Craniofacial Microsomia

Babies with very small jaws may have breathing problems. If this is the case for your baby, your child’s doctors will discuss the options to manage and correct the cause.

Examples are:

• Ways to help position your baby so they can breathe more easily
• Surgery to lengthen the jaw and move it forward
• For severe breathing problems, placing a breathing tube in the windpipe (tracheostomy)

Sometimes children have trouble breathing while they sleep (obstructive sleep apnea). Over time, this may lead to problems with how the heart and lungs work. It also can add to problems with learning and behavior.

We will ask questions during clinic visits to check if breathing is a problem for your child. To better understand their breathing patterns, we may monitor your child overnight in our sleep lab.

The treatment options and timing will depend on your child.

Some babies with CFM have 1 of these conditions, which may affect their feeding:

• Small jaw
• Cleft lip
• An opening in the roof of their mouth (cleft palate)
• Cleft in the side of the mouth (macrostomia)

Your baby may not be able to breastfeed/chestfeed or use regular bottles. We help you with feeding techniques during infancy.

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

If your baby has a cleft lip or palate, our surgeon will talk with you about surgeries.

• Cleft lip surgery usually takes place when a baby is 3 to 4 months old.
• Cleft palate surgery usually happens at about 1 year.

Learn more about cleft lip and palate treatments.

At least half of children with craniofacial microsomia have hearing loss. They may have:

• Ear canals that are missing or very narrow
• Problems with the 3 bones in the middle ear that transmit sound
• Problems with the inner ear
• Nerve or brain processing delays related to hearing

Most often, babies’ hearing is screened in the hospital soon after birth or within a few days.

If problems are found, we do further testing. A specialist trained to test hearing in infants and children (audiologist) will perform the tests.

As your child grows, they will have a variety of hearing tests based on their hearing status and ear health.

Our ear, nose and throat specialist (otolaryngologist) and audiologist will talk with you and your child about hearing. This includes:

• If your child can benefit from hearing aids
• How to prevent further hearing loss
• The best place to sit in the classroom when your child reaches school age

The small size of the outer ear may make it hard for your child to wear hearing aids. One option might be hearing aids anchored to the bone with a tiny screw.

Your child may need a CT (computed tomography) scan to see their middle and inner ear. We do this when your child is about 5 years old. At that age, the ear is almost fully grown. CT scans help doctors decide if surgery is likely to help restore hearing.

Many children with craniofacial microsomia have ears that are small, not the usual shape or missing. This is called microtia (PDF). Our Otolaryngology Program is 1 of the few in the country to offer comprehensive treatment for microtia. Our team is skilled at rebuilding ears using synthetic (alloplastic) materials or cartilage from the child’s ribs (autologous). Our options also include a prosthetic ear that can be worn and taken off. Read more about microtia treatment.

CFM affects each child differently. We tailor treatment to your child’s and family’s needs.

Surgery for a cleft of the mouth

Some children with CFM have a cleft along the corner of the mouth. Muscles around their mouth are separated. This makes their mouth wider than is typical (macrostomia), which can make it hard to feed and form sounds for speech.

This can be treated by surgery that makes a complete ring of muscle around your child’s mouth.

• Surgeons carefully cut into the skin, the muscle and the tissue inside the mouth.
• They use a small zigzag cut (incision). This helps to hide any scars.
• Surgeons use nearby muscles to form a ring of muscle around your child’s mouth.
• They also bring the corners of the mouth closer together. This gives the mouth a more typical shape and function.

Facial reanimation for palsy

Some children with CFM have weakness or trouble moving their face. This is called facial palsy. It happens because the nerves in the face are not working right.

Often, facial palsy improves in the first few years of life.

If your child has severe problems with facial movement, your child’s team will include specialists from our Facial Reanimation Clinic. They will determine if surgery is likely to improve your child’s facial movement.

Options to improve facial symmetry

In about 65% of people with CFM, the 2 sides of their face look different (facial asymmetry).

If you or your child has concerns about how their face looks, talk to your child’s team. The treatment options depend on your child’s age. They range from counseling to surgery.

Skin tags

Some children with CFM have skin tags, most often in front of their ears. Skin tags are tiny extra pieces of skin. They may have a small narrow stalk connecting the skin bump to the surface of the skin, or they may be attached deeper below the skin. They are painless.

If your child has skin tags, your team will talk with you about whether to remove them.

Many children with CFM have a small lower jawbone. This can cause problems with breathing.

Your child’s teeth may not fit together well. Some children also have a small upper jaw.

A dentist or orthodontist will check if your child’s teeth are too crowded or if they are missing some teeth. The orthodontist also checks how well the upper and lower jaws fit together.

Starting at about 6 years old, we will talk with you about the best treatment and timing for your child.

• Some children may need a dental appliance or braces for the best dental function and appearance — usually when they are a teenager but sometimes sooner.
• Your child might have orthodontics alone or along with surgery, based on their needs.
• Many children with CFM need orthodontics to properly align their teeth when their face and jaw are nearly done growing (age 13 to 16 years).

Learn more about craniofacial orthodontics.

Enlarging the jaw

Many children with a small jaw benefit from surgery to make their jaw longer (orthognathic surgery) and correct their bite. Our craniofacial orthodontist and craniofacial plastic surgeon work together to plan the best treatment for your child.

Your child's team may recommend:

• A bone graft to lengthen the jaw or to make a new jaw joint. A graft uses bone or cartilage from another part of the body. We first think about this option when your child is 4 to 7 years old.
• Mandible distraction to increase the size of your child’s lower jaw. See a series of photos that show how we used this option for a child with craniofacial microsomia.
• Le Fort I maxillary advancement if your child’s upper jaw needs to be larger and moved forward.

Some children with CFM speak differently because of their physical differences (for example, with the jaw). Some children have a cleft lip or palate. In others, the muscles in the back of the mouth don’t work right. This is a type of velopharyngeal dysfunction (VPD).

VPD and clefting can affect your child’s ability to make speech sounds.

If you or your child's doctors have concerns about your child’s speech, your child will see a speech and language pathologist (SLP).

Treatment options may include:

• Speech therapy to help develop more normal speech patterns
• A custom-made speech appliance called an obturator
• Surgery if your child has a type of VPD called velopharyngeal insufficiency

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

Some children with CFM have a pinkish-white growth on their eye (epibulbar dermoid). If your child does, an eye doctor (ophthalmologist) on our team will check your child’s eyes for other differences.

The doctor monitors the growth on the eye to make sure it does not affect your child’s sight. If it is large or getting in the way of vision, the doctor may recommend surgery to remove it.

Some children with CFM have problems with the spine. Usually, the problem is in the neck, called the cervical spine.

Your child’s craniofacial pediatrician will order X-rays when the bones are well formed. This happens when your child is at least 3 years old.

If the X-rays show differences in the way your child’s bones fit together, the doctor will order more tests to get further details and see if your child needs treatment.

Children with CFM have an increased chance of kidney problems. We recommend that all children with this condition have an ultrasound of their kidneys when they begin treatment at Seattle Children’s.

We are looking to make sure that the kidneys formed in the typical way. If your child has a normal ultrasound, they will not need another one in the future.

This ultrasound usually takes less than 20 minutes. It does not require needle pokes or medicine.

It is rare for children with CFM to have problems with their heart. Your child’s doctor may decide that your child needs an echocardiogram (ultrasound picture of the heart) to check for problems. If you have concerns, talk to your child’s doctor.