Overview

What Is Pierre Robin Sequence?

What Is Pierre Robin Sequence?

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Pierre Robin Sequence (PRS) is a disorder where the lower jaw (mandible) is smaller than normal and the tongue is pushed back. This causes breathing and swallowing problems. Most babies with this problem also have a cleft palate.

Some babies have severe breathing problems and need to have a breathing tube placed to allow them to breathe safely. Other babies have less severe breathing problems or mostly swallowing problems. Unfortunately, doctors who aren’t as familiar with PRS sometimes overlook babies with less severe breathing problems.

What Causes Pierre Robin Sequence?

The first thing to know is that you didn't do anything wrong if your baby has PRS. Like many medical problems, PRS is caused by random genetic mutations that happen when babies develop in the womb.

A small lower jaw (also called mandible) causes PRS problems. Some children with PRS have other medical problems. Other children with PRS only have the problems caused by having a small jaw. About one third of children with a small jaw have a condition called Stickler Syndrome, which causes vision problems.

For this reason, doctors recommend that all babies with PRS have an appointment with a pediatric ophthalmologist before they are one year old. Sometimes doctors will recommend that children with PRS have an appointment with genetics doctors. Understanding why your child has PRS can help can help doctors spot additional medical problems. It can also help give parents peace of mind.

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How Does PRS Affect Families?

Babies with PRS can place a large strain on families. From breathing problems, to challenging feeding, to fear of severe breathing episodes, the impact this disorder places on families can be considerable.

Understanding the strain on your family is important and plays a role in deciding the best treatment options. One of our surgeons, Dr. Skirko, is in the process of developing a quality of life instrument to help measure and understand this impact.

Treatments

Some babies can improve their symptoms with positioning. Positioning is when you hold and place your baby in special ways that will help her breathe better. Parents shouldn’t place babies with PRS on their back, for example.

Positioning can help prevent your child’s tongue from falling backwards and blocking her throat. But parents must carefully watch their children.

Some babies need a tube placed in their nose to help keep their airway open. Placing a tube in your child’s nose is less invasive than surgery. But doctors who place the tube may need intensive training, and babies with tubes will need to be monitored closely.

Pierre Robin Sequence & Mandible Distraction Osteogenesis

Babies with severe cases of PRS often need surgery to correct their abnormal jawbone. This type of surgery is called mandible distraction osteogenesis. In this procedure, surgeons work to slowly stretch and lengthen your child’s jawbone. Your child can then grow new bone while their jaw is being lengthened.

In rare cases, some children with PRS may need a tracheostomy tube. A tracheostomy tube is a tube that doctors insert in your child’s windpipe to help her breathe better. If your child has PRS and has a tracheostomy, our surgeons can evaluate your child to see if she is eligible for mandible distraction surgery.

Monitoring Children With PRS

Children with PRS children often have surgery to fix their cleft palate repaired when they are about one year old. If they have a very wide cleft, they may need a staged palate repair. They also need monitoring for speech problems.

Children with PRS need to be watched carefully as they grow to make sure their upper and lower jawbones grow normally. In some cases, children may need other surgeries down the road. Your surgeon can discuss the best way to monitor your child.

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