There are many surgical techniques for craniosynostosis. However, the indications for and timing of surgery still remain unclarified. Most of the skull growth in craniosynostosis is completed in the first year, and the bone is strong enough to undergo distraction osteogenesis. However, previous reports showed that patients operated on before 1 year of age had better IQ than those operated later in life. This report aims to consider the best timing for cranial expansion and surgical strategy for Apert syndrome. From January 2002 to December 2011, 13 patients with Apert syndrome were operated on and were followed up for more than 5 years. Nine patients underwent operations before 1 year of age (early surgery group) and three patients underwent operations later in life (late surgery group). They underwent fronto-orbital advancement for primary surgery. We evaluated postoperative developmental quotient every year and cephalic index (CI) measured by three-dimensional computerized tomography (3D-CT) at the age over 5 years retrospectively. Eleven of 13 patients improved their developmental quotient scores, with no significant intergroup differences. The CI evaluation showed cases with remnant brachycephalic deformity in both groups. Two patients with remnant plagiocephalic deformities tend to have primary surgery early in life compared to the others. Thus the delay in primary surgery had little influence on psychological development. We conclude that the primary surgery can be delayed unless the intracranial pressure needs to be controlled. In addition, fronto-orbital advancement could not sufficiently improve the brachycephalic appearance, other procedures like posterior vault distraction might be better alternatives.
Keywords: Apert syndrome; Development Quotient; Distraction osteogenesis; Syndromic craniosynostosis.
© 2017 Japanese Teratology Society.