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Craniofacial Microsomia

• A congenital disorder that causes underdevelopment of one side of the face or the lower half of the face, typically the ear and jaw
    o In a small percentage of cases, both sides of the face are affected.
    o Eyes, cheeks and neck may also be affected.
• The second most common facial birth defect after clefts, occurring in one in 3,500 to 4,500 infants

This condition has many other names, including:
• Hemifacial microsomia
• First and second branchial arch syndrome
• Otomandibular dysostosis
• Oculo-auriculo-vertebral sequence
• Facio-auriculo vertebral syndrome
• Goldenhar syndrome
• Lateral facial dysplasia

Symptoms

• Associated with underdevelopment of parts of the face
    o Some babies will have a slightly smaller jaw with a tiny raised piece of skin (skin tag) in front of the ear.
    o Common for babies to have just one side of the face that is small and underdeveloped
    o Ear may be abnormally shaped or missing entirely
• Aside from the deformities, most babies with this condition do not have other medical problems.

Common features include:
 
• Small ear
• No ear canal
• Facial tags
• Other ear deformities
• Facial palsy
• Small cheekbone
• Epibulbar dermoid (pinkish-white growth on the eye)
• Wide mouth
• Cleft lip and palate
• Small opening of the mouth
• Small lower jaw
• Crooked lower jaw

What causes craniofacial microsomia?

• This condition develops in the fetus during the first three months of pregnancy.
• While the cause is uncertain, craniofacial microsomia appears to occur due to poor blood supply to the face, which restricts development to this area.
• In most cases it is not inherited. However, a couple who has had a child with craniofacial microsomia carries a 3 percent risk of having another child with this condition.

Treatment of craniofacial microsomia

• Our craniofacial team will determine the proper treatments and timing of the treatments to provide the best results for your child.
• Treatment factors include how severely your child has been affected and your child's age.

Ears
• Surgery to correct the shape of your child's ears is usually recommended between 6 and 8 years of age. By this time, your child's ears have almost reached their adult size.
• A variety of different techniques are available – and you’ll discuss options with our craniofacial surgeon to choose the best method for your child.

Face
• In cases where the cheekbones are underdeveloped, a grafting procedure may be required.
o Autologous (patient's own) tissue or alloplastic (synthetic) implants may be used depending on the situation.
o Timing of facial grafting depends on many factors and is usually performed around 10 years of age.

Feeding and breathing
• In severe cases, a small jaw or a cleft palate associated with craniofacial microsomia may cause difficulty breathing and feeding as an infant.
• Breathing problems may be resolved with positioning devices, tubes and jaw advancement procedures.
• In some cases, it may be necessary to place a breathing tube in the windpipe known as a tracheostomy.

Hearing
• We recommend a hearing screening within the first few days of birth.
• If hearing is impaired, our ear, nose and throat specialist and audiologist will discuss how to minimize hearing impairment and prevent further loss.

Jaw and teeth
• If your child has a small lower jaw, a procedure called distraction osteogenesis may be recommended.
    o This surgery involves making a break on both sides of the lower jaw – and then placing a small metal distraction device under the skin and across the breaks, also called the osteotomy.
    o The device is used to slowly and gradually open the osteotomy gap and move the jaw forward.
    o As the jaw moves forward, the tongue also moves forward, which opens your baby's airways.

Your child also may have missing teeth. If this is the case, we may recommend that a dentist or orthodontist evaluate your child's teeth and the upper and lower jaw to determine how well the jaws fit together.