Craniosynostosis and Craniofacial Disorders

Craniosynostosis is a congenital deformity of the infant skull that results when the skull sutures (fibrous joints) between various skull bones fuse improperly. Skull growth is limited at the fused suture, and growth at the remaining sutures results in an abnormal shape to the infant skull. Craniosynostosis is usually apparent in infancy, and is characterized by an abnormal but characteristic head shape, depending upon how many and which sutures are involved. In most cases, the problem is solely cosmetic and leads to an abnormal head shape; sometimes, the growth of the facial bones is affected as well. Rarely is the growth of the skull restricted enough to cause increased pressure in the head and lead to headaches, visual loss, or developmental delays.

The severity of the deformity depends on which sutures close, the point in the development process that the closure occurred, and the success or failure of the other sutures to compensate by expansion.

The majority of cases of craniosynostosis are sporadic, and there is no family history or other causal factor. In some instances, craniosynostosis is inherited and can be associated with other developmental problems.

The diagnosis is usually apparent simply by examining the child's head. A radiological examination is usually necessary to confirm the problem, characterize the deformity, and guide the corrective surgical procedure. Plain x-rays of the skull usually show the problem well; computerized tomography (CT) scans provide additional information about the fused sutures and the status of the underlying brain. Three-dimensional (3-D) computed tomographic (CT) scans may provide information to guide surgical correction.

Surgery involves releasing the fused suture and correcting the cosmetic deformity. A number of different procedures are available to correct the deformity. When multiple sutures are fused and the pressure in the head is raised, surgery may be necessary to relieve intracranial pressure and accommodate for brain growth, as well as improve cosmetic appearance.

One of the most common reasons for a malformed head shape is occipital flattening (or plagiocephaly), a condition which is frequently confused with lambdoid craniosynostosis (fusion of the lambdoid suture in the back of the head). In occipital flattening, the infant's head is flattened in the back (the occiput) because the infant lies persistently on the back of the head (often with the head turned preferentially to one side). In addition, the ear on that side is often pushed forward, and even the forehead may seem a bit prominent compared with the other side. This produces a parallelogram shape to the infant's head. X-rays can show a small amount of bone build-up, which is misinterpreted as fusion of the lambdoid suture; however, occipital plagiocephaly is not caused by lambdoid craniosynostosis and usually does not need to be treated surgically. See the monograph on this website for additional information on occipital plagiocephaly.

To make an appointment, please call 717-531-8952.

Our Craniofacial clinic is held in conjunction with Penn State Hershey Plastic Surgery.

Diagnosis

Usually, craniosynostosis is suspected on the basis of an unusual head shape. Plain X-rays may show that a suture(s) has closed, but is not very accurate in making the diagnosis. The preferred imaging is with a 3-dimensional CT (computerized tomography) scan. This test uses X-rays to produce multiple pictures at various levels of the skull. A computer is used to compile the pictures and produce a 3-dimensional image of the skull and sutures. This method is very accurate in assessing whether fusion between bones has occurred.

Treatment Options

The treatment of craniosynostosis is surgical. Surgery is generally recommended if the closed sutures have lead to an increase in pressure on the brain or to marked skull deformity. The fused suture(s) can be removed and the shape of the skull remodeled to form a more natural shape. Such surgery is often performed by a neurosurgeon and a craniofacial plastic surgeon working together. In more severe cases, the surgery may be done in stages to achieve the best result.

In cases where the head shape is abnormal but the sutures have not closed, a nonsurgical approach to reshaping the head is available. A cranial molding helmet can be fitted to the child. This method uses the child's own head growth to help reshape the skull by allowing growth in areas which appear flattened and restricting growth in areas which appear prominent. The helmet is generally worn almost constantly for a period of weeks to months and is most successful in younger infants. Once the child approaches one year of age, the rate of head growth slows and the helmet therapy is less successful.