Anesthetic management of pediatric patients with Sturge-Weber syndrome: our experience and a review of the literature.

Abstract

Sturge-Weber syndrome (SWS) is a rare sporadic congenital neurocutaneous syndrome which is characterized by vascular malformation involving the brain, face and eye. The anesthetic management is complicated by its localized as well as systemic manifestations, associated anomalies and difficult airway due to the presence of angiomas of the oral cavity and airway. We retrospective analyzed the perioperative anesthetic management of children with SWS undergoing ophthalmic surgery and reviewed the literature. Medical records and anesthetic charts of all the children with SWS who had undergone an ophthalmic procedure under general anesthesia during the past 6 years were reviewed. Information related to the demographic profile, preoperative evaluation, anesthetic techniques, and perioperative complications were collected and analyzed. Forty children with SWS received general anesthesia for an ophthalmic procedure within the 6-year period. The median age of the children was 3 years. 30 (92.5%) children had facial port-wine staining, 10 (25%) had facial hypertrophy, 15 (37.5%) had a history of convulsion and 4 (10%) children had mental retardation. Inhalational induction was performed in 34 (85%) children (sevoflurane 82.8%). A laryngeal mask airway (LMA) and endotracheal tube were used in 32 (80%) and 5 (12.5%) cases, respectively. One patient had difficult mask ventilation and difficult LMA insertion. There was no significant problem in any of the other children. Preoperative evaluation with airway assessment should be performed with the knowledge of local and systemic manifestation of the syndrome. Proconvulsant and anticonvulsant properties of the anesthetics, as well as drug interactions of antiepileptic medications should be considered when planning anesthesia. Avoiding a rise in intracranial and intraocular pressures, vigilant intraoperative monitoring and postoperative care are the key for conducting safe anesthesia in these children. For ophthalmic procedures, LMAs can be used for airway maintenance with minimal complications in children with SWS.