What is Robin Sequence?
Robin sequence (RS) (pronounced Ro-BEN), also called Robin syndrome or Pierre Robin syndrome, is a craniofacial condition that affects the jaw, the tongue, the airway, and the roof of the mouth.

At 7-10 weeks of pregnancy, the lower jaw usually grows quickly, leaving a space at the bottom of the mouth for the tongue. Robin sequence starts when the lower jaw remains relatively small (called micrognathia), pushing the tongue to the roof of the mouth and back toward the throat (called glossoptosis). In this position, the tongue can block the roof of the mouth (the palate), possibly causing a hole called a cleft palate. In this position, the tongue can also cause problems with breathing after birth. This sequencing of events is why the condition is referred to as Robin sequence.

Could a child born with Robin Sequence have other health problems?
Most babies born with Robin sequence have no other health problems. This form is called isolated. Some babies have other health issues, such as Stickler syndrome or 22q Deletion syndrome. It is important that babies born with RS be evaluated by a team of professionals who can make a diagnosis.

How common is Robin Sequence?
RS occurs once in 8,000 births in isolated form and once in 14,000 births as part of other syndromes. In comparison, cleft lip and/or palate occurs about once in every 600 births.

What health issues can be expected with RS?
Robin sequence varies from person to person. One person may have severe problems related to the jaw, tongue, airway, and palate. Another may experience some but not all of those issues. Occasionally, a person born with Robin sequence does not have a cleft palate.

There are a few health issues commonly related to RS:

• The small jaw associated with RS can cause problems with breathing. Because the tongue rests at the back of the mouth, close to the throat, it can fall back and block the airway.
• RS can also affect eating. A baby with a cleft palate is unable to seal off the mouth from the nose. Because air and milk leak out of the open space, the baby is unable to breastfeed or drink from a regular baby bottle.
• A child born with RS can have problems with hearing. If a child has a cleft palate, fluid may build up behind the eardrum, causing short-term hearing loss (if a child has a syndrome or another medical condition, hearing loss may be permanent). As a baby grows, any hearing loss can affect speech and language. A cleft palate can affect speech in other ways, too, even after it is repaired during infancy.

Who treats Robin Sequence?
The issues linked with Robin sequence are complex but treatable. A child born with the condition needs to receive care from many types of professionals. It is important to find a cleft palate or craniofacial team, a group of professionals who work together to evaluate a child’s needs and plan their care.

A cleft palate team includes a surgeon, a speech-language pathologist, an orthodontist, and access to other specialists. A craniofacial team treats patients with conditions beyond cleft lip and palate; it includes a craniofacial surgeon and access to a psychologist and other specialists. Both types of teams treat patients with Robin sequence. To find a list of ACPA-approved team, look here.

What is the treatment timeline for Robin Sequence?
The treatment for Robin sequence varies from child to child, depending on symptoms and growth, among other factors.

The most important issues to treat during infancy relate to breathing and feeding (see details, below). Late in infancy, a child born with a cleft palate will need surgery to close it. The cleft team will also recommend an evaluation for hearing and possible placement of hearing tubes in the eardrum. In the years that follow, a child will be evaluated by a speech-language pathologist and will possibly receive speech therapy.

Treatment usually begins after birth and can last through childhood and into the teen years. It is critical that a child with RS be treated by a cleft palate or craniofacial team.

How are jaw and breathing issues assessed?
During infancy and early childhood, most children with Robin sequence have some trouble breathing. Early on, a baby needs a full exam. Members of the child’s team may do x-rays, CT scans, a sleep study, and a scope of the airway (an exam performed with a small camera to check for specific problems). The team may also examine how a child swallows.

How are jaw and breathing issues treated during infancy?
The airway can be managed in several ways during infancy, depending on a baby’s needs: through physical positioning, with soft tubes placed in the nostrils, with surgery, or with observation.

