Pierre Robin Sequence
Pierre Robin Sequence, also known as Pierre Robin syndrome or malformation, is a rare congenital birth defect. It is characterized by an underdeveloped jaw, cleft palate, a tongue that falls back into the throat, and airway obstruction. The pediatric plastic surgery team at University Hospitals Rainbow Babies & Children’s has experience and expertise in treating this disorder and other congenital craniofacial defects.
Why Choose UH?
Coordinated care is especially important for children with Pierre Robin syndrome because of the multiple issues that can arise. At UH Rainbow Babies & Children’s, a team of specialists work together to treat children with Pierre Robin sequence and to address the various problems that the birth defect can cause. These include breathing, hearing, feeding and sleeping issues.
All clinicians work closely together to manage your child’s condition. Pediatric specialty areas that may become involved in your child’s care include:
- Otolaryngology (ENT)
- Plastic surgery
- Speech therapy
The craniofacial specialists at UH Rainbow Babies & Children’s are at the forefront of researching and developing innovative therapies for conditions such as Pierre Robin sequence. Through the use of leading-edge surgical techniques and technologies, our team is able to provide the most advanced care for children with Pierre Robin sequence.
Diagnosis and Treatment
Pierre Robin sequence is usually diagnosed during a physical exam at birth. If your child has Pierre Robin sequence, you can expect treatment to come in stages. Since the condition affects a variety of functions including hearing, breathing and feeding, several specialists will be involved in your child’s care.
Traditional treatments for the disorder involve the use of breathing and feeding tubes and bone grafting procedures. These treatments can pose some risks; however, these risks can be reduced by manipulating the jawbone and inserting a device to gradually move it forward, along with the tongue. The device can be adjusted and removed once the patient’s condition has improved and the airway is no longer obstructed.
In more mild cases, management may consist of positioning your child in a side or face-down position to allow the tongue to fall forward and relieve the obstruction.
Jaw size, tongue placement and cleft palate can contribute to difficulties feeding a child with Pierre Robin sequence. Infants with a mild form of the condition can learn to feed using specially adapted nipples and bottles. Cleft palate associated with Pierre Robin sequence is typically repaired through surgery before age 2.
Parents and doctors should continue to monitor child as they develop, paying attention to things such as jaw and tooth development and speech.