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Craniofacial Anomalies

There are a large number of conditions that affect the shape of a child’s face and head. Some basic features include:

When a baby is born, the skull bone is really a collection of many smaller bones which touch one another at sites, known as sutures. The most noticeable is the anterior fontanel (soft spot) where four bones meet.
As the brain grows, the sutures allow for rapid expansion in an equal fashion. It’s the brain that essentially determines the head size and shape under normal circumstances. If for any reason one or more sutures closes too early the brain is forced to grow in a different direction where the bones are not resisting growth.

This is like blowing up a balloon but pinching it as it inflates. The air goes in but the balloon inflates in the direction away from where you are pinching it.

This condition is known as craniosynostosis. Some of these conditions are inherited and associated with other developmental problems, however, the majority are sporadic and not associated with developmental problems. Certain craniosynostses can be detected at birth while others are not obvious for several months. Since it is in the first year of life that the most rapid head growth occurs, it is usually the only significant time to diagnose and treat these conditions.

How are Craniofacial Anomalies diagnosed?

Certain conditions are obvious and a physician can make the diagnosis by just examining the baby. In other cases, a radiographic study may be needed. The most popular study is a three dimensional CT scan, but most cases may be proven with a simple set of skull x-rays. Unfortunately, it is possible no single radiological test is adequate to make a clear diagnosis. In these cases, it may be best to wait a few weeks to months to see if the condition is going to progress.

Cases of infants who develop flatness on the back side of the head have increased over the past few years. It stems from research done several years ago that determined SIDS (sudden infant death syndrome) may be linked to babies sleeping face down. Since then, it is recommended that babies sleep on their side or back.

Often there is an associated bulging of the forehead and a displacement of the ear on the same side of the flattening. The great majority of these children do not have a synostosis but rather a condition known as positional molding or “lazy lambdoid.” The lambdoid is the involved suture. The majority of these children should improve without surgery, utilizing either repositioning techniques or a specially molded helmet. Surgery may be needed for advanced cases.

Is this operation necessary?

In the minority of cases, if you do not treat, the synostosis pressure can build up in the head and cause injury to the brain. The majority of cases will see clear deformity to the skull and face that the child will have to live with for his entire life. These two reasons are usually enough to justify the risks associated with surgery.

The timing and type of surgery is very much dependent on the type of synostosis and the preferences of the neurosurgeon. A team of surgeons, a craniofacial plastic surgeon and a pediatric neurosurgeon often perform these operations. The majority of operations are performed in the first year of life, and some even in the first few months of life.

Treatment of Craniofacial Anomalies

Most operations require extensive removal, re-contouring and, finally, replacement of the skull. Often this is associated with blood loss sufficient to require a transfusion. Fortunately, these operations are elective and blood donation can be performed. Many different techniques are available to the surgeon to replace the bone securely at the end of the operation.

In addition to surgical sutures and wires, very fine plates and screws can be used. These materials are essentially too small to be felt under the skin. In experienced hands, the results of surgery are usually excellent and only rarely would further surgery be required.

In recent years, minimally invasive procedures are offered. Operations can be done through one to two inch incisions. In some cases, the same bone removal is performed and, in others, a more limited removal of the fused suture is removed and then the child is placed in a special helmet to aid in the re-contouring at the skull for the first year of life. These minimally invasive procedures are generally done at a young age, often in the first few months of life.