings were late losers also). The loose tooth he has now is not loosening up because of the crowding of the teeth around it; it is starting to stick straight out. Should we wait for it to come out or take him in and have it extracted? Also, is it time for him to start some orthodontics? He has only lost two lower teeth at this time.


Although I suspect that you are familiar with Pierre Robin Syndrome, I will provide a brief overview. When a child is born with micrognathia (abnormal smallness of one or both jaws), glossoptosis (tongue obstruction), and a high- arched or cleft palate, this is called Pierre Robin Syndrome. These conditions can be found as an isolated compound or can be associated with other syndromes or other developmental anomalies. The smallness of the lower jaw and its retrognathic ("backward") position are usually the primary conditions associated with this syndrome.

Pierre Robin Syndrome occurs in about 5 to 22 babies per every 100,000 births. One hypothesis about the development of this syndrome is that the arrested growth of the lower jaw prevents the tongue from dropping down into its normal position. The tongue then interferes with the closure of the palate during palatal formation. More recent evidence suggests that genetic influences may cause the growth disturbances in the upper and lower jaws.

In the early stages of life, respiration and feeding can be problematic; therefore, careful supervision may be needed to prevent airway obstruction, esophageal reflux, pneumonia, and exhaustion. After the first few months of life, growth of the lower jaw and better control of the tongue allow for a significant reduction in these type of effects. In fact, the growth of the lower jaw can be quite extraordinary in the first four years of life, and a normal jaw profile can be seen between the ages of four and six years old. Some patients will require orthodontic treatment for improved occlusion (relationship of upper and lower jaws and teeth) and esthetics.

For your son, I recommend having his dentist evaluate the loose tooth. It may need to be extracted to enhance comfort, esthetics, and/or function. Ask your dentist for a referral to an orthodontist. A consultation with an orthodontist now may reveal the need for treatment at the present time. Otherwise, you will at least get an idea from the orthodontist about when to proceed. I do not think your eight year old son is too young to begin consulting with an orthodontist.

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