Positioning: When a baby is born with RS, members of the cleft team often ask caregivers to take special precautions at home during the baby’s sleep time. Babies born with RS need to sleep on their stomach (prone). Prone positioning lets the tongue fall forward, opening the airway. An adult needs to monitor the baby to make sure the airway stays open and the baby is breathing comfortably.

Nasal Tubes: Some cleft teams recommend that babies born with RS use soft, flexible tubes called nasopharyngeal tubes (also referred to as a nasal trumpet, nasopharyngeal airways, or NPA). These tubes are inserted into the nostrils to help with breathing.

Surgery: If breathing issues are severe, members of the cleft team may recommend surgery. About one in three babies born with RS needs surgery during their first year. The goal of surgery is usually to open the airway by pulling the tongue forward in the mouth. A surgeon either stitches the bottom side of the tongue to the lower lip, called a tongue-lip adhesion, or lengthens the lower jaw, called mandibular distraction.

In rare cases, if surgery does not do enough to help with breathing, the team may suggest a tracheostomy, a procedure to place a breathing tube directly into the windpipe through the neck.

Observation: For some children born with RS, jaw issues self-correct with time. A small lower jaw may grow quickly during early childhood, reaching normal size by about age six. Children who do not experience this growth may need surgery later in childhood to increase the size of the jaw.

How are sleep issues treated for Robin Sequence?
Some children born with RS have sleep apnea during childhood or the teen years. Sleep apnea is a disorder in which breathing stops and starts repeatedly during sleep. The team may ask caregivers to look and listen for signs of apnea, such as snoring and waiting for long periods between breaths. Sleep apnea can be treated with a breathing machine called a CPAP, or in some cases with surgery to enlarge the jaw.

How can I feed my baby born with Robin Sequence?
Babies born with a cleft palate are able to have skin-to-skin time with caregivers but are unable to form the suction necessary to breastfeed or draw milk from a regular baby bottle. Babies born with cleft palate should drink formula or pumped breast milk from a special bottle.

Caregivers often need help feeding a baby with RS. Right after birth, a feeding specialist on a child’s team meets with families to show caregivers how to use a special bottle, how to hold the baby while feeding, and how to read the baby’s signals. Most babies born with RS feed successfully with one of these bottles. They usually need to be held upright for feeding or in a side-lying position.

While feeding a child with RS can be challenging, in most cases it gets better with time and special care. A specialist on the cleft team can help families with bottle feeding, and later on, with solid foods.

Will my baby need a feeding tube?
In rare cases, when a baby can’t get enough nutrition from a special bottle, a member of the team may suggest a feeding tube. Infants with feeding tubes should continue to see the feeding specialist for as long as the tube is in place. In certain cases, such as for children with tracheostomies, a feeding plan may be more involved.

Sometimes, a baby with RS will stop wanting to eat or drink. This is called oral aversion. It can happen after a baby has had a lot of medical interventions or is tube-fed. It is important for tube-fed babies to suck on a pacifier during their tube feeds so that they can learn that they need to suck to eat and so they can practice oral skills. The pacifier (or another kind of non-nutritive sucking) has other benefits. It can calm a baby. It also keeps the tongue and jaw in a good position for breathing.

What should be done for a tongue-tie?
Some babies with RS are born with a tongue-tie, a short band of tissue that connects the tongue to the bottom of the mouth (any baby can be born with a tongue-tie). While doctors typically recommend cutting a tongue-tie for a baby without RS in order to help with feeding, cleft teams usually recommend keeping it in place for a baby with RS. A tongue-tie can help keep a baby’s tongue in an optimal position, away from the airway. Eventually, a tongue-tie can be cut, but the judgment should be made by a surgeon who regularly treats children with this condition.

How is a cleft palate treated?
Palate-repair surgery is a surgical procedure to close a cleft palate in order to help a child with eating and speech. As a baby grows, the team will decide on the best time for this operation. It typically takes place between ages 9 and 18 months.

