A lower jaw smaller than normal, a tongue that falls back and a palate that is not closed, the Pierre Robin sequence is a rare malformation that affects 80 to 100 babies each year in France.

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Characteristics of Pierre Robin syndrome

From the second month of pregnancy, the face of an embryo has two buds that later form the nostrils and the mouth. The lower jaw is small, the tongue is upright in the mouth, and the palate is not finished.

After a few weeks, in some fetuses, the jaw does not develop properly. It does not grow or move normally and this causes the chin to retract, which itself will force the tongue to drop backwards and therefore to remain in an upright position. This is what will prevent the proper closure of the palate.

This domino effect is called the Pierre Robin Sequence. It is the name of a surgeon who described this disease in the 1930s. Today, doctors believe that the origin may be bone or neurological.

In utero, the fetus does not acquire either the swallowing reflex or the sucking reflex. At birth, the child therefore has difficulty feeding, but may also have breathing difficulties and gastroesophageal reflux.

A few months after birth, however, it is possible to correct this poor closure of the palate with a surgical operation.

Pierre Robin sequence: operating the palace

Mila is 11 months old, was born without a palate and is going to be operated on.

A new life after the operation

After the operation, food cannot go back up the baby’s nose, which could prevent him from eating his whole meal. The child will also be able to learn to speak without difficulty, and will not have a nasal voice, one of the risks of this pathology.

Over the months, the lower jaw will grow naturally and the chin will therefore come forward. Coline, one year old, was operated on two months ago. Our teams met her parents, very happy to see their daughter’s progress on a daily basis.

How to have your child followed?

Regular multidisciplinary medical follow-up is necessary for the good development of the child. Until adulthood, he is followed by a pediatrician, a speech therapist and an orthodontist.

For more information on Pierre Robin Sequence isolated or associated with other pathologies, the “Tremplin” association has been advising families affected by this disease for more than 20 years.

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