Apert syndrome is one of the craniosynostosis syndromes which, due to its association with other skeletal anomalies, is also known as acrocephalosyndactyly. It is a rare congenital anomaly which stands out from other craniosynostosis due to its characteristic skeletal presentations.

PURPOSE: Non-syndromic sagittal and/or metopic craniosynostosis, occurring once in every 4,000 live births, accounts for half of all craniosynostosis cases. Despite success in identifying the genes underlying rare syndromic craniosynostoses, mutations in these genes are very rarely found in their non-syndromic counterparts. We considered that the often sporadic occurrence of non-syndromic crani...

Craniosynostosis, premature closures of the skull sutures, results in dysmorphic features if left untreated. Brain growth and cognitive development may also be impacted. Craniosynostosis repair is usually performed in young infants and has its perioperative challenges. This article provides background information about the different forms of craniosynostosis, with an overview of associated anom...

Purpose. We describe a case of 3-year-old girl with rhombencephalosynapsis, a rare cerebellar anomaly. Patient. A 3-year-old girl was admitted to our hospital due to congenital torticollis and asymmetry of face, skull and trunk. Craniosynostosis was suspected due to abnormal head shape. 3D-CT revealed closure of the sagittal suture without scaphocephalic skull. Due to atypical craniosynostosis ...

INTRODUCTION: Craniosynostosis surgery is commonly performed for children at academic centers across the US. Previous literature has found increased hospital and surgical volume to correlate with better post-operative outcomes and decreased costs. However, the association between annual hospital volume of craniosynostosis surgery on inpatient complications and resource utilization is not well s...

In this review, we discuss in detail our current procedure for treating craniosynostosis using multidirectional cranial distraction osteogenesis (MCDO). The MCDO method allows all phenotypes of skull deformity to be reshaped by distraction osteogenesis, except in patients who are 5 months of age or younger and patients with posterior cranial vault problems. We report the results of clinical dat...

OBJECTIVE To review research and theory regarding the neurobehavioral correlates and outcomes of single-suture, or isolated, craniosynostosis in children. METHODS A critical review of 17 studies of the hypothesized association between isolated craniosynostosis and neurodevelopment. RESULTS Isolated craniosynostosis is associated with a three- to fivefold increase in risk for cognitive defic...

Apert syndrome is an autosomal dominant disease characterized by craniosynostosis, midface hypoplasia and syndactyly. In general, patients present in early childhood for craniofacial reconstruction surgery. Anesthetic implications include difficult airway, airway hyper-reactivity; however, possibility of raised intracranial pressure especially when operating for craniosynostosis and associated ...

BACKGROUND AND PURPOSE Complex and syndromic craniosynostosis can be complicated by raised intracranial pressure (ICP), which in the absence of other identifiable origins, is probably caused by venous hypertension. Children with these conditions have been shown to have narrowing of the sigmoid sinus-jugular vein complex. Evidence of bony narrowing of the jugular foramina in children with comple...