Surgical strategy for Apert syndrome: Retrospective study of developmental quotient and three ‐dimensional computerized tomography

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 and were followed up for more than five years. Nine patients underwent operations before 1 year of age (early surgery group) and 3 patients underwent operations later in life (late surgery group). They underwent fronto‐orbital advancement for primary surgery. We evaluated post‐operative 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.
Source: Congenital Anomalies - Category: Genetics & Stem Cells Authors: Tags: Original Article Source Type: research