Craniofacial Microsomia at North Austin Campus

Craniofacial microsomia (CFM) is a rare craniofacial condition where part of the face of a child is small or underdeveloped. It is the most common craniofacial condition after cleft lip and palate, but not all children with CFM will need treatment. 

Most cases of CFM were not passed on from a parent. Researchers continue to investigate what causes CFM. CFM usually affects the ear and lower jaw, but can also result in differences in the eye, cheek and neck. Some children with CFM also have conditions affecting the spine, heart, and kidneys. Our team of specialists at TCH North Austin are ready to provide all the screening tests and care your child with CFM will need. 

Are their different types of Craniofacial Microsomia?

Craniofacial microsomia (CFM) can present in many ways, from something very minor that will never need medical treatment, to very severe presentations that will need a dedicated team of specialists at Texas Children's Hospital North Austin to help the child throughout their growing years. 

It can be confusing when you try to search the internet for information on craniofacial microsomia since several different names can be used for the same condition. Examples are:

• Hemifacial microsomia
• First and second branchial arch syndrome
• Oculo-auriculo-vertebral (OAV) sequence
• Goldenhar syndrome

Goldenhar is a specific type of CMF with findings that can include a pinkish-white growth on the surface of the eye called an epibulbar dermoid, abnormalities of the cervical (neck) spine, and possible weakness of the face muscles (facial palsy).

Children with mild CFM may only have a slightly smaller lower jaw with a raised piece of skin and cartilage (branchial remnant) in front of their ear. Other children can have one side or both sides of their face being much smaller than than usual, and can have a small abnormally shaped ear (microtia) with heating loss. Patients with CFM can also have a cleft of the upper lip, or in some cases the cleft comes from the corner of the mouth (macrostomia). 

What tests will my child need if they have Craniofacial Microsomia?

• If our team thinks your child has CFM after the first examination, we will want to make sure that they do not have any problems in other parts of their body. We may recommend that your child have an ultrasound to check the shape of their kidneys. This test is easy and takes about 20 minutes. 
• To examine the formation of their spine, we will get X-rays of your child’s cervical (neck) spine at around 3 years of age when the bones are fully formed. If there are abnormalities on the X-ray, we will refer your child to one of our spine specialists. We will also watch your child’s lower (thoracic and lumbar) spine to make sure that it is growing straight.
• Although it is rare for children with CFM to have problems with their hearts we may suggest that your child have an ultrasound of the heart (echocardiogram) to check for problems. If your child needs special heart care, Texas Children’s has one of the best cardiac programs in the country. 

What specialists will my child with CFM need to see?

The Craniofacial Program at Texas Children’s North Austin has nationally recognized specialists to meet all your child’s needs. At your first visit your craniofacial provider will determine which of these other specialists your child will need to meet:

• Craniofacial, Plastic and Reconstructive surgeon
• Craniofacial Orthodontist
• Pediatric Dentist
• Hearing specialist (audiologist)
• ENT (otolaryngology) specialist in airway assessment
• ENT specialist in hearing aids and ear canal surgeries
• Eye doctor (ophthalmologist)
• Dietitian
• Genetic counselor
• Child Psychologist
• Speech pathologist

What procedures can help treat Craniofacial Microsomia?

At Texas Children’s North Austin Campus, our experienced craniofacial team will tailor treatment recommendations to the specific CFM needs of your child. Some children with CFM may only require one or two minor surgeries, whereas other children may benefit from specialized staged surgeries into their teenage years to meet all their needs. Our surgical treatment plans are always focused on procedures that give your child the most benefit with the least number of surgeries. In some cases, we may recommend delaying a surgery until your child is older to get the most benefit. Treatments will depend at what age we first meet you and your child. 

• Soon after being born your child will need a hearing test to determine if they need to meet our hearing specialists. During infancy we also make sure your baby is feeding and breathing well. Specialized feeding bottles and sleep positioning devices may be needed. We usually repair clefts of the lip or corner of the mouth around 6 months of age. If a cleft palate is present, we will repair that with a surgery at one year of age. In rare severe cases, some infants struggle so much with feeding and breathing that they do better with a special breathing tube through the skin of the neck (tracheostomy) that is placed by our airway specialists, and a feeding tube through the skin of the belly into the stomach (gastrostomy tube). Small skin and cartilage growths (branchial vestiges) in front of the year can be removed at one year of age or wait until your child is older.
• In the pre-kindergarten years we will check how your child’s jaws are growing and have our dental specialists examine their teeth development. If the lower jaw is not growing well, we prefer to delay treatment whenever possible, but some children benefit from earlier lengthening of the jaw with a procedure called distraction osteogenesis. If the joint of the lower jaw (TMJ) is missing, our surgeons specialize in a procedure to reconstruct the joint using a piece of the bone and cartilage from your child’s rib. This is done through small 1” scars and is done between age 5-7 years. 
• Around 5 years of age your child may need a CT scan (computerized tomography) to look at the middle and inner ear structures. CT scans are used to find out whether surgery to restore hearing by our Ear, Nose and Throat (ENT) specialists will be helpful. There are many options for treating abnormally shaped ears. We will discuss these with you and your child. Some families choose to do nothing; others choose to have surgery.
• During middle school years we will continue to monitor your child’s breathing. If we have concerns about how well they breath when they sleep, we will arrange a sleep study to be done at the hospital. During these years we can also talk with you and your child about surgeries that can be done to help the balance or symmetry of their face. These include techniques like micro-fat grafting, cheek reconstruction, or eyelid surgeries. 
• In the early teenage years most children with CFM will need orthodontics. Our orthodontist is one of the very few specialists in the country with recognized fellowship training in craniofacial treatment. Your orthodontist will help determine if your teenager will benefit from surgery to reposition the jaw (orthognathic surgery).
• In very severe cases of CFM a child with CFM can only breath safely using a tracheostomy. Many of these children have had multiple previous surgeries to try to remove the tracheostomy but without success. Our team specializes in a surgical technique that has been successful in treating these children. Families from across the country come to North Austin for this specific procedure. The procedure is called craniofacial counter-clockwise distraction osteogenesis or C3DO.