Craniosynostosis
|
Brachycephaly |
Description Craniosynostosis is the premature fusion or closure of the cranial sutures. This can occur as a single suture such as the metopic (trigonocephaly), sagittal (scaphocephaly), unilateral coronal (plagiocephaly), and bilateral coronal (brachycephaly) or as more than one suture in any given case. This results in abnormal head growth and requires cranial vault remodeling to correct functional and related deformities of the skull. Syndromal craniosynostosis is closure of multiple sutures of the cranium producing a constellation of symptoms and signs that collectively characterize the appearance and abnormal growth of the head and face. Specific examples of syndromic craniosynostosis are Aperts, Crouzons, Pfeiffer, Carpenter, Saethre-Chotzen, and Kleeblattschadel anomaly (closure of all sutures). In these syndromic cases, the children's faces and heads are programmed to grow abnormally and require repeated and ongoing surgical correction to give optimal functional and aesthetic results. |
Brachycephaly
A brachiocephalic deformity consists of an abnormally broad head with a high forehead. The broad nature of the shape causes the length of the skull to be shortened in the front to back plane. Brachycephaly is associated with the early closure of the coronal sutures and is also present in many syndromal abnormalities, such as Aperts, Crouzons, Pfeiffer, Saethre-Chotzen and Carpenters syndromes.
Kleeblattschadel
Term used to describe a trilobular head or cloverleaf deformity of the skull which occurs when all the sutures are closed. Seen from the front, the skull shape resembles that of a cloverleaf. Surgical procedures by a skilled craniofacial surgeon can give these patients a more normal appearing head.
Oxycephaly
Oxycephaly is a form of craniosynostosis which describes an abnormally high conical head shape due to the fusion of both the coronal and sagittal sutures.
Plagiocephaly
This type of craniosynostosis results in both skull and facial asymmetry and the head shape is often described as "lopsided" in appearance. It is caused in the front by the premature fusion of one of the coronal sutures. At the back of the skull, the occipital asymmetry is produced when the lambdoid suture closes early.
Scaphocephaly
This term describes a boat-shaped head which is associated with the early closure of the fontanelle (soft spot) and sagittal suture. This is the most common form of craniosynostosis. It is usually relatively benign condition in that intracranial pressure is usually in the normal range. Cranial surgery is usually recommended, however, to give a more normal appearance.
Trigonocephaly
Trigonocehpaly is a head deformity which gives the front of the head a wedge-shaped appearance. It consists of a narrow pointed forehead with close-set eyes. The overhead view shows this "wedge-shaped" forehead region and a bony ridge running up from the root of the nose. This deformity is associated with premature fusion of the metopic suture and can be corrected with surgical intervention.