Latex-Free Anesthesia for Craniosynostosis Surgery Associated With Xia-Gibbs Syndrome: A Case Report

Abstract

This case report describes the anesthetic management of a one-year-old patient with Xia-Gibbs syndrome, which is a rare genetic condition caused by a mutation in the AHDC1 gene. The procedure involved calvarial vault remodeling and fronto-orbital advancement to correct a left coronal craniosynostosis. In addition, the patient had a history of seizures and latex-fruit syndrome, which necessitated careful preoperative planning and management. Despite the difficulties provided by the patient's cranial abnormalities and the paucity of literature on anesthetic experiences with the condition, the treatment was completed successfully and without complications. Insights are offered about the anesthetic approach for this syndromic pediatric patient undergoing neurosurgery with a high risk of bleeding. It is important to understand and prepare for the perioperative implications of this disease in order to achieve a safe outcome.

Keywords: anesthetic challenges; bleeding risk; craniosynostosis; latex-fruit syndrome; pediatric anesthesiology; rare genetic mutation; total cranial vault remodelling; xia-gibbs syndrome.

Figure 1. Our patient with Xia-Gibbs syndrome and his dysmorphic features

A notable right frontal bulge seen in the (A) frontal view of the child with XGS, and in two tomographic slices, the axial (B) and the sagittal (C)

Figure 2. Original photo of the comparison of a cranial vault three-dimensional model and the patient's already surgically remodeled skull

Figure 3. Calvarial vault remodeling with fronto-orbital advancement on the patient