Sturge–Weber syndrome (SWS) is a neurocutaneous syndrome characterized by facial and leptomeningeal angioma, glaucoma, seizures, and neurological disability.¹ It is a rare nonhereditary condition with an incidence of 1 per 50,000 live births. It has no racial or sex predilection.² Cutaneous angiomas called port-wine stains (PWS) are present in 96% of patients with SWS.³ They are usually distributed unilaterally along dermatomes supplied by the ophthalmic and maxillary parts of the trigeminal nerve.⁴ However, they are sometimes bilateral or involve the neck, limbs, or other body parts.⁵

Intraorally, angiomas may involve not only the lips, buccal mucosa, palate, gingiva, and floor of the mouth but also the pharynx, larynx, trachea, and bronchi.^{5, 6} Anesthetic techniques such as tracheal intubation may cause the rupture of angiomas, which may lead to uncontrolled hemorrhage.

In addition, oral changes have been reported in 40% of patients with SWS because of gingival overgrowth and asymmetric jaw growth.⁷ Soft or hard tissue hypertrophy often requires soft tissue correction and bone surgery.⁸Furthermore, these oral changes may make mask ventilation and intubation difficult.

The anesthetic management of patients with SWS has been reported in previous studies.^{3, 6, 9} However, data on the anesthetic management of patients with SWS are limited in oral surgery, even though patients with SWS are predicted to be adapted to orthognathic surgery because of their asymmetric jaw growth. This report presents airway management using submental intubation in a patient with SWS in sagittal split ramus osteotomy (SSRO) under general anesthesia. This report aimed to explore better anesthetic management for avoiding the rupture of angiomas in a patient with SWS.

Informed consent for case reporting was obtained from the patient and his parents.