Perioperative management of pediatric patients with inborn errors of metabolism during liver transplantation

Table 3 Anesthetic implications in inherited metabolic disorders during liver transplantation

Metabolic defect involving	Disorders	Specific anesthetic considerations
Carbohydrate	Glycogen storage disease I	Dextrose infusion. In case of hyperglycemia-insulin to be started along with dextrose infusion. Avoid Ringer’s Lactate. Difficult IV access and airway
	Glycogen storage disease III and IV	Dextrose infusion. In case of hyperglycemia - insulin to be started along with dextrose infusion. Avoid Succinylcholine. Difficult IV access and airway
Amino acids	Tyrosinemia type I	Dextrose infusion. Fracture prone hence caution while positioning
	Maple syrup urine disease	Ensure BCAA free supplements, isoleucine and valine supplements are give preoperatively. Dextrose infusion. Avoid Ringer’s lactate and resuscitation with Albumin. Hemodialysis standby
	Urea cycle disorders	Intraoperatively use Ammonia scavenging agents such as sodium benzoate and/or phenylbutarate and arginine infusion along with serial ammonia monitoring. Dextrose infusion along with intralipid infusion and low protein parenteral nutrition. Avoid Ringer’s lactate and resuscitation with Albumin. Hemodialysis standby
	Methyl malonic Acidemia	Avoid propofol, atracurium, cisatrcurium, Nitrous oxide, Naproxen, Ketoprofen. Intraoperatively use ammonia scavenging agents such as sodium benzoate and Carnitine along with serial ammonia monitoring. Dextrose infusion. Avoid Ringer’s lactate and resuscitation with Albumin. Metronidazole prophylaxis. Hemodialysis standby. Continue carnitine postoperatively. Renal sparing immunosuppression
	Propionic acid acidemia
Fatty acids	Fatty acid oxidation defects	Dextrose infusion. Perioperative seizure prophylaxis. Avoid Ringer’s lactate and resuscitation with Albumin. Hemodialysis standby
Peroxisomal	Primary hyperoxalosis type 1	Perioperative CRRT. Platelet dysfunction. Hypertensive crises. Renal sparing Immunosuppression. Fracture prone hence caution while positioning
Bile transporter defects	PFIC-1, VI	Malnutrition/growth retardation. Fracture prone hence caution while positioning
Mitochondrial disorders and energy cycle defects	DNA depletion defect	Perioperative seizure prophylaxis. Avoid succinylcholine, isoflurane and propofol
Bilirubin conjugation	Crigler Najjar syndrome	Malnourished/growth retardation. Fracture prone hence caution while positioning
Cholesterol excretion	Familial hypercholesterolemia	May need planning for lipid apheresis 2-4 weeks prior to surgery. If on Preoperative blood thinners, appropriate bridging and safe discontinuation to be planned. Anticipate Difficult airway due to facial asymmetry and xanthomas. Anticipate difficult vascular access Perioperative thromboembolic events