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Craniofacial Microsomia

Craniofacial microsomia is a congenital disorder that causes underdevelopment of one side of the face or the lower half of the face, typically the ear and jaw. In a small percentage of cases both sides of the face are affected. The eyes, cheeks and neck may also be affected. Craniofacial microsomia is 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 and lateral facial dysplasia.

Symptoms

Craniofacial microsomia is associated with underdevelopment of parts of the face. Some babies will have a slightly smaller jaw with a tiny raised piece of skin (skin tag) in front of the ear. It is also common for babies to have just one side of the face that is small and underdeveloped. The 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 it is uncertain as to what causes this condition, it 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

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

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. There are a variety of different techniques that are used to accomplish this and the craniofacial surgeon will discuss the options with you to choose the best method for your child.

Face

In cases where the cheekbones are underdeveloped a grafting procedure may be required. A variety of techniques may be employed to accomplish this goal. Autologous (patient's own) tissue or alloplastic (synthetic) implants may be used depending on the situation. 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. 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. Then gradually, the device is used to slowly open the osteotomy gap and move the jaw forward. As the jaw moves forward, the tongue also moves forward, which open's 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.

 

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