Anesthetic Mx in a child with Sturge-Weber syndrome
Sturge- Weber syndrome (SWS) is a neurocutaneous disorder, presents with a facial port-wine stain in the ophthalmic distribution of the trigeminal nerve, glaucoma, vascular eye abnormalities, or an ipsilateral occipital leptomeningealangioma.

The present case has been reported in the Journal of Anaesthesiology Clinical Pharmacology.

A 2-year-old, male, known case of SWS was posted for syringing and probing for nasolacrimal duct obstruction. Detailed preoperative assessment revealed history of delayed milestones and recurrent episodes of focal motor seizures with last episode 5 months back (on phenytoin). The mouth opening was 1.8 cm with multiple hemangiomas observed in oral cavity. There were three more hemangiomas on chest, arm, and face. Systemic examination did not reveal anything significant. The computed tomography scan of brain revealed choroidal hemangioma.

The authors planned to use a supraglottic device (ProSeal laryngeal mask airway [PLMA]) for intraoperative ventilation. After preoxygenation, attaching the achieving inhalational induction of anesthesia with sevoflurane and securing an intravenous line Inj. Glycopyrrolate (5 μg/kg) and inj. Fentanyl (2 μg/kg) were given as premedication. A PLMA size 2, with stylet in situ technique, was used to secure the airway.

In this technique, stylet was introduced through drain tube till the distal-most end of PLMA. The PLMA was bent 90° at shaft and adequate lubricant jelly was applied on the dorsal surface. PLMA was inserted very gently avoiding any vigorous movement. After removal of stylet, PLMA's position was confirmed by insertion of adult fiberscope.

Anesthesia was maintained with sevoflurane (1–1.5 MAC), 66% nitrous oxide in oxygen with spontaneous ventilation. At the end of approximately. 25-minute, the PLMA was gently removed in deeper plane and postextubation oxygenation for 5 minutes was done. The recovery was uneventful and patient was discharged after 6 hours.

Key takeaways:-
The key to safe anesthetic management in such patients is by controlling the convulsive disorder perioperatively, attenuation of hemodynamic responses during airway manipulation and surgery, careful intubation/extubation, avoiding trauma to the angiomatous lesions, and adequate pain relief.

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