Van der Woude Syndrome

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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 normal growth and intelligence. It affects 1 in 35,000 to 1 in 100,000 newborns.

Children with van der Woude syndrome 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 one of two that are important in forming the structures of 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.
Other names for van der Woude syndrome
This syndrome is also called:
- Lip-pit syndrome
- Cleft lip and/or palate with mucous cysts of lower lip
- VDWS

Seattle Children's Craniofacial Center

When it comes to diagnosing and treating craniofacial conditions, our team is among the most experienced in the world. No matter how rare the condition or syndrome, we have likely cared for someone like your child.

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.

Diagnosing van der Woude Syndrome

Sometimes cleft lip is diagnosed before a baby is born because the cleft can be seen during an ultrasound. Ultrasounds are part of regular pregnancy care.

Usually, cleft lip and palate are diagnosed after birth. Cleft lip is easy to notice.

Cleft palate may not be noticed at first. Cleft palate 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.

Your doctor may also do a blood test to see if your child has a variant in a gene that can cause Van der Woude syndrome. This can help tell if your child has van der Woude syndrome.

Other conditions caused by changes in the IRF6 gene include popliteal pterygium syndrome (PPS). That condition 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.

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 gene variant to their child.

Treating Van Der Woude Syndrome

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.

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, 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.
Cleft palate repair

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.
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. It is often done at the same time as surgery to fix 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 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.
Dental care and aligning the teeth
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.