Reliable manifestations of increased intracranial pressure in patients with syndromic craniosynostosis

Abstract

Purpose: Systematic examination of increased intracranial pressure (ICP) is important during the follow-up period after surgical repair of syndromic craniosynostosis. In these patients, postoperative progress can be unclear due to the involvement of multiple sutures and the high incidence of relapse due to the progressive nature of the disease and to genetic variability. In this study, we investigated the clinical manifestations of increased ICP in syndromic craniosynostosis patients before and after surgery.

Materials and methods: We collected pre- and post-operative data from patients with syndromic craniosynostosis from January 2004 and December 2014 on the clinical manifestations of increased ICP, namely, the presence of 1) subjective symptoms, 2) visual disturbances and papilledema, 3) thumbprinting phenomenon (beaten copper appearance) on skull x-ray, and 4) hydrocephalus on computed tomography.

Results: A total of 17 syndromic craniosynostosis patients were included in this study, and three distinct patterns of disease progress were noted. Among all patients who underwent cranioplasty, the significant finding with regards to clinical manifestations was amelioration of the beaten copper appearance on skull x-ray after surgery. Likewise, among patients with recurrent increased ICP during the postoperative follow-up period, numerous clinical manifestations were noted, including subjective symptoms (33.3%), papilledema (50%), ventricular dilation (66.6%), and a beaten copper appearance on skull x-ray (100%).

Conclusion: Close monitoring to detect increased ICP is important during follow-up of patients with syndromic craniosynostosis. Among non-invasive methods for indirectly assessing ICP post-operatively, a beaten copper appearance on skull x-ray may be a reliable indicator of increased ICP.

Keywords: Crouzon syndrome; Increased intracranial pressure (ICP); Syndromic craniosynostosis.