Postoperative Mental and Morphological Outcome for Nonsyndromic Brachycephaly

Abstract

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. In a subgroup or patients tested for the FGFR3 P250R mutation (n = 48), mental and morphological assessments were analyzed. Before surgery, mental status was better in the patients tested before 1 year of age (p < 0.001). The preoperative mental assessment always correlated with the postoperative assessment (p < 0.0001). The postoperative mental outcome was better when surgery was performed before the patient reached 1 year of age (p < 0.02). Although both the morphological and functional outcomes were better in the subgroup of noncarriers of the mutation, the differences were not statistically significant. Prominent bulging of the temporal fossae was frequently responsible for poor morphological outcome in carriers of the mutation. This study confirms the need for early corrective surgery before 1 year of age in brachycephalic patients to prevent impairment of their mental development. Suboptimal morphological and mental outcomes can be expected in patients with nonsyndromic brachycephaly who carry the FGFR3 P250R mutation. Primary correction of the temporal bulging should be performed in conjunction with fronto-orbital advancement to improve the morphological outcome in patients with the mutation.

Nautilus: craniotomia dinâmica - nova técnica cirúrgica e resultados preliminares

“…All techniques proposed and performed since the dawn of medical science to this day have aimed to provide space for the expansion of the brain in order to prevent or minimize compression damage 11 . The consensus is that treatment should be performed as early as possible because the longer it occurs after the first year of life, the greater are the neurological sequelae observed [12][13][14] . It is believed that after 2 years of age, the brain no longer needs sutural expansion to finish its growth because it can be achieved by simple cap remodeling alone.…”

Outcomes of endoscopic suturectomy with postoperative helmet therapy in bilateral coronal craniosynostosis

“…More recently, concerns have been raised that delaying intervention may result in increasing risk of more subtle cognitive deficits. 1,8,15 Numerous techniques for treating bilateral coronal synostosis have been described, but traditional management has focused on variations of a bilateral frontoorbital advancement (FOA) to increase the AP dimension of the skull and normalize the relationship between the supraorbital rim and the cornea. 6,[12][13][14] Advancements are more durable and safer when delayed until after the age of 6 months.…”

Connecting raised intracranial pressure and cognitive delay in craniosynostosis: many assumptions, little evidence

“…However, in an expanded 1988 study of ICP monitoring in children with craniosynostosis, Renier and Marchac 55 observed no functional benefit from surgery, concluding for their 66 patients with "North African oxycephaly" (a conveniently stereotyped combination of bicoronal and sagittal synostosis) that "operations were likely to stop the worsening of the mental impairment, but did not seem to correct already impaired intelligence" and that "surgery does not improve the [IQ] once it is already impaired." A similar conclusion was drawn by Arnaud et al 4 in a study of "mental outcome" in 99 children with "isolated" brachycephaly, a proportion of whom had the FGFR3 mutation and less than half of whom had both pre-and postoperative evaluations; these children were surgically treated "to prevent mental impairment caused by the intracranial hypertension," although no ICP details were given. "Surgery did not improve a child's mental status but may prevent deterioration in mental function."…”

“…"Surgery did not improve a child's mental status but may prevent deterioration in mental function." 4 Indeed the strongest predictor of postoperative mental outcome was a child's preoperative evaluation (p < 0.0001).…”

“…They include 1) the direct cerebral effects of any gene mutation or chromosomal abnormality; 36,86 2) hydrocephalus 13 (the number so affected was not given in Renier et al's 1982 paper 56 ); 3) chronic airway obstruction; 53 4) feeding difficulties and "failure to thrive"; 5) the developmental consequences of impaired vision and/or hearing; 6) low societal and familial expectations, including teasing; 64 and 7) the ill effects of previous surgical interventions, including those from anesthesia. 51 Further complicating the issue is uncertainty over the normal range of childhood ICP.…”