Craniosynostosis: To study the spectrum and outcome of surgical intervention at a tertiary referral institute in India

Abstract

A BSTRACT Aims and Objectives: This study aimed to analyze the spectrum and surgical outcome of cases of craniosynostosis operated at a tertiary referral institute in India Design: This was a cross-sectional study. Materials and Methods: We retrospectively examined 60 cases of craniosynostosis operated at our institute from 2008 to 2014 (with a minimum follow-up of 2 years). Data was collected including name, age, gender, involved sutures, other medical conditions, whether syndromic craniosynostosis or not, whether symptoms and signs of intracranial hypertension were present or not, associated findings on magnetic resonance imaging of brain and cervico-medullary junction, type of surgery performed, age at which surgery was performed, perioperative complications (if any), and findings on follow-up. To be able to analyze the surgical results, we used the seven category classification system used by Sloan et al. Results: Craniosynostosis affected more men than women. The incidence of syndromic craniosynostosis was 11.67%. Mean age at first surgery was 3.85 years. Chiari malformation was present in 80% of the Crouzon’s syndrome cases, 62.5% of the oxycephaly cases, and 4.44% of the non-syndromic, non-oxycephaly cases. Intracranial hypertension was present in 80% of the Crouzon’s syndrome cases, 75% of the oxycephaly cases, and 6.67% of the non-syndromic, non-oxycephaly cases. Perioperative complications were present in 42.86% of the syndromic craniosynostosis cases, 50% of the oxycephaly cases, and 15.56% of the non-syndromic, non-oxycephaly cases. Compromised overall correction was present in 4 of 7 cases of syndromic craniosynostosis, 3 of 8 cases of oxycephaly, and 2 of 45 cases of non-syndromic, non-oxycephaly group. Conclusion: The study highlights the importance of educating the masses so that cases of craniosynostosis present early. The incidence of Chiari malformation, intracranial hypertension, and perioperative complications was significantly higher in the syndromic craniosynostosis and oxycephaly groups than in single-suture craniosynostosis. The best surgical outcome and the least perioperative complications were seen in the trigonocephaly group. Compromised overall correction and reoperations were more common in the syndromic and complex craniosynostosis groups than in single-suture craniosynostosis.

Preprocedural Electrophysiological Monitoring in Craniofacial Surgery for a Patient with Chiari Malformation

“…1,3,5 However, in patients with craniosynostosis there is a high incidence of cranio-cervical junction and/or cervical spine anomalies that makes prolonged neck hyperextension dangerous. 6,7 Since the modified prone position was first introduced in the late 1900s, it has been contraindicated in the setting of tonsillar herniation such as in CM because of the increased risk of spinal cord compression and/or infarction. 1,8 In patients with CM, the cerebellar tonsils can move significantly with neck positioning.…”

Epilepsy in Patients with Craniosynostosis: A Systematic Review

“…If there is any sign of raised intracranial pressure, such as enlarged fontanelles, progressive optic atrophy, convulsions, or multiple-suture synostosis, most surgeons perform surgery as quickly as possible [6]. There is a lack of literature reporting the complications of craniosynostosis, especially epileptic seizures, and most of them are included in clinical series [7,8].…”