Walker-Warburg Syndrome is a rare form of congenital muscular dystrophy. A patient with Walker-Warburg Syndrome may face a number of challenges in perioperative management.
Problem
Solution
malignant hyperthermia
avoid medications or anesthetics that may trigger hyperthermia; monitor body temperature; monitor for hypercapnea
rhabdomyolysis
avoid medications or anesthetics that may trigger rhabdomyolysis; monitor blood for evidence of rhabdomyolysis (color, CK)
difficult intubation due to malformations (cleft palate, short neck, micrognathia, receding mandible, etc)
identify problems and be prepared for a difficult intubation; consider inhalation induction with sevoflurane to avoid suppression of spontaneous ventilation
delayed gastric emptying; reflux and aspiration
evaluate for gastric retention and reflux; consider preoperative fasting if present
central and/or obstructive apnea
avoid preoperative sedation; monitor oxygen saturation during and after surgery; consider supplemental oxygen
seizures
monitor during and after surgery; avoid hypoxia and drugs that might trigger seizures
raised intracranial pressure in association with hydrocpehalus
consider paralyzing patient; avoid coughing; adequately ventilate to avoid hypercapnea
hyperkalemia due to sudden release of potasssium from myopathic muscles
monitor ECG and blood
weak respiratory muscles
avoid nondepolarizing muscle relaxants; monitor oxygen and carbon dioxide
Medications to avoid may include:
(1) succinylcholine
(2) opioids
(3) most volatile anesthetics
(4) sedatives
(5) nondepolarizing muscle relaxants
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