Anesthetic management for a patient with very-long-chain acyl-coenzyme A dehydrogenase deficiency

Iwata, Koichi; Tanabe, Kumiko; Sugiyama, Y.; Tanaka, Midori; Takenaka, Motoyasu; Iida, Hiroki

doi:10.1007/s00540-012-1457-8

Cited by 7 publications

(11 citation statements)

References 5 publications

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“…Propofol is often considered contraindicated in VLCADD . It can contain up to 100 mg of fat per milliliter, most of which is long chain .…”

Section: Reviewmentioning

confidence: 99%

“… and Iwata et al . . This has resulted in the widespread, erroneous recommendation to avoid volatile anesthesia in VLCADD.…”

Section: Reviewmentioning

confidence: 99%

“…Postoperative myalgia and raised creatine kinase are important signs of rhabdomyolysis, and the use of depolarizing agents could potentially cause diagnostic confusion. Avoidance of neuromuscular blockade or monitoring of blockade and ensuring full reversal prior to emergence from anesthesia are specifically mentioned in case reports as it is unknown whether VLCADD patients have altered sensitivity to neuromuscular blockade.…”

Section: Reviewmentioning

confidence: 99%

“…We have compared the anesthetic techniques of three case reports discussing four patients and an unpublished case. These patients are an 8‐month‐old male for a percutaneous endoscopic gastrostomy , a 9‐year‐old male for a resection of a phimosis both of whom had the neonatal form of the disease, an 11–year‐old female with the childhood form for placement of an intravenous access device , a 28‐year‐old female with the adolescent onset form for a hysteroscopic myomectomy , and 46 years old who also has the adolescent onset form of the disease, who presented for a hemithyroidectomy (Redshaw CL, Stewart C, unpublished data). We will summarize the anesthetic techniques used (Table ) and examine the underpinning evidence to provide a more accurate safety profile of anesthetic agents.…”

Section: Introductionmentioning

confidence: 99%

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Anesthetic agents in patients with very long‐chain acyl‐coenzyme A dehydrogenase deficiency: a literature review

Redshaw

Stewart

2014

Pediatric Anesthesia

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Very long-chain acyl-coenzyme A dehydrongenase deficiency (VLCADD) is a rare disorder of fatty acid metabolism that renders sufferers susceptible to hypoglycemia, liver failure, cardiomyopathy, and rhabdomyolysis. The literature about the management of these patients is hugely conflicting, suggesting that both propofol and volatile anesthesia should be avoided. We have reviewed the literature and have concluded that the source papers do not support the statements that volatile anesthetic agents are unsafe. The reports on rhabdomyolysis secondary to anesthesia appear to be due to inadequate supply of carbohydrate not volatile agents. Catabolism must be avoided with minimal fasting, glucose infusions based on age and weight, and attenuation of emotional and physical stress. General anesthesia appears to be protective of stress-induced catabolism and may offer benefits in children and anxious patients over regional anesthesia. Propofol has not been demonstrated to be harmful in VLCADD but is presented in an emulsion containing very long-chain fatty acids which can cause organ lipidosis and itself can inhibit mitochondrial fatty acid metabolism. It is therefore not recommended. Suxamethonium-induced myalgia may mimic symptoms of rhabdomyolysis and cause raised CK therefore should be avoided. Opioids, NSAIDS, regional anesthesia, and local anesthetic techniques have all been used without complication.

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“…Propofol is often considered contraindicated in VLCADD . It can contain up to 100 mg of fat per milliliter, most of which is long chain .…”

Section: Reviewmentioning

confidence: 99%

“… and Iwata et al . . This has resulted in the widespread, erroneous recommendation to avoid volatile anesthesia in VLCADD.…”

Section: Reviewmentioning

confidence: 99%

Section: Reviewmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Anesthetic agents in patients with very long‐chain acyl‐coenzyme A dehydrogenase deficiency: a literature review

Redshaw

Stewart

2014

Pediatric Anesthesia

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“…There are published reports of volatile anesthetics safely used in adults with very long-chain acyl-coenzyme A dehydrogenase deficiency. 4 A single report of volatile-associated rhabdomyolysis in a pediatric patient was likely due to inadequate caloric supplementation rather than the anesthetic agent. 2 All anesthetics inhibit complex I, potentially obstructing both fatty acid and glucose metabolism.…”

Section: Discussionmentioning

confidence: 99%

Volatile anesthesia for a pediatric patient with very long‐chain acyl‐coenzyme A dehydrogenase deficiency: A case report

Hess

Rice

Jochman

et al. 2018

Pediatric Anesthesia

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The perioperative transition of serum biomarkers of a 1.5-year-old boy with very-long-chain acyl-CoA dehydrogenase deficiency

Awano

Yamada

et al. 2021

Molecular Genetics and Metabolism Reports

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Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCADD, OMIM 201475 ) is a congenital fatty acid oxidation disorder. Individuals with VLCADD should avoid catabolic states, including strenuous exercise and long-term fasting; however, such conditions are required when undergoing surgery. The perioperative management of VLCADD in infants has rarely been reported and details regarding the transition of serum biomarkers reflecting catabolic status have not been disclosed. Herein, we present the perioperative clinical and biological data of cryptorchidism in a 1.5-year-old boy with VLCADD. The patient was diagnosed through newborn screening and his clinical course was very stable. Genetic testing of ACADVL revealed compound heterozygous variants c.506 T > C (p.Met169Thr) and c.606-609delC (p.L216*). The enzyme activity of the patient with VLCAD was only 20% compared to that of healthy control. Left orchiopexy for the pediatric cryptorchidism was planned and performed at 1 and a half year of age. Induction anesthesia involved thiopental, fentanyl and rocuronium. The glucose infusion rate was maintained above 6.6 mg/kg/min starting the day before surgery until the operation was completed. Anesthesia was maintained with sevoflurane at approximately 2%. The serum concentration of tetradecenoylcarnitine were stable during the operation, ranging between 0.08 and 0.19 μM (cutoff <0.2 μM), and never deviated from the reference range. Concentration of other serum biomarkers including free fatty acid, 3-OH-butyrate, and creatine kinase, remained similarly unchanged. In this report, we describe the uneventful perioperative management of unilateral orchiopexy for left cryptorchidism in a 1.5-year-old boy with VLCADD using sufficient glucose infusion and volatile anesthesia.

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Anesthetic management for a patient with very-long-chain acyl-coenzyme A dehydrogenase deficiency

Cited by 7 publications

References 5 publications

Anesthetic agents in patients with very long‐chain acyl‐coenzyme A dehydrogenase deficiency: a literature review

Anesthetic agents in patients with very long‐chain acyl‐coenzyme A dehydrogenase deficiency: a literature review

Volatile anesthesia for a pediatric patient with very long‐chain acyl‐coenzyme A dehydrogenase deficiency: A case report

The perioperative transition of serum biomarkers of a 1.5-year-old boy with very-long-chain acyl-CoA dehydrogenase deficiency

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