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. Some children have breathing problems in their first months of life and others develop problems 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 your child will be helped by treatments like jaw surgery.

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

The options include:

• An orthodontic airway plate (OAP) to position your child’s tongue forward and keep their airway open. It stimulates the tongue to come forward and the lower jaw to grow, which also enlarges their airway. The special appliance is 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 to virtually try on the OAP 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 when they sleep to prevent their tongue from falling back and blocking their upper airway.
• Providing oxygen via 2 tiny prongs (nasal cannula) in their nose to prevent oxygen levels from dropping.
• Placing a small tube through your baby’s nose into the upper 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 is left 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.
• Giving medicine to prevent backup (reflux) 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.

Children with obstructive sleep apnea who are older may benefit from wearing a mask while they sleep that keeps air flowing into their airway. The mask is connected to a CPAP (continuous positive airway pressure) 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, such as an NG (nasogastric) tube.

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 understand the different types of bottles.

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, your 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 (cleft palate), they will need surgery to repair it. Usually this happens between 12 and 18 months, when a child is developing new speech sounds.
• During cleft palate surgery, the cleft is closed. Muscles at the back of the roof of the mouth are put in their proper place across the cleft.
• Most often, surgery takes 3 hours. Your child usually 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. It is often done 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 normal speech patterns.
• A custom-made speech appliance called an obturator.
• Surgery on the roof of the mouth or throat. Your surgeon and speech pathologist 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 are able to give your child the treatment they need.

• 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 are important, starting with baby teeth.
• 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 will likely need orthodontic treatment to align their teeth during the teen years.
• As your child grows, the position of their upper and lower teeth gets better. This is partly because of growth of the lower jaw. It is also because the upper jaw grows less than normal in children with clefts.
• Sometimes when a child’s jaws are finished growing, their lower jaw is still too small in relation to the 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.

Our experience with craniofacial conditions helps us find problems early and take steps to prevent or treat them. We care for 15 to 20 new babies with Robin sequence every year.

We have more surgeons specializing in cleft repair than any other center in the country. Read about Seattle Children’s expertise in craniofacial surgery.

Our center has experts in every field your child might need. These 50 specialists work together to diagnose and care for our patients.

Our team meets weekly to discuss children with complex needs and determine the best care plan for each child. We work together, and with you and your family’s doctor, to manage your child’s personalized care.

A craniofacial pediatrician, nurse, family service coordinator and social worker will work closely with your family. They coordinate your child’s care and make sure all your questions are answered.

Your pediatrician guides your child’s treatment and decides if other specialists are needed. These may include an ear, nose and throat doctor (otolaryngologist), craniofacial plastic surgeon, oral-maxillofacial surgeon, orthodontist, feeding therapist, dietitian, audiologist and speech and language pathologist (SLP).

We care for care for your child’s physical, emotional and comfort needs. Read more about the supportive care we offer.

We provide the treatment that is right for your child at the right time.

After your baby is born, we deal with any breathing problems right from the start. We measure your baby’s growth, teach you feeding techniques and take any needed steps to help your baby thrive. We regularly check your child’s vision and hearing and their speech and language development.

As your child grows, we do surgeries and provide other treatments to fix problems that affect their breathing, feeding, hearing, speech development and bite (occlusion).

A diagnosis of Robin sequence can be scary. We take time to explain your child’s condition. We help you understand your treatment options and make the choices that are right for your family.

As your child gets older, we make sure they are involved in decisions about their medical care.

Our child life specialists and social workers help your child and your family through the challenges of this condition. We connect you to community resources and support groups.

Seattle Children’s provides craniofacial care for children in an area one-fourth the size of the continental United States. We also care for children from across the globe. This experience has helped us develop systems to provide outstanding, personalized care close to home and at a great distance.

Seattle Children’s receives more research funding from the National Institutes of Health (NIH) than any other craniofacial center in the United States.

Our researchers are working to:

• Help children breathe and sleep better if they have airway problems caused by Robin sequence
• Ensure the best outcomes for children born with conditions related to clefts
• Understand more about what causes clefting and find ways to prevent it

Learn about Robin sequence research and cleft-related studies at Seattle Children’s.