Authors: X Liang, MD, PhD; T Carter, CRNA; AJ de Armendi, MD, PhD, MBA

Anesthetic Management for a Pediatric Patient with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency

Background:

	Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) is an autosomal recessive disorder involving the initial steps in fatty acid metabolism with potential for hypoglycemia, rhabdomyolysis, liver failure, fatty acid intermediate toxicity, and mitochondrial dysfunction. There is a paucity of articles related to the anesthetic management of VLCADD. Authors suggest avoidance of propofol and volatile anesthetic.[1,2 3] Opioids, NSAIDS, and regional anesthesia have been administered safely. We present an alternative approach to the anesthetic management of a pediatric patient with VLCADD with the administration of etomidate, remifentanil, ketamine, rocuronium, sugammadex, dexmedetomidine and fentanyl.
Case Report:
	A 14 year old patient (80kg) with childhood VLCADD who presented for port insertion. PMHx included multiple hospitalizations for recurrent rhabdomyolysis due to VLCADD exacerbations with food poisoning, menses, exercises and dehydration. Plasma creatinine kinase (CK) levels ranged 30-57,000 U/L during episodes. PSHx was significant for G-tube placement.  Medications included: Paroxetine hydrochloride and levocarnitine.
	Anesthesia Management
The patient was admitted the day prior to surgery and anesthesia was consulted. Blood was sent for baseline levels of liver/kidney function, electrolytes, glucose, and CK. All results were WNL except for a CK (520 U/L). IVF with D10% ½NS at 125 ml/h was initiated.  The patient refused local anesthesia/sedation.  General anesthesia was discussed with the family. 
On the day of surgery, IVF with D10% LR 200ml/h (glucose 4 mg/kg/min) was administered. Patient was premeditated with midazolam 2 mg IV. General anesthesia was induced with morphine 10 mg IV slowly, lidocaine 50 mg IV, etomidate 24mg IV, and rocuronium 80 mg IV.   TIVA was maintained with a remifentanil infusion at 0.1-0.2 mcg/kg/min and ketamine 50 mg lasting 102 minutes.  Sugammadex 160mg IV antagonized the NM block. Fentanyl (40mcg IV) and dexmedetomidine (20 mcg IV) were administered at extubation.  The PACU course was unremarkable. Intra-op and post-operative laboratory showed no signs of hypoglycemia, hyperkalemia or CK elevation (Table 1). Patient was discharged home the following day without any complications.
Discussion:

VLCADD is associated with abnormal fatty acid metabolism especially during stress.  The literature has a paucity of articles on the anesthetic management for patients with VLCADD. Glucose infusion to avoid hypoglycemia and dehydration is important.  Opioids, benzodiazepine, NSAIDS, local anesthetic had been safely used. In view of the lack of reports in the literature for the anesthetic management for pediatric patients with VLCADD, we present a successful alternative anesthetic management never previously reported. 


07 / Anesthetic Management for a Pediatric Patient with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD)