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Implications of a Vertex Bulge following Modified Strip Craniectomy for Sagittal Synostosis

Marucci, Damian D. Ph.D., F.R.A.C.S.; Johnston, Christine P. B.Sc., M.R.C.S.; Anslow, Philip F.R.C.R.; Jayamohan, Jayaratnam B.Sc., F.R.C.S.; Richards, Peter G. F.R.C.S., F.R.C.P.C.H.; Wilkie, Andrew O. M. D.M., F.R.C.P.; Wall, Steven A. F.R.C.S., F.R.C.P.C.H.

Plastic & Reconstructive Surgery: July 2008 - Volume 122 - Issue 1 - pp 217-224
doi: 10.1097/PRS.0b013e3181774240
Pediatric/Craniofacial: Original Articles

Background: Modified strip craniectomy is a common treatment for early isolated sagittal synostosis. The authors assessed the significance of the development of a progressive vertex bulge following strip craniectomy as a predictor of raised intracranial pressure or multiple suture synostosis.

Methods: All cases of sagittal synostosis treated by modified strip craniectomy (removal of the sagittal suture with lateral barrel staving) at the authors’ institution were reviewed. Eighty-nine patients with isolated sagittal synostosis were treated by modified strip craniectomy, usually before 6 months of age, between 1995 and 2005. Seven patients were noted to have developed a progressive vertex bulge. The vertex bulge was noted an average of 8 months postoperatively (range, 2 to 25 months). The clinical records of these seven patients were evaluated with regard to their clinical course, radiologic investigations, genetics testing, intracranial pressure monitoring, and the need for further surgery.

Results: Computed tomographic scanning demonstrated new synostosis involving other calvarial sutures in five patients. Five patients underwent intracranial pressure monitoring, and this was elevated in four patients. One patient required a ventriculoperitoneal shunt for hydrocephalus. All patients underwent genetic screening, and two were found to have fibroblast growth factor receptor (FGFR) mutations (one FGFR2 and one FGFR3 mutation). All patients required reoperation (calvarial remodeling) for either raised intracranial pressure, deteriorating head shape, or both.

Conclusions: A progressive vertex bulge after modified strip craniectomy is a sign of possible raised intracranial pressure, the development of progressive multiple suture synostosis, or both. It is an indication for genetic testing for FGFR mutations.

Oxford, United Kingdom

From the Oxford Craniofacial Unit and the Departments of Plastic and Reconstructive Surgery, Radiology, and Pediatric Neurosurgery, West Wing, John Radcliffe Hospital, and the Weatherall Institute of Molecular Medicine, Nuffield Department of Clinical Laboratory Sciences, University of Oxford.

Received for publication June 13, 2007; accepted November 1, 2007.

Presented at the Royal Australasian College of Surgeons Annual Scientific Congress, in Sydney, Australia, May 16, 2006, and at the European Society of Craniofacial Surgery Meeting, in Oxford, United Kingdom, September 16, 2006.

Steven A. Wall, F.R.C.S., F.R.C.P.C.H.; Oxford Craniofacial Unit, West Wing; John Radcliffe Hospital; Headley Way; Headington, Oxford OX3 9DU, United Kingdom; craniofacial@orh.nhs.uk

Disclosure: None of the authors has any commercial associations or financial relationships of relevance to this study.

©2008American Society of Plastic Surgeons