Pierre Robin sequence is the combination of a small lower jaw, a tongue that seems too large for the mouth because the jaw is so small, and breathing difficulty. These babies have trouble breathing because the lower jaw is positioned too far back, causing the tongue to block the airway. About half of these babies also have a cleft palate.
Is this condition painful?
No, this condition is not painful, but some babies may have severe breathing difficulty.
Are there different types of Pierre Robin sequence?
There is a spectrum of severity with this condition. Some babies may be more severely affected than others.
Who gets Pierre Robin sequence?
Any baby can be born with Pierre Robin sequence. There may be many different causes, and it may be inherited in some cases.
What causes Pierre Robin sequence?
It is thought that abnormal position of the baby in the womb, with pressure on the developing lower jaw, may cause this condition. In addition, there may be genetic influences involved.
What are the main issues related to Pierre Robin sequence?
Babies with Pierre Robin sequence have problems with breathing and feeding, both due to the small lower jaw. In most babies, the breathing problems can be managed with prone positioning, that is, keeping the baby face down so that the tongue does not fall back and block the airway. However, when the jaw is extremely small and breathing is very difficult, the child may not be able to feed adequately and expends all of his energy in trying to breathe. In these cases, surgery is required to prevent failure to thrive or even death.
Are there other problems that occur commonly with Pierre Robin sequence?
About half of the children with Pierre Robin sequence have cleft palates. The palate is usually repaired at about 18 months of age.
What is the treatment for children with Pierre Robin sequence?
While every patient is treated individually, with treatment plans made specifically for him or her, some generalizations are possible. If the deformity is mild and breathing can be managed with prone positioning, no surgery is required. However, if the deformity is severe and breathing is a problem, surgical treatment is necessary. Traditionally, a tracheostomy would be done; this is an operation that creates an opening into the windpipe in the neck that bypasses the obstruction of the airway at the level of the tongue. When the child is older and the jaw has grown forward, the tracheostomy tube can be removed. However, there are two other surgical options that might be appropriate in some cases and could eliminate the need for a tracheostomy. Tongue-lip adhesion is a temporary attachment of the tongue to the lower lip; this pulls the tongue forward and can relieve the airway obstruction. The most recent surgical advance that has been used in these cases is distraction osteogenesis. Distraction osteogenesis is a technique by which the lower jaw can be gradually moved forward over several days time, using a device that is surgically attached to the jaw. As the jaw moves forward, the tongue also moves forward, and the airway obstruction is relieved. These treatments would be done very early in life, sometimes within the first week.
What happens after my baby is born with Pierre Robin sequence?
These babies are usually observed in the Neonatal Intensive Care Unit initially. The parents of babies who are able to breathe and feed adequately are instructed on prone positioning and feeding techniques. When the baby is doing well, he or she is transferred to the ward and then goes home with the parents.
Babies who have more difficulty with breathing and feeding stay in the Intensive Care Unit and may have a breathing tube placed and their breathing assisted by a ventilator. This is usually temporary until the appropriate surgical treatment is determined. Special x-rays and computerized tomography (CT) scans may be done as part of the evaluation. An examination of the airway is usually performed by the otolaryngologist and, in some children, a sleep study is performed to see how severe the breathing difficulty is during sleep. When all this information has been analyzed, a decision is made by the treatment team about the appropriate surgical procedure.
What sorts of specialists will be involved in my child’s care?
The optimal treatment of children with Pierre Robin sequence is achieved in a multidisciplinary setting. Usually, the specialists involved include the neonatologist, craniofacial surgeon (a plastic surgeon who has done additional specialized training), an otolaryngologist (ENT doctor), a pediatric anesthesiologist, a developmental pediatrician, a geneticist, and the pediatric plastic surgery nurse.
Will we get to know our surgeon?
Babies with Pierre Robin sequence who require surgery often have this done soon after birth. Things may seem overwhelming for you because events may proceed fairly rapidly. However, your surgeon will sit down with you and very carefully explain everything that is being done, and the reasons behind each test and procedure. You will be able to ask questions and have everything explained thoroughly to you. Ideally, if the diagnosis is made prenatally, you will have had the chance to meet your surgeon before the delivery; the information gained from a prenatal consult can help prepare you for what may happen after your baby is born. Happily, after surgical treatment, most babies do well and have no further problems. Babies with a cleft palate will require another surgery to repair the palate. All of these children are followed by their surgeon over the long-term, to carefully assess their growth and development.