Crouzon syndrome is the most common syndrome among the craniosynostosis group. Crouzon syndrome accounts for about 4.8% of all of them. It commonly has autosomal dominant inheritance with complete penetrance and variable expressivity from subtle to severe forms and characterized by craniosynostosis, exophthalmos, and hypoplastic maxilla with relative mandibular prognathism. Mutation of the fibr...

C raniosynostosis, the premature fusion of one or more sutures of the skull, is a common craniofacial anomaly, with an estimated incidence of 1/2000 to 1/3000 births. 2 Characteristic deformities of skull shape occur as a result of different patterns of sutural fusion, while compensatory skull expansion occurs at unaffected sutures to accommodate the growing brain. Premature fusion of the sagit...

The management of children with craniosynostosis is multidisciplinary and has evolved significantly over the past five decades. The treatment is primarily surgical. The anesthetic challenges continue to be the management of massive blood transfusion and prolonged anesthesia in small children, often further complicated by syndrome-specific issues. This two-part review aims to provide an overview...

The management of children with craniosynostosis is multidisciplinary and has evolved significantly over the past five decades. The treatment is primarily surgical. The anesthetic challenges continue to be the management of massive blood transfusion and prolonged anesthesia in small children, often further complicated by syndrome-specific issues. This two-part review aims to provide an overview...

#201750). FGFR2 mutations show variable clinical penetrance and patients with the same FGFR2 mutation can exhibit diverse clinical features. Therefore, FGFR2-related craniosynostosis syndromes are usually named according to the accompanying extra-cranial manifestations. ABS is a rare type of craniosynostosis syndrome. ABS is typically distinguished by systemic bony fusions of skull sutures and ...

Various operative techniques are available for the treatment of craniosynostosis. The patient's age at presentation is one of the most important factors in the determination of the surgical modality. Minimally invasive suturectomy and postoperative helmet therapy may be performed for relatively young infants, whose age is younger than 6 months. It relies upon the potential for rapid brain growt...

A case of severe craniosynostosis-radial aplasia (Baller-Gerold) syndrome is described in a newborn male, following a pregnancy complicated by polyhydramnios and intrauterine growth retardation. Death occurred after two hours owing to a prolonged apnoeic spell. Extensive agenesis of the frontal and parietal bones, resulting in a very large fontanelle, in addition to coronal bilateral craniosyno...

In 1851, Virchow introduced the term craniosynostosis to describe a variety of abnormalities in calvarial growth. These skull deformities are usually apparent in infancy. When an abnormal calvarial configuration is detected, a radiologic evaluation is necessary to characterize the deformity and to guide the corrective surgical procedure. Affected children are believed to have an improved outcom...

BACKGROUND Hypofibrinogenaemia is one of the main reasons for development of perioperative coagulopathy during major paediatric surgery. The aim of this study was to assess whether prophylactic maintenance of higher fibrinogen concentrations through administration of fibrinogen concentrate would decrease the volume of transfused red blood cell (RBCs). METHODS In this prospective, randomised, ...

The brain and skull represent a complex arrangement of integrated anatomical structures composed of various cell and tissue types that maintain structural and functional association throughout development. Morphological integration, a concept developed in vertebrate morphology and evolutionary biology, describes the coordinated variation of functionally and developmentally related traits of org...