Clinical StudiesDelayed Presentation of Isolated Sagittal Synostosis With Raised Intracranial Pressure and Secondary Chiari Malformation With Cervical SyringomyeliaSofos, Stratos S. MRCS, MBBCh; Robertson, Ben FRCS; Duncan, Christian FRCS (Plast); Sinha, Ajay FRCS Author Information Alder Hey Children's Hospital, Liverpool, UK. Address correspondence and reprint requests to Stratos S. Sofos, MRCS, MBBCh, Alder Hey Children's Hospital, E Prescot Rd, Liverpool L14 5AB, UK; E-mail: [email protected] Received 6 November, 2016 Accepted 12 February, 2017 The authors report no conflicts of interest. Journal of Craniofacial Surgery: July 2017 - Volume 28 - Issue 5 - p 1334-1336 doi: 10.1097/SCS.0000000000003734 Buy Metrics Abstract Sagittal craniosynostosis (SC) is the most common type of premature suture fusion presenting in approximately 1 in every 5000 births with a 3:1 male:female ratio. The most common indication for surgery is the improvement of the cosmetic appearance of the skull, since a cranial deformation may have a significant psychosocial impact on affected patients. Relief from raised intracranial pressure is a further indication for surgery, although an increased intracranial pressure (ICP) can be demonstrated only in a minority of affected children at diagnosis. It is even more rare to have Chiari malformations (CMs) secondary to ICP in an isolated SC, as shown in a study by Strahle et al (Neurosurg Focus 2011;31:E2), demonstrating only 5 (3%) of the 183 with isolated sagittal synostosis having a CM. The authors present the rare case of a 4-year-old boy presenting late with raised intracranial pressure (40 mm Hg) and his management with 1 stage vault expansion and decompression of CM. © 2017 by Mutaz B. Habal, MD.