Overview
Scaphocephaly, is the premature closure of the sagittal suture. The sagittal suture is the long suture on the top of the head that runs from front to back starting at the anterior fontanelle and extending backwards along the middle of the skull to the back of the head. The resulting deformity is a long and thin skull which has been compared to the keel of a boat.
Premature fusion of the sagittal suture is the most common type of craniosynostosis, constituting more than half of all cases. The incidence of sagittal synostosis in the population is approximately 1 in every 4,200 births with a male to female ratio of 3:1.
Causes
The long narrow head shape is secondary to premature fusion of the sagittal suture. Scaphocephaly is usually isolated and not associated with syndromes. It occurs frequently in premature infants.
The growth of the skull bones does not occur at the fused sagittal suture but does occur at the coronal and lambdoid sutures. The skull becomes elongated as the frontal and occipital bones compensate for the restricted lateral growth of the parietal bones.
Symptoms
The head typically is elongated in the anterior-posterior diameter and shortened in the biparietal diameter and the ridging of the sagittal suture is palpable.
Often the fontanelle, or soft spot at the top of the head, is absent or closed. A ridge can be seen, or felt running along the top of the head. The skull looks very long when viewed from the side and when viewed from above, the skull is wider near the forehead and gets narrower towards the back. When looking straight on at the infant’s face, the forehead seems quite prominent, and the sides of the skull look narrow.
Diagnosis
The deformity is usually noticed at birth or soon after when the infant’s head starts growing. There are usually no associated systemic abnormalities. Skull radiography (plain X-rays, computerized tomography (CT) scan and magnetic resonance imaging (MRI)) can confirm the diagnosis of scaphocephaly.
Treatment
Traditional surgery for sagittal craniosynostosis involves releasing (re-opening) of the fused suture and widening the skull by opening up the coronal and lambdoid sutures on both sides of the head. Several variants of the original technique have been extensively described in the literature.
Prognosis
Surgical correction of scaphocephaly leads to significant improvement in the head shape over the ensuing months. Follow up to ensure normal brain and skull development is required.
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