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.



malignant hyperthermia

avoid medications or anesthetics that may trigger hyperthermia; monitor body temperature; monitor for hypercapnea


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


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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