10/07/2012
Many people often ask me what Pierre Robin Sequence is. I realized that I spend so much time raising awareness about other childhood illnesses yet I have never really said much about what this is that my son has. I have found that explaining it is so difficult because there are so many factors involved that are hard to understand let alone explain. It is pronounced Pierre (like the name) Ro-ban Sequence. It took me about 3 months until I realized I was saying it wrong the whole time.
This definition came from wikipedia:
PRS is characterized by an unusually small mandible (micrognathia), posterior displacement or retraction of the tongue (glossoptosis), and upper airway obstruction. Incomplete closure of the roof of the mouth (cleft palate) is present in the majority of patients, and is commonly U-shaped. It is not known how this abnormality occurs in infants, but one theory is that, at some time during the stage of the formation of the bones of the fetus, the tip of the jaw (mandible) becomes 'stuck' in the point where each of the collar bones (clavicle) meet (the sternum), effectively preventing the jaw bones from growing. It is thought that, at about 12 to 14 weeks gestation, when the fetus begins to move, the movement of the head causes the jaw to "pop out' of the collar bones. From this time on, the jaw of the fetus grows as it would normally, with the result that, when born, the jaw of the baby is much smaller (micrognathia) than it would have been with normal development, although it does continue to grow at a normal rate until the child reaches maturity.