Craniofacial Syndromes
Craniosynostosis is a congenital deformity of the infant skull that occurs when the fibrous joints between the bones of the skull (called cranial sutures) close prematurely. Due to this closure, the baby develops an abnormally shaped skull because the bones do not expand normally with the growth of the brain. The condition is usually apparent in infancy as an abnormal but characteristic head shape and, in some patients, abnormal facial features. In some cases, the growth of the skull is restricted enough to cause increased pressure in the head and can lead to headaches, visual problems or developmental delays. Source: www.aans.org
Diagnosis & Surgery
Diagnosis can usually be made by feeling the skull for suture ridges and soft spots, as well as checking neck position and facial deformities. 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 may show the deformity, but computerized tomography (CT or CAT scans) provide more precise information about the fused sutures and the status of the underlying brain. Three-dimensional (3-D) CT scans may provide additional information to guide surgical correction.
Surgery: Significant advancements have been made in the surgical treatment of cranial deformities. In more complex cases, there is a team approach utilizing the expertise of a pediatric neurosurgeon and craniofacial surgeon. Surgery involves releasing the fused suture and reshaping the brow, eye orbits and skull as needed. The goal of surgery is to correct cosmetic deformities and allow for normal expansion of the brain within the cranium. Most experts recommend that babies undergo surgery between the ages of three to eight months, depending on the case and surgical procedure. Early intervention is beneficial for several reasons, aside from prevention of further deformities: the bones are most malleable at this age, bone re-growth is quicker and more likely and rapid brain growth benefits from skull remodeling. Source: www.aans.org
