1 | INTRODUCTION
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.