Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder presenting unique anesthetic challenges due to extensive vascular malformations. Key considerations include difficult airway management due to extensive facial and oral angiomas, seizure control, and maintaining stable intracranial and intraocular pressures. This case report discusses the perioperative management of a 57-year-old female with SWS undergoing dental surgery under general anesthesia and synthesizes the current literature on anesthetic considerations and management. Preoperative examination revealed a facial port-wine stain covering more than 75% of the face, extensive oral angiomas, macroglossia, and severe lip hypertrophy, raising concerns for difficult airway management and risk of hemorrhage. A rapid sequence induction was performed following upright preoxygenation, and video laryngoscopy provided a grade I view of the glottis. Endotracheal intubation was accomplished atraumatically, and the case proceeded uneventfully. The patient was extubated but experienced mild desaturation in the post-anesthesia care unit, prompting overnight observation before discharge at baseline oxygenation. This case highlights the importance of anticipating and planning for difficult airway management, maintaining vigilance for perioperative seizure control, and implementing strategies to minimize elevations in intracranial and intraocular pressures.
Kodra et al. (Thu,) studied this question.
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