Pierre Robin Sequence

Posted on May 7th, 2007

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Pierre Robin Sequence (sometimes referred to as Pierre Robin Syndrome) is a common variation of simple cleft palate. The baby has a combination of cleft palate, a small underdeveloped lower jaw (retrognathia) and a falling of the tongue into the back of the mouth (glossoptosis). This never occurs in combination with cleft lip and is never inherited or passed on.

The incidence is one child in every 30,000. The milder form of the sequence is relatively common whereas the more developed form is quite rare where the baby may have some problems breathing due to the tongue partially obstructing breathing because of its position at the back of the mouth.

It is commonly accepted that the cause of this sequence is that the baby has been cramped in the womb with the head pressed more firmly onto the chest than usual. This physically holds back the lower jaw growth and pushes the tongue up into the roof of the mouth, stopping the natural closure of the palate in the early days of development.

This would explain the fact that almost all the babies’ jaws catch up in growth over the first year of life and that this is not an inherited condition.

Repair of the cleft palate is often carried out at 9 or 12 months of age when the jaw has grown forward. This is to diminish any chance that if the operation is carried out earlier it might push the tongue back further into the back of the throat bringing on breathing problems.

A small number of babies who have breathing problems at birth because the jaw is so small may need nursing on their face in a special frame for some time while the jaw develops and normal breathing is restored.

It is important that babies with breathing problems are seen by a paediatrician and a plastic surgeon.

Speech and Feeding

The child may see a speech and language therapist in the early stages if there are feeding difficulties. Some Pierre Robin babies have difficulty in getting their feeding established due to their cleft and small mandible. If this is not the case, parents should ensure that the child is taken to the speech and language therapist at about the age of 8 months. The therapist will not be able to do much direct work with the child at this stage but can advise parents on how best to develop communication directly. A referral to a speech therapist is not always automatic and often the parent will have to request one.

Hearing

Often a child with Pierre Robin Sequence will have a hearing problem which can have an effect on speech and language development. It is important to have the child assessed for hearing loss at an early age so that the appropriate treatment can be carried out.

With the help of the plastic surgeon, paediatrician, speech therapist, audiologist, ENT surgeon and the dentist, the child with Pierre Robin Sequence can look forward to a bright future.

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