Van der Woude Syndrome

Usually van der Woude syndrome happens because of changes in a gene that is important in forming tissues of the head and face. The gene is called IRF6 (interferon regulatory factor 6).

Sometimes the condition is caused by a new genetic change that was not passed on by a parent. Other times, a child inherits an abnormal gene from their mother or father.

We do not know why this happens. It is not because of anything that the parents do or do not do during pregnancy.

This syndrome is also called:

• Lip-pit syndrome
• Cleft lip and/or palate with mucous cysts of lower lip
• VDWS

Each year, we provide care for more than 300 children with complex conditions that involve clefts.

Cleft lip and palate is the most common problem we treat. We have more surgeons specializing in fixing clefts than any other center in the country. Read about Seattle Children’s expertise in craniofacial surgery.

Over the past 5 years, we have cared for 22 children with van der Woude syndrome.

The Craniofacial Center at Seattle Children’s 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 decide on 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 care and make sure all your questions are answered.

The pediatrician guides your child’s treatment and decides if other specialists are needed. Your child’s team may include a geneticist, genetic counselor, craniofacial plastic surgeon and ear, nose and throat specialist (otolaryngologist). As needed, your child will also be treated by a feeding therapist, dietitian, orthodontist, 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.

A diagnosis of van der Woude syndrome can be scary. We take time to explain your child’s condition. We help you fully understand your treatment options and make 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 support your child and family through the challenges of this condition. We help you find community resources and support groups.

Seattle Children’s provides craniofacial care for children in an area ¼ the size of the continental United States. We also care for children from across the globe. This has helped us develop systems to provide outstanding, personalized care at great distances.

Our team has created a plan (clinical care pathway) to treat cleft lip and palate. It guides our treatment of related syndromes like van der Woude. Our plan is based on evidence about what is most effective, and it outlines treatment from your child’s diagnosis onward.

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:

• Understand more about what causes cleft lip and palate
• Find ways to prevent clefts from happening
• Achieve the best possible outcomes for children born with clefts and related conditions

Learn about cleft-related research at Seattle Children’s.

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.

The Craniofacial Genetics team explains test results and gives you information about your child’s condition. Counseling can help you make informed decisions about family planning and your child’s treatment.

Our genetic counselors also advise people with genetic syndromes about their chance of having a child with the condition. A person with van der Woude syndrome has a 50% chance in each pregnancy of passing the abnormal gene to their child.

A gap in the lip or roof of the mouth can cause problems with breast- and bottle-feeding. We help you with feeding techniques to help your baby grow well.

We will check regularly to make sure that your baby is gaining weight. A dietitian may see you in the clinic to help you with your child’s diet.

If your child has a gap in their lip, they will need surgery when they are 6 months old.

If your child has lip pits or mounds, your doctor will talk with you about whether to remove them. Sometimes the lip pits are removed because they connect to cells that create mucus. Some families choose to remove the pits or mounds because of the way they look.

The pits or mounds can be removed at the same time as surgery for cleft lip. They can also be removed later, based on your child’s needs.

If your child has a gap in the roof of their mouth (cleft palate), they will need surgery to repair it when they are about 1 year old.

The cleft is closed during cleft palate surgery. Muscles at the back of the roof of your child’s mouth are moved across the cleft.

Usually, surgery takes less than 3 hours. Your child usually will stay in the hospital 1 to 2 nights.

For details, see Treating Cleft Lip and Cleft Palate.

We check your child’s hearing throughout their childhood.

A specialist trained to test hearing in babies and children (audiologist) will check your child’s hearing. If there are concerns, an ear, nose and throat doctor (otolaryngologist) will see your child.

Most children with cleft palate have fluid buildup behind their eardrum. This can make it harder to hear.

If this is a problem for your child, we recommend inserting small plastic tubes in your child’s eardrum. This keeps fluid from building up inside your child’s middle ear. It is often done at the same time as surgery to fix cleft palate.

If your child has cleft palate, they may have problems with speech and language.

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

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.
• 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.
• A speech appliance called an obturator that is specially made for your child.

Tooth problems are more likely to happen in children with clefts than in other children. Careful dental care is important, starting with baby teeth. Your child should have regular visits with your family dentist.

Our orthodontists are a key part of the team. We coordinate dental and orthodontic needs with other treatments to get the best results.

• Your child is likely to need orthodontic treatment to help line up their teeth if their cleft affects the gum line (the hard palate) or the back of the roof of the mouth (soft palate). Read more about craniofacial orthodontics.
• Orthodontic treatment helps prepare your child if they need surgery for a cleft in the part of the jaw that holds the teeth. The surgery is called alveolar bone graft.
• At 12 to 18 years, your child has final orthodontic treatment to correct their bite and align their teeth. For some children, this will include surgery on the jaws. For details on jaw surgery, see Treating Cleft Lip and Cleft Palate.