JBI Database of Systematic Reviews & Implementation Reports 2013;11(11) 54 - 63 doi: 10.11124/jbisrir-2013-1093 Page 54 Risk factors for developing recurrent raised intracranial pressure post cranial vault remodelling in nonsyndromic craniosynostosis in children: a systematic review protocol Xenia Doorenbosch MBBS 1,2 Jared Campbell BHsc(Hons), PhD 2 Kandiah Umapathysivam PhD 2 1. The Joanna Briggs Institute, School of Translational Health Science, The University of Adelaide 2. The Australian Craniofacial Unit, Women's and Children's Hospital, Adelaide Corresponding author Xenia Doorenbosch xenia.doorenbosch@adelaide.edu.au Review question/objective The objective of this review is to identify the factors associated with an increased risk for developing recurrent raised intracranial pressure in nonsyndromic children who have had previous cranial vault remodelling surgery. Background An infant's skull is made up of several pairs of floating bone plates connected by fibrous tissues called sutures. The sutures allow for deformation during birth and expansion of the cranial vault in relation to brain volume growth. Brain growth pushes the two sides of the patent sutures apart, thereby enabling growth of the skull. The main sutures are named sagittal, metopic, coronal and lambdoid sutures. These sutures close at different ages by ossification; the metopic closes between three to nine months of age and the sagittal, lambdoid and coronal sutures close between 22 to 39 years of age. 1 The term craniosynostosis was first described by Otto in 1830 2 and is defined as premature fusion of one or more cranial sutures which occur in isolation or in the context of a craniodystopic syndrome. It affects approximately one in 2000 births. 3 There are several classifications of craniosynostosis; isolated or compound, primary or secondary and either syndromic or nonsyndromic. 4 Isolated craniosynostosis refers to fusion of a single suture and compound refers to premature fusion of multiple cranial sutures. Syndromic synostosis typically involves premature fusion of multiple sutures and are hereditary forms of craniosynotosis with extracranial features which are associated with mutations of fibroblast growth factor receptor family (FGFR1, FGFR2, FGFR3), TWIST1 and MSX2 gene. 1 The well known syndromes associated with craniosynostosis include Pfeiffer, Apert, Crouzon, Muenke and Saethre-Chotzen. 4 Nonsyndromic craniosynostosis relates to sporadic cases without the presence of extracranial features. Primary craniosynostosis occurs in the absence of an associated syndrome or