S7A-04 SESSION 7A: SAGITTAL SYNOSTOSIS SAGITTAL SYNOSTOSIS WITHOUT SCAPHOCEPHALY – A REVIEW OF 8 CASES

Abstract

Introduction: Sagittal Synostosis presenting with scaphocephaly is the most common form synostosis with a prevalence of up to one in 2000 live births. However sagittal synostosis without scaphocephaly is rarely reported in the literature, with only one case series of 8 patients reported to date1. We also identified a large cohort and reviewed our patients to expand upon the current knowledge of the clinical presentation and management of this group. Methods: A search was conducted using the hospital database in order to identify craniofacial patients with a diagnosis of sagittal synostosis. On review, 306 patients were identified, 8 (2.6%) of which had a diagnosis of sagittal synostosis without scaphocephaly (Male 6, Female 2) where scaphocephaly was taken as a cephalic index less than 73. Cases were reviewed using the hospital database and the patient’s electronic records. Results: All patients had a normal cranial index. Only one patient had symptoms of headache with a strong family history of migraine. Clinical features of the headache were not in keeping with raised pressure but due to the chronic nature, intracranial pressure (ICP) monitoring was undertaken and was normal. All patients had normal ophthalmological assessment. None of these patients required vault surgery. Conclusion: To the best of our knowledge, this is jointly the largest case-series conducted amongst this group. Results from the previous case series revealed that in 6 of the 8 cases, intracranial pressure monitoring was required. Of those six patients, 4 (67%) were said to be abnormal and later went on to have vault surgery. Our data challenges this as none of our series required intracranial monitoring. This could be more in keeping with the fact that there are presumably many patients in the community with this diagnosis who are not identified due to the absence of the classic scaphocephalic head shape. Further studies into cases of this condition would need to be identified to allow a wider and more comprehensive comparison