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Condition | Anesthetic considerations | Anesthetic management |
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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. |
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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). |
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