Anaesthesia for Sturge-Weber syndrome

Progressive retinal vessel malformation in a premature infant with Sturge-Weber syndrome: a case report and a literature review of ocular manifestations in Sturge-Weber syndrome

“…Ocular alterations in SWS are seen in approximately 30-60% of patients [7]. The vascular anomalies include the facial nevus flammeus [1,2], ipsilateral leptomeningeal angiomatosis [8], diffuse choroidal hemangioma , and rarely retinal vascular anomalies [30,31]. The GNAQ gene, involved in regulating intracellular signaling pathways [32], has been identified as a potential cause for SWS [5].…”

“…Chavala et al reported a patient with a bilateral facial nevus flammeus who developed exudative retinal detachments in both eyes without mentioning the diffuse choroidal hemaniogma [80]. A case of unique subretinal osseous metaplasia in SWS with endophthalmitis, glaucoma and complicated cataract was reported by Pavlenko et al [39] Glaucoma is another frequent ophthalmic complication of SWS, occurring in 43.82% of all cases [9,30,31,. It can be further classified into early-onset glaucoma and late-onset juvenile glaucoma.…”

Anaesthetic management of a case of Sturge-Webers syndrome

“…[9] The choice of anaesthesia should be influenced by detailed clinical and radiological investigation. General anaesthesia may necessitate avoidance of drugs that can cause an increase in the intraocular pressure, such as succinylcholine and ketamine.…”

“…The SWS patients may present for facial and cosmetic surgeries, dental procedures, trabeculectomy, and goniotomy, examination under anaesthesia or seizure control surgery. [ 9 ] The choice of anaesthesia should be influenced by detailed clinical and radiological investigation. General anaesthesia may necessitate avoidance of drugs that can cause an increase in the intraocular pressure, such as succinylcholine and ketamine.…”