Hemifacial Microsomia

What are the signs and symptoms of hemifacial microsomia?

Patients with hemifacial microsomia have differences in the structures of their faces. Their ears and jaws are usually smaller and abnormally formed on the affected side. The skull, cheekbone and eye can also be affected. In 10% to 25% of children, both sides of the face are affected.

• Skull: The temporal fossa may be affected, which can lead to differences in the head shape and jaw joint.
• Cheek: Differences in the cheek bones and upper jaw may cause a flattened appearance. The muscles, nerves and soft tissues can also be affected and cause weakness or asymmetry on the affected side. The child can have skin tags or macrostomia (wide mouth).
• Jaw: The jaw can be shortened and crooked on the affected side, causing a slanted or abnormal bite. A patient’s chin can be off-centered and they can also have abnormalities with teeth.
• Eye: The orbital bony structures and the eye may be smaller on the affected side or can be entirely missing. Patients can get epibulbar dermoids (benign cysts) or notches on or around their eye.
• Ear: Differences in the ear can range from appearing normal to anotia (no ear). Patients often have some degree of microtia (a small or abnormally developed ear). Hemifacial microsomia sometimes causes hearing loss.

How do we test for hemifacial microsomia?

Your child’s doctor will check their face and body to help them diagnose the condition. They may also do a CT or MRI scan to  get more detailed images of the skull and facial structure. The team at our Radiology Department is pediatric-trained and very experienced in helping kids feel safe and comfortable while getting imaging done.

How do we diagnose hemifacial microsomia?

The Craniofacial Program at Children's Colorado will do a thorough check of your child. Your child may also see other specialists to better diagnose and treat their particular condition. Together, we will determine the right diagnosis for your child that will help us create a treatment plan that is unique to your child’s needs.

How do we treat hemifacial microsomia?

The main concerns in early treatment planning for children with hemifacial microsomia have to do with vision, breathing, hearing and jaw growth. Sometimes hemifacial microsomia affects one or more of these, and other times it doesn’t affect any of them. Our craniofacial experts work with other specialists from our hospital, such as ophthalmologists, pulmonologists and audiologists, to deal with vision, breathing and hearing issues.

Based on your child’s unique condition, our team will work to treat every affected area, which can include:

Cheek

The orbit and zygoma (cheekbone) may need to be reconstructed with bone grafts or implants. The timing of this type of craniofacial surgery is determined by your child's symptoms. If there are no concerns about the function of the orbit, this surgery is usually done after the age of 7, when the growth of the face is almost done.

Jaw

A small jaw is often part of hemifacial microsomia and can cause breathing and eating problems. Positioning, special feeding devices or tubes may help with these issues. If the symptoms are bad enough, your child’s doctor may recommend a procedure to lengthen the mandible (lower jaw). Some children with difficulty breathing will need a tracheostomy. Some children with a particular type of lower jaw deformity may get a bone graft to lengthen the mandible, which is usually done between 6 to 12 years old.

To help lengthen the jaw earlier in life, doctors might recommend a procedure called distraction osteogenesis, in which we place a device that slowly stretches the jaw, or we place a rib bone graft to help the jaw to grow. Distraction can be done when a child is a baby or when they have severe breathing problems. Rib grafts can be done as early as 4 years old.

Patients who need to have their jaw lengthened will likely need braces to help straighten their teeth. They may also need jaw surgery as a teenager to fix their bite and improve facial symmetry.

Mouth

If your child has a cleft palate, we may need to do surgery between 3 months and 18 months of age. Your care team might also recommend speech therapy.

Eye

We work with ophthalmologists to check your child’s vision and how well their eyes work. This tells us if they need treatment to make their vision better. Our doctors may prescribe eye ointment to use when sleeping to protect the corneas.

Ear

We work with audiologists and otolaryngologists to check your child’s hearing and make recommendations for hearing assistive devices if needed. Your child’s doctors will address differences on the outside part of your child’s ear when their ears have grown to about 80% of their adult size. Depending on what your care team finds, your child may need surgery. These surgeries are often broken into different stages and start after the age of 6. Instead of surgery, we might recommend a prosthetic ear or do an external ear reconstruction using rib bone grafts or plastic ears.