Long-term Outcomes of Non-syndromic and Syndromic Craniosynostosis: Analysis of Demographic, Morphologic, and Surgical Factors

Abstract

In this study, we analyzed the outcomes of patients (followed for 5-38 years, average 17.3 years) with craniosynostosis and evaluated their long-term prognosis. In all, 51 patients who underwent surgery for craniosynostosis between 1982 and 2015, including 12 syndromic and 39 non-syndromic cases, were included. The average age at the initial surgery was significantly lower in the syndromic group than that in the non-syndromic group (9.8 months old vs. 19.9 months, respectively). The surgical procedures did not significantly differ between the two groups, but repeat surgery was significantly more common in the syndromic group than in the non-syndromic group (4 children [30.8%] and 3 children [7.7%], respectively). The children requiring repeat surgery tended to be younger at the initial surgery than those who did not. Those patients who required repeat surgery did not have significantly different surgical procedures initially. The incidence of developmental retardation was 49.0% (43.5% in the non-syndromic group and 66.7% in the syndromic group), and only two children in the non-syndromic group displayed recovery. This study is the first to analyze the prognosis for patients who were followed for at least 5 years after cranioplasty. Repeat surgery was common, especially in syndromic patients. Severity of skull deformity and early initial surgery may be important factors determining the need for repeat surgery. Developmental retardation was also common, and improvement was rare even after surgery.

Keywords: craniosynostosis; long-term outcome; non-syndromic; syndromic.

Fig. 1

(a) Case 5. A non-syndromic child with plagiocephaly had the 1st operation using conventional fronto- orbital advancement at the age of 4 months. At 4 years, she had the 2nd operation using conventional cranioplasty due to forehead backslide and flattening. (b) Case 1. A child with Pfeiffer syndrome with oxycephaly had the 1st operation using conventional fronto-orbital advancement at the age of 5 months. At the age of 15 months, she had the 2nd operation using fronto-orbital advancement by distraction osteogenesis due to the backslide of the forehead and orbital bandeau.