Craniosynostosis is the premature fusion of skull sutures that normally separate bone plates on the skull. This early suture fusion causes areas of growth restriction in areas of the skull that results in abnormal head shapes. Craniosynostosis may be either syndromal (associated with other congenital problems) or nonsyndromal. A geneticist can assist in determining whether the synostosis is syndromal or nonsyndromal. The early fusion of the suture results in characteristic patterns of head shapes depending on which suture is involved.
The treatment of craniosynostosis is surgical and involves combinations of removal of sections of the skull along with reshaping of the involved areas. These procedures are coordinated with a craniofacial surgeon and a pediatric neurosurgeon.
Some of the characteristic patterns include:
Sagittal Craniosynostosis
This is craniosynostosis resulting from the premature fusion of the sagittal suture, which is in the midline of the skull. This is the most common form of craniosynostosis. The resulting deformity is scaphocephaly or “boat shaped” skull. Premature fusion of the sagittal suture results in restriction of growth from side-to-side and increased growth from front-to-back. There may also be prominence of the frontal skull (frontal bossing) and posterior skull (occipital bossing) and ridging along the midline of the skull. The early treatment of sagittal craniosynostosis may involve excision of the involved suture along with some other skull reshaping. The treatment of late craniosynostosis involves a total cranial vault reshaping.
Metopic Craniosynostosis
This form of craniosynostosis results from the premature fusion of the metopic suture, which is in the midline of the forehead. The premature fusion of this suture results in trigonocephaly or “triangular shaped” skull. The patients have a ridge in the midline of their skull with narrowing between the temples. They may also have narrowing between their eyes (orbital hypotelorism). The treatment of this deformity involves an incision over the top of the skull from ear to ear. The procedure then involves removing the front of the skull and the top of the eye sockets and reshaping of the bone.
Unilateral Coronal Craniosynostosis
Is the result of the fusion of the right or left side of the coronal suture. The forehead is flat on the involved side with prominence (bossing) on the opposite side. There is bulging in the temporal area on the involved side. The orbit is more vertically oriented on the involved side. The nasal root (bridge of the nose) deviates toward the involved side and the nasal tip deviates away from the opposite side. The correction of this deformity involves removing the frontal area of the skull and also the top of the eye sockets and reshaping the bone segments. They are then replaced into position.
Bilateral Coronal Craniosynostosis
Is caused by the fusion of both the right side and left side of the coronal suture. This type of craniosynostosis is frequently the result of a craniofacial syndrome. The skull is very short in a front-to-back direction and is also very tall vertically (turribrachycephaly). The patients also have prominence in the temporal areas bilaterally. Correction of this deformity involves removal of the frontal skull and upper eye portion of the eye sockets with reshaping and replacement. In many cases, after the frontal area has been corrected, the patient will require a total cranial vault reshaping to decrease the vertical height of the head and lengthen the skull front-to-back.
Lambdoid Craniosynostosis
Results from the premature fusion of the lambdoid sutures on the posterior aspect of the skull. The deformity results in a trapezoidal- shaped skull when viewed from above with flatness on the side of the involved suture. There is bulging (bossing) in the mastoid area on the involved side and the ear on the involved side is pulled posteriorly relative to the uninvolved side. There is prominence of the frontal skull opposite the involved suture. It is critical to carefully evaluate children with flatness in the back of the skull. Most of the children have deformational plagiocephaly that can be treated with either positioning or a molding helmet. Lambdoid synostosis, however, requires surgical therapy.
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