Purpose: Walker-Warburg syndrome is a rare autosomal recessive congenital muscular dystrophy presenting with hydrocephalus, type II lissencephaly, cerebellar malformation, and ocular anomalies. Here, we describe the use of perioperative total intravenous anesthesia in a pediatric patient with Walker-Walburg syndrome.
Clinical features: A 2-month-old girl with Walker-Walburg syndrome was admitted for urgent ventriculoperitoneal shunt insertion. Anesthesia was induced using intravenous atropine (100 μg), ketamine (10 mg), and fentanyl (15 μg). The patient was monitored for various clinical parameters based on American Society of Anesthesiologists standards. Anesthesia was maintained using intermittent doses of 0.5 mg intravenous midazolam and 5-10 μg fentanyl in incremental boluses. The postoperative course was uneventful.
Conclusions: To our knowledge, no published cases have reported the use of total intravenous anesthesia in patients with Walker-Walburg syndrome who have potential risks for anesthetic-induced malignant hyperthermia. This report described the key features, potential anesthetic management plan, and current literature review of Walker-Walburg syndrome.
Keywords: Congenital muscular dystrophy; malignant hyperthermia; perioperative; ventriculoperitoneal shunt.
Copyright: © 2022 Saudi Journal of Anesthesia.