Delayed Presentation of Isolated Sagittal Synostosis With Raised Intracranial Pressure and Secondary Chiari Malformation With Cervical Syringomyelia

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.

Late Presenting Sagittal Craniosynostosis: An Update to a Standardized Treatment Protocol

“…This is challenging for primary care physicians in the community, and these evaluations are often made in a delayed manner. Even so, delayed surgical intervention in patients with sagittal craniosynostosis has been associated with poorer neurodevelopmental outcomes, [16][17][18] so we must continue to educate our pediatric colleagues about this diagnosis.…”

“…Although reconstruction of sagittal craniosynostosis within the first year of life takes advantage of increased healing potential of the infant skull and dura, various surgical techniques for the correction of sagittal craniosynostosis at a later age have also been investigated. 17,[24][25][26][27][28][29][30] The potential increase in intracranial volume after surgical reconstruction is reduced in older patients compared with younger patients with sagittal craniosynostosis. 30 Hudgins et al first presented a technique of total calvarial reconstruction to shorten the anteroposterior length, widen the biparietal diameter, and address frontal and occipital abnormalities.…”

Delayed Presentation and Treatment of Sagittal Synostosis: A Case Report

“…It affects between 4,200 and 5,000 live births. Sagittal synostosis causes scaphocephaly and a reduced cephalic index [1]. Patients usually present in the first year of life after the unusual head shape is noticed by a parent or health visitor.…”