Pierre Robin Sequence

About Pierre Robin Sequence

Pierre Robin Sequence is named after a French physician who identified the main features of the condition.

  • Micrognathia - a small lower jaw
  • Glossoptosis - a tendency for the base of the tongue to ball up and fall backwards into the throat causing obstruction and therefore breathing difficulties.
  • Cleft Palate - A cleft palate or a high arched palate without a cleft may be present.

The jaw bone continues to grow during childhood and usually fully correct by adult life. The condition is rare. Estimates range from 1 in 8,000 to 30,000.

It is generally thought that this is a sequence of events arising from the haw being compressed which then leads to the tongue being projected upwards and this in turn interferes with the closure of the palate. However, it is not fully known why the condition occurs. Maternal virus in the early stage of pregnancy and folic acid deficiency are other areas than have been researched, but nothing conclusive has been determined. Pierre Robin Sequence usually occurs in isolation, but it can also feature in other syndromes/conditions that have genetic links, such as Sticklers Syndrome. Careful investigation is therefore required by experienced doctors to ensure that a correct diagnosis and appropriate care is given to babies born with Pierre Robin Sequence.

All babies with Pierre Robin have some difficulties. Some have no problems with their breathing and minor feeding difficulties. Others have moderate difficulties, which require them to be in hospital until breathing and feeding patterns can be established. A smaller group of babies have great difficulties in both areas, which can persist for several months. These babies require assistance with breathing which might include the use of a nasal prong or a tracheotomy.

If a cleft palate is present, repair can take place anytime between 6 and 19 months of age, depending both on the severity of breathing difficulty and on the preferences of the plastic surgery.

 

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