Copper and Anesthesia: Clinical Relevance and Management of Copper Related Disorders

Table 1

Summary of the major anesthetic considerations in the management of patients with Menkes and Wilson’s diseases. [47–51].

Condition	Anesthetic considerations	Anesthetic management
Menkes disease	Seizures	Preoperatively: Continue anticonvulsant regimen. Check levels.; Intraoperatively: Consider alternate routes of administration including intravenous, rectal, and subcutaneous or nasogastric.
	Gastroesophageal reflux	Consider prophylaxis and endotracheal intubation.
	Difficult intravenous cannulation	Use ultrasound for central intravenous access for placement and to identify vascular abnormalities.
	Capillary fragility	Consider group and hold with cross match where clinically indicated.
	Hypothermia	Use warmed intravenous fluids, theatre temperature regulation, forced air warmers, and humidification of inspired gases.
	Neuraxial anesthesia	Relatively contraindicated due to risk of bleeding from vessel fragility.
	Muscle relaxation	May not be necessary under deep volatile anesthesia because of hypotonia.; Larger doses of vecuronium may be needed secondary to liver enzyme induction.; Suxamethonium may be best avoided due to risk of hyperkalemia.
	Opioid related respiratory depression	Multimodal nonopioid analgesics and careful local anesthesia by wound infiltration.; Consider postoperative respiratory monitoring where clinically indicated.
	Post operative analgesia	Risk of bleeding or hematoma formation with intramuscular or subcutaneous routes.
Wilson’s disease	Neurological and psychiatric	Delayed metabolism of hypnotic sedative drugs may exacerbate neurological or psychiatric postoperatively.
	Hepatic	Impaired metabolism and elimination of anaesthetic agents and morphine.; Reduced mean arterial pressure may further aggravate hepatic function.; Propofol clearance not significantly impaired; Reduce Thiopentone dosage.
	Regional or neuraxial anesthesia	Acceptable in absence or significant coagulopathy (INR > 1.4) or thrombocytopenia (platelets < 100,000 mm^−3).
	Cardiovascular	ECG or echocardiography if coronary artery disease or cardiomyopathy suspected.
	Renal	Fluid and electrolyte abnormalities common. Severe liver dysfunction may result in hepatorenal syndrome which may require dialysis perioperatively.
	Muscular	Avoid or reduce dosage of nondepolarizing neuromuscular blockers (NDMB).