Occipital expansion without osteotomies in Apert syndrome

Abstract

Introduction

Cranial expansion has been the mainstay of initial management of children with Apert syndrome. Surgical timing is a balance between the risk of relapse if performed too early and the possibility of raised intracranial pressure and ossification defects if performed later. Primary occipital expansion has been proposed as a method to delay the timing of frontal surgery. We have applied the principal of spring expansion of patent sutures to expand the posterior cranial fossa without a cranial osteotomy.

Patients and methods

All new Apert syndrome patients seen during the 3-year period December 2004–December 2007 underwent initial occipital expansion without osteotomy using spring expansion of the patent lambdoid suture.

Results

Four new Apert syndrome patients underwent posterior spring expansion of the patent lambdoid suture. Good occipital expansion was achieved in all cases. Standard frontal advancement was performed 5–18 months later. No relapse after frontal advancement has been seen after mean follow-up of 41 months.

Conclusions

Spring expansion of the patent lambdoid suture is an alternative technique to expand the posterior cranial fossa. Compared to current techniques it has very low morbidity. Occipital expansion is thought to treat several of the mechanisms responsible for raised intracranial pressure in Apert syndrome. When performed at 6 months of age it has enabled us to delay the time at which we would normally perform frontal advancement surgery until a time when the surgical result is likely to be more stable.