Van der Woude Syndrome
Contact the Craniofacial Center
What is van der Woude syndrome?
Van der Woude syndrome is a genetic form of cleft lip and palate.
Your baby may have:
- A gap in their lip (cleft lip) or the roof of their mouth (cleft palate) or both
- Small mounds of tissue or pits on their lower lip
Most children with this have typical growth and intelligence. The condition affects 1 in 35,000 to 1 in 100,000 newborns.
Van der Woude syndrome is also called lip-pit syndrome or cleft lip and/or palate with mucous cysts of the lower lip.
Children with this condition need care by providers in many areas of healthcare. At Seattle Children’s Craniofacial Center, our team has experts from 19 different specialties.
What causes van der Woude syndrome?
Usually, van der Woude syndrome happens because of changes in 1 of 2 that are important in forming the upper and lower lip and roof of the mouth (palate). The genes are called IRF6 and GRHL3.
Sometimes the condition is caused by a new change that was not passed on by a parent. Other times, a child inherits the gene variant 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.
Craniofacial Care at Seattle Children’s
What are the symptoms of van der Woude syndrome?
The symptoms of van der Woude syndrome vary from child to child.
You may notice these differences in your child:
- Pits near the center of their lower lip. These small indents in the skin may look wet if they contain cells that create saliva or mucus.
- Small mounds of tissue on their lower lip.
- A gap in their lip (cleft lip).
- An opening in the roof of their mouth (cleft palate).
- Some children with van der Woude syndrome have missing teeth.
The pits and mounds are different sizes in different children. This means they can be hard to see or very easy to see.
How is van der Woude syndrome diagnosed?
Sometimes cleft lip is diagnosed before a baby is born because the cleft can be seen during an .
Usually, cleft lip and palate are diagnosed after birth. Cleft lip is easy to notice. However, cleft palate may not be obvious at first. It is hard to see, especially if the soft tissue inside the mouth covers the gap in the roof of your child’s mouth (submucous cleft palate).
You and your child’s doctor may discover a cleft palate only after your child shows other signs, like having a hard time eating.
The doctor may recommend that your child have an evaluation at the Craniofacial Genetics Clinic. At the clinic, you can learn about testing options to see if your child has a variant in a gene that can cause van der Woude syndrome.
Changes in the IRF6 gene can cause other conditions, such as popliteal pterygium syndrome (PPS). PPS is more severe and may include webbing behind the knees.
-
Genetic counseling and testing
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.
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.
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 down the gene that causes it.
How is van der Woude syndrome treated?
There is no single care plan for van der Woude syndrome. The treatments and timing we recommend depend on how the syndrome affects your child. Based on your child’s needs, our specialists provide a wide range of services to help with feeding, lip differences, cleft palate, hearing, speech, teeth alignment and other concerns.
-
Feeding evaluation and management
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.
-
Surgery for lip differences
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, the doctor will talk with you about whether to remove them. Sometimes a surgeon recommends removing the lip pits because they connect to cells that create mucus. Some families choose to remove the pits or mounds because of the way they look.
We can remove the pits or mounds either at the same time as surgery for cleft lip or later on, based on your child’s needs.
-
Cleft palate repair
If your child has a gap in the roof of their mouth (cleft palate), they will need surgery to close it when they are about 1 year old. A surgeon will move muscles at the back of the roof of your child’s mouth across the cleft.
Usually, surgery takes less than 3 hours and children stay in the hospital 1 to 2 nights.
For details, see Treating Cleft Lip and Cleft Palate.
-
Hearing evaluation and ear tubes
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. We often do this at the same time as surgery to close cleft palate.
-
Speech development
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 treating your child’s cleft palate, 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.
- A speech appliance called an obturator that is specially made for your child.
Your child’s surgeon and SLP will work together to recommend what is best for your child.
-
Dental care and aligning the teeth
Tooth problems are more common in children with clefts.
If a cleft extends through the gum line, it will affect how your child’s teeth develop. They are more likely to have teeth that are misshapen, crowded or missing.
Children with clefts are at higher risk for cavities. The hard coating (enamel) on their teeth may have weak areas that decay easily. Careful tooth 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.
- Your child is likely to need orthodontic treatment to align their adult teeth if their cleft affects the gum line (the hard palate) or the back of the roof of the mouth (soft palate).
- Most children with clefts have an evaluation by a craniofacial orthodontist on our team by age 6. We coordinate dental and orthodontic care with other treatments to get the best results.
- 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 adjust their bite and align their teeth. For some children, this will include surgery on 1 or both jaws. For details on jaw surgery, see Treating Cleft Lip and Cleft Palate.
Why choose Seattle Children’s for van der Woude syndrome treatment?
Each year, Seattle Children’s Craniofacial Center cares for hundreds of children with syndromes that involve cleft lip and palate — including van der Woude syndrome.
Many of our patients are newly diagnosed babies. We also work with children who received their initial care at another hospital. We treat children, teens and young adults up to age 21 and sometimes beyond.
- Cleft lip and palate experts: Seattle Children’s has more surgeons who specialize in treating clefts than any other center in the country. We also perform more surgeries for this condition than any other center in the region that includes Washington, Alaska, Montana and Idaho. Read about Seattle Children’s expertise in craniofacial surgery.
- Team-based care: We’ll bring together all the team members your child needs, such as a craniofacial ; nurse; ; ; ; ; craniofacial ; ear, nose and throat doctor (); feeding therapist; ; ; ; and .
- The right treatment at the right time: If an ultrasound during pregnancy shows that your baby has cleft lip and possible cleft palate, we offer prenatal counseling to help you prepare. As your child grows, we provide a range of services on a schedule that matches your child’s needs.
- Support for your whole family: A diagnosis of van der Woude syndrome can be scary. We take time to explain your child’s condition. We help you understand your child’s treatment options and make the choices that are right for your family.
- Research to advance care: Our team has created a clinical care pathway to guide our treatment of cleft-related syndromes like van der Woude based on research about what is most effective. Our research is helping us 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
Contact Us
Contact the Craniofacial Center at 206-987-2208 for an appointment, a second opinion or more information.
If you live outside of Washington, Alaska, Montana and Idaho, please contact our coordinator for out-of-area patients at 206-987-0814.
Providers, see how to refer a patient.
Paying for Care
Learn about paying for care at Seattle Children’s, including insurance coverage, billing and financial assistance.