How are hearing and speech issues treated?
All babies born in the U.S. undergo a hearing test at birth. Babies born with RS will need follow-up care by members of a cleft palate or craniofacial team. An audiologist will perform hearing tests as a baby grows. A pediatrician and an ENT surgeon will check for ear problems and treat temporary hearing loss.

If a baby is having problems with hearing and/or frequent ear infections, the ENT on the team may insert tubes into the eardrum at the time of cleft lip or palate repair. Tubes can treat the temporary hearing loss until the palate begins to function properly.

Because children born with RS can have problems with speech, they need to be seen by a speech-language pathologist, an expert who assesses, diagnoses, treats, and helps prevent problems with communication and swallowing. The speech pathologist may suggest speech therapy or other treatment.

Will future children be affected by Robin Sequence?
Many parents wonder if RS will occur in future pregnancies. People born with the condition may wonder whether it could happen for their future biological children.

The chances of RS happening again depend in part on how it was caused. RS can be caused by genes, by an event during pregnancy, or both. A geneticist is an expert on how characteristics are passed down in biological families (inherited). The geneticist on a child’s team can do blood tests to learn about a person’s genes.

Caused by genes: Sometimes, genetic tests show a chromosomal change or genetic mutation in a patient born with RS, which may indicate the presence of a genetic syndrome in that person (some examples of related syndromes include Stickler syndrome, 22q11 deletion syndrome, and Treacher Collins syndrome). Knowing about a syndrome affects a person’s treatment. It also tells the geneticist and family about the chances of RS happening again.

Parents who have had a child with isolated RS (without a syndrome) probably have a higher risk over the general population of having another child with this condition. This is because they may have genetic characteristics related to RS that they could pass on to another child.

While Robin sequence can be passed on from a parent to a child (inherited), it can also occur with no family history. Scientists do not yet know the likelihood of this occurrence.

Caused by an environmental event: Sometimes, a person is born with RS because of an event during pregnancy. This manifestation is called environmentally induced. Knowing certain information about that pregnancy can help the geneticist predict the chances of the condition happening again.

It is very important for people born with RS and their biological relatives to see a geneticist. A geneticist can help families learn about future pregnancies and find out if a person has a syndrome that needs medical care now.

Citations:

American Cleft Palate Craniofacial Association. Pierre Robin Sequence. ACPAcares.org. Accessed September 1, 2024. https://acpacares.org/resource-center/.

Atanelov Z, Aina T, Smith T, et al. Nasopharyngeal Airway. [Updated 2024 Jan 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK513220/

Blanc F, Harrewijn I, Duflos C, Maggiulli F, Captier G. Nasopharyngeal Tube and Functional Treatment in Pierre Robin Sequence: A Tertiary Clinical Experience From 150 Cases. Cleft Palate Craniofac J. 2022;59(7):891-898. doi:10.1177/10556656211031105

Butterworth S, Fitzsimons KJ, Britton L, et al. Investigating the Impact of Additional Congenital Malformations on Speech Outcomes at age Five in Children  with a Cleft Palate. The Cleft Palate Craniofacial Journal. 2024;0(0). doi:10.1177/10556656241287759

Collins B, Powitzky R, Robledo C, Rose C, Glade R. Airway Management in Pierre Robin Sequence: Patterns of Practice. The Cleft Palate Craniofacial Journal. 2014;51(3):283-289. doi:10.1597/12-214

Gasparin M, Barth FL, Schweiger C, Collares MVM, Levy DS, Marostica PJC. Sensory-Motor-Oral Stimulation Combined with Early Sucking During the Mandibular Distraction Osteogenesis Process in Children with Robin Sequence. The Cleft Palate Craniofacial Journal. 2024;0(0). doi:10.1177/10556656241264710

Kirschner RE, Low DW, Randall P, et al. Surgical airway management in Pierre Robin sequence: is there a role for tongue-lip adhesion?. Cleft Palate Craniofac J. 2003;40(1):13-18. doi:10.1597/1545-1569_2003_040_0013_samipr_2.0.co_2

Page reviewed by Cassi Smola, MD; ACPA Family Resources Committee