Management of secondary turricephaly in craniofacial surgery

In children with syndromic craniofacial disorders, such as Crouzon and Apert syndromes, who are managed surgically, a difficult problem that can occur is secondary turricephaly. One of the more widely accepted theories as to why this deformity occurs is that a lack of skull base growth results from fusion of the basal and facial sutures. Despite initial adequate forehead and orbital bandeau advancement, many of these patients require subsequent procedures, which do not always correct the characteristic deformity. We have identified a subset of 11 syndromic children who developed this characteristic deformity of turricephaly after primary reconstruction, 6 of whom required either secondary or tertiary procedures. Only 5 patients had a good outcome with a mean follow up of 4.5 years (range 1-8 years). Our surgical methods, and our rationale for the timing of surgery are discussed, and the literature on the management of this problem is reviewed.