Robin Sequence

Our Craniofacial Genetics Clinic helps identify conditions caused by changes in genes. Our geneticists and genetic counselors help you understand the pros and cons of genetic testing. They explain test results.

A genetic counselor also will give you information about your child’s condition. Counseling can help you make informed decisions about family planning and your child’s treatment.

Robin sequence also has been called:

• Pierre Robin syndrome
• Pierre Robin sequence
• Pierre Robin malformation sequence
• Robin anomalad
• Pierre Robin complex

The first priority is keeping your baby’s upper airway open so they can breathe easily.

We will watch your child’s breathing and growth throughout childhood. It is common for babies with Robin sequence to breathe well right after being born and then to develop more airway obstruction in the early weeks after birth. While some children have breathing problems in their first months of life, others develop problems (like obstructive sleep apnea) later.

Your child may have these tests to help their care team understand the cause of breathing problems and decide on the best treatment:

• Blood tests to check for the right balance between oxygen and carbon dioxide (CO2).
• Looking inside your child’s windpipe using a flexible tube with a light and video camera (endoscope). The doctor inserts the tube through your baby’s nose.
• Monitoring your child’s breathing while they sleep to learn if they have obstructive sleep apnea. Learn about overnight sleep studies (PDF) (Arabic) (Simplified Chinese) (Somali) (Spanish) (Vietnamese).
• CT (computed tomography) scan of the facial bones and airway. This helps doctors understand the jaw, tongue and breathing space and decide if treatments like OAP and jaw surgery will help your child.

The best treatment depends on your child’s age and if they have breathing problems during sleep, during feeding or most of the time.

The options include:

• An OAP to hold your baby’s tongue in a safe position to keep their airway open. It stimulates their lower jaw to grow, which enlarges their airway over time. For many children, the improved jaw position avoids the need for jaw surgery. The OAP is like a retainer with an extension. It’s custom-made using a mold of your baby’s mouth. To be sure it fits exactly right, we use a CT (computed tomography) scan of your baby’s jaw and airway. This lets us try on the OAP virtually before fitting it in your baby’s mouth. Most babies stay in the hospital 2 to 4 weeks for OAP treatment. Your baby will wear their custom OAP at all times during treatment, usually 3 to 6 months. We will teach you to remove the plate for cleaning each day and put it back in place.
• Keeping your baby on their stomach (called prone positioning) when they sleep to prevent their tongue from falling back and blocking their upper airway.
• Providing oxygen via a nasal cannula to prevent oxygen levels from dropping.
• Placing a small tube through your baby’s nose into the upper airway to prevent the airway space from being blocked. This is called a nasopharyngeal airway.
• Surgery to make your child’s lower jaw (mandible) larger. This involves using a small metal device (distractor) to lengthen the jawbone over 2 weeks. As the lower jaw moves forward, the tongue moves forward, and the airway at the back of the throat opens up. The distractor stays in place for 2 more months while the bones heal in their new position. Read more about mandible distraction.
• Placing a breathing tube in the windpipe (tracheostomy) if breathing problems are severe and we cannot treat the problems without surgery.
• Giving medicine to prevent backup of stomach acid into the throat if gastroesophageal reflux is a problem for your baby.
• Using feeding tubes to avoid the stress of eating if breathing problems happen while your child eats.

As your baby grows, their breathing may improve because their tongue and jaw position change.

Older children with obstructive sleep apnea may benefit from wearing a mask while they sleep that keeps air flowing into their airway. The mask is connected to a continuous positive airway pressure (CPAP) device. Children younger than 4 years usually are too small or unwilling to use the mask while sleeping.

Once your baby is breathing comfortably, feeding is usually much easier.

Starting soon after birth, we regularly check your child’s weight and growth.

The tongue position and breathing problems caused by Robin sequence can make it hard for your child to suck, swallow and breathe. In babies treated with an OAP, the improved tongue position may help them feed from a bottle. Some babies may need a special bottle or nipple, or to be held in a certain way while feeding. We can show you helpful feeding techniques. Many babies with Robin sequence need added nutrition from a temporary feeding tube to help them grow.

