Craniofacial Anomalies
Deformities of Skull Shape
Craniosynostosis is the premature fusion of one or more sutures (the growth centers) of
the skull. This problem occurs in infants and can lead to an abnormal head shape. The
resulting shape of the skull is characteristic of and can be classified by the affected
suture. Surgical correction is often necessary depending on the severity of the deformity.
Most often this is performed by craniofacial surgeons.
Syndromic Synostosis
Aperts Syndrome
Crouzon’s Syndrome
Pfeiffer Syndrome
Carpenters Syndrome
Saethre-Chotzen Syndrome
Isolated Craniosynostosis
Scaphocephaly, Trigonocephaly,
Brachycephaly, Oxycephaly, Plagiocephaly, Kleeblattschadel
Scaphocephaly
(from Greek "skaphe"; light boat or skiff)
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
(from Greek "trigonos"; triangular)
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.
Brachycephaly
(from Greek "brachys", short)
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 Apert, Crouzon, Pfeiffer,
Saethre-Chotzen and Carpenters syndromes.
Oxycephaly
(from Greek "oxys", sharp)
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
(from Greek "plagios", slanting)
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.
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.
