Understanding Pierre Robin Sequence

Pierre Robin Sequence is thought to affect about one out of every 8500 babies, but not many people know about this condition.

Pierre Robin involves infants that are born with lower jaws that are smaller than normal, and this can lead to problems throughout the child’s life.

The Signs of Pierre Robin Sequence

Someone will be diagnosed with Pierre Robin Sequence when they experience the following birth defects that all appear together:

  • A small lower jaw accompanied by a receding chin
  • Breathing problems
  • A tongue that tends to back up in the rear of the mouth while falling back in the direction of the throat
  • A large tongue in comparison to the jaw
  • Natal teeth, or teeth that have already erupted at birth

In some cases, a horseshoe-shaped cleft palate may also be present. Recurring ear infections are also common.

Pierre Robin Sequence Causes

Pierre Robin is not well understood, so it is not known exactly what causes the sequence. Most experts don’t believe that it has anything to do with maternal behavior during pregnancy, but if a child is only born with Pierre Robin and no other congenital birth defects, the positioning of the fetus during the early weeks of pregnancy could be at play.
The lower jaw will grow from gestational weeks 7 to 10, and the growth experienced during this time is quite rapid. The tongue will descend from between the two portions of the palate, and if the lower jaw doesn’t grow properly, the tongue can remain high in the mouth longer than usual, preventing the palate from closing. The displacement of the tongue can also result in breathing difficulties.

Risks Associated with Pierre Robin Sequence

There are several unique risks associated with Pierre Robin Sequence that parents need to become aware of. Infants living with this condition should never be placed onto their backs in order to prevent the tongue from moving back into the airway. Babies may have problems with eating due to the position of the cleft palate and tongue, and in some cases, food may be aspirated into the lungs. In these cases, it may be necessary for parents to use specialized bottle nipples or to have a feeding tube placed into their child’s nose.

Like all children with a cleft palate, kids born with Pierre Robin will be at risk of developing a build-up of fluid behind the ear tubes. In these cases, ear tubes may be needed in order to drain the fluid. Without this intervention, temporary hearing loss may result and can interfere with language and speech development. If hearing problems or issues with the ears are identified, your child will need to have early hearing tests in order to better understand the scope of the problem.

Surgical Interventions

In some cases, surgery may be necessary in order to correct some of the symptoms of Pierre Robin Sequence, but this will depend entirely on the severity of the condition. Surgery may be needed to repair a cleft palate or to improve breathing, and specialized devices may be needed in order to aid in feeding and to protect the airway. In most cases, the small jaw will continue to grow on its own within the first years of life, but if this doesn’t happen, surgery may be needed.

Your child’s medical provider should be able to diagnose Pierre Robin Sequence with a simple physical examination. However, it may be beneficial to meet with a genetic counselor to rule out any other potential problems that could be linked to this syndrome. Early intervention can be a necessity when it comes to avoiding invasive treatment options in the future.

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