Postoperative cognitive outcome for synostotic frontal plagiocephaly.

Object: The goals of this study were to evaluate the cognitive outcome after surgery for synostotic frontal plagiocephaly and to compare the effects of early (< 1 year of age) and late (> or = 1 year of age) surgical correction on intellectual development.

Methods: In a prospective study involving 220 patients, both preoperative and postoperative cognitive function were measured using developmental quotient (DQ) or intelligence quotient (IQ) determination. Preoperative intracranial pressure (ICP) was also measured, and the presence of the fibroblast growth factor receptor 3 (FGFR3) P250R mutation was assessed.

Genetic considerations in nonsyndromic midline craniosynostoses: a study of twins and their families.

Object: The origins of both sagittal synostosis (scaphocephaly) and metopic synostosis (trigonocephaly) remain unclear. Genetic and environmental factors probably play a role. Twin and family data of patients with these conditions are presented.

Fetal exposure to sodium valproate associated with Baller-Gerold syndrome: case report and review of the literature.

Case Report: We report three patients with a history of maternal valproate use during pregnancy who presented with a combination of metopic suture synostosis and upper limb malformations, which could be diagnosed as Baller-Gerold syndrome (BGS). The patients underwent surgical treatment for the craniofacial deformity, during which standard frontocranial reconstruction was performed.

Review Of The Literature: Only 32 patients have been reported in the world literature and these cases support the emerging view that BGS is not a distinct syndrome, but should instead be considered to be an heterogeneous phenotype with variable etiology.

Postoperative mental and morphological outcome for nonsyndromic brachycephaly.

Bilateral coronal synostosis causes functional and morphological problems that require fronto-orbital advancement in infancy to correct the brachycephalic deformity and to prevent mental impairment caused by the intracranial hypertension. In this study, 99 children with isolated cases of brachycephaly were prospectively followed to study their preoperative and postoperative mental outcome, which was evaluated using developmental or intelligence quotients. Several factors were analyzed: age before treatment, age at the time of surgery, and the correlation between mental assessments before and after surgery.