Focus session on the changing "epidemiology" of craniosynostosis (comparing two quinquennia: 1985-1989 and 2003-2007) and its impact on the daily clinical practice: a review from Necker Enfants Malades

Abstract

Introduction: The aim of this study was to evaluate the evolution in craniosynostosis in terms of incidence and management in the last 20 years and to discuss the clinical implications and future perspectives with regards to the practical organization of daily practice.

Methods: The relative incidence and management modalities of craniosynostosis hospitalized at the Craniofacial Unit of Necker, French National Referral Center for Faciocraniosynostosis, in two different quinquennia: 1985-1989 (group A) and 2003-2007 (group B) were reviewed.

Results: A total of 1,286 children were included in our study; group A 472, group B 814, that is an overall increase of 1.7-fold in the second period of the study. Sagittal synostosis remained the most frequent type (45% of cases in both groups). Conversely, the number of cases of Pfeiffer syndrome increased from seven patients to 20 (x2.8) and metopic synostosis cases increased from 49 to 193 (x3.9). Mean age at surgery in group A was 22.5 months and 13 months in group B.

Discussion: The comparison of the two quinquennia shows that there has been a change in the incidence of the different types of synostosis. The timing for surgery has also changed with the treatment of younger children in group B compared to group A. The principles of the surgical treatment of monosutural craniosynostosis have remained unmodified in our center. However, there has been an evolution in the techniques due to the introduction of new tools such as internal and external distractions, springs, and resorbable plates.