If your baby has a cleft palate, they will not get enough suction to suck milk out of the breast or regular bottle. When they suck, the roof of their mouth will not close off the mouth from the nose. Babies with cleft palate usually need special bottles and nipples. We can help you with different types of bottles and finding the right feeding strategy for your baby.

Options for babies who need more than special bottles

If your baby is not able to get enough nutrition by mouth, they may need a tube through their nose to their stomach for the first few months of life. This is called a nasogastric feeding tube or NG tube.

If the feeding tube is needed for many months, the doctor may recommend using a tube through their abdominal wall into their stomach. This is called a gastrostomy tube.

Many babies with Robin sequence also have problems with:

• Backup of stomach acid into the throat. Medicine and changes to your child’s diet can help.
• Sucking liquid into the windpipe. We treat this with special feeding techniques, thickening milk and using a feeding tube. The feeding tube might go from the nose to the stomach (NG tube) or to the beginning of the small intestine (nasoduodenal or ND tube).

In children with Robin sequence that is not part of a syndrome (isolated), feeding is usually not a problem after your child is 1 year. As your child grows, they get stronger and their airway gets bigger. If Robin sequence is related to a syndrome, feeding may continue to be a challenge because of other problems.

• If your child has a gap in the roof of their mouth, they will need surgery to repair it. Usually this happens between 12 and 15 months, when a child is developing new speech sounds. Sometimes surgery happens later for children with significant breathing problems or other health issues.
• A surgeon closes the cleft and puts the muscles at the back of the roof of the mouth in the typical place across the cleft.
• Usually, surgery takes 3 hours, and your child will stay in the hospital 1 to 2 nights. Read more about treatments for cleft palate.
• Because cleft palate repair may affect your child’s airway, your team will check your child carefully before and after surgery for any sign of breathing problems.

We check your baby’s hearing throughout their childhood. Most children with cleft palate have fluid buildup behind their eardrum. This can make it harder to hear.

• Every 6 to 12 months, a specialist trained to test hearing in babies and children (audiologist) will check your child’s hearing.
• If there are problems, an ear, nose and throat doctor (otolaryngologist) will see your child.
• If fluid buildup is a problem for your child, we recommend putting small plastic tubes into their eardrums. These tympanostomy tubes keep the middle ear clear of fluid. We often do this at the same time as surgery to fix cleft palate.

Cleft palate can affect your child’s ability to talk. A speech and language pathologist (SLP) will regularly check your child’s ability to talk and how their language is developing. We start even before your child’s cleft palate is repaired, as early as 9 months.

Some children with cleft palate have a condition called velopharyngeal dysfunction (VPD). They may have trouble making correct speech sounds, even after the cleft is repaired.

Depending on your child’s needs, we may recommend:

• Speech therapy to develop more typical speech patterns
• A custom-made speech appliance called an obturator
• Surgery on the roof of the mouth or throat

Your child's surgeon and SLP will work together to recommend what is best for your child.

Teeth problems are more likely in children with Robin sequence than in other children. An orthodontist and dentist experienced in caring for children with craniofacial conditions can provide the treatment your child needs.

• Children with clefts are at higher risk for cavities. The hard coating (enamel) on their teeth may have weak areas that decay easily. Regular dental visits and careful tooth care, like daily toothbrushing, are important, starting with baby teeth. Your child should have their first visit with a pediatric dentist or family dentist by age 1.
• Teeth crowding may be a problem as your child’s permanent teeth come in. Some children need to have teeth pulled because there isn’t enough room for all of the adult teeth.
• Your child should have their first orthodontic evaluation at age 6, about the time their adult teeth start to appear. Many children with Robin sequence benefit from orthodontic treatment during their school age and teen years.
• As your child grows, the position of their upper and lower teeth gets better. This is partly because the lower jaw grows. It is also because in children with clefts, the upper jaw grows less than is typical.
• Sometimes when a child’s jaws are done growing, their lower jaw is still too small in relation to their upper jaw. Orthodontic treatment alone may not be enough to fix the problem. Your child may need surgery to make their lower jaw larger. Learn more about mandibular advancement.
• If your child also has a condition like Stickler syndrome, they may need surgery on their upper jaw.