Anaesthesia for Sturge-Weber syndrome

Area of Science:

• Ophthalmology
• Pediatric Surgery
• Neurology

Background:

• Sturge-Weber syndrome is a rare congenital disorder characterized by a port-wine stain, neurological abnormalities, and potential ocular complications.
• Congenital glaucoma and buphthalmos are serious conditions that can lead to vision loss if not treated promptly.
• This case highlights the anesthetic and surgical considerations for managing ocular conditions in infants with complex syndromes.

Observation:

• A 6-month-old male infant diagnosed with Sturge-Weber syndrome presented with congenital glaucoma and bilateral buphthalmos.
• Physical examination revealed facial hemangioma involving the trigeminal nerve distribution, left-sided megalocornea, and bilateral buphthalmos.
• Ophthalmic examination under general anesthesia preceded a trabeculotomy procedure performed two days later.

Findings:

• General anesthesia was induced using halothane, oxygen, and nitrous oxide, with muscle relaxation achieved via atracurium.
• The patient was intubated gently, and vital signs remained stable throughout the surgical procedure.
• Post-operative recovery was uneventful, with no observed complications.

Implications:

• This case demonstrates the feasibility and safety of surgical intervention for congenital glaucoma and buphthalmos in infants with Sturge-Weber syndrome.
• Careful anesthetic management and surgical technique are crucial for successful outcomes in these complex pediatric cases.
• Early diagnosis and treatment of ocular manifestations in Sturge-Weber syndrome are essential for preserving vision.