Clinical Scorecard: Perioperative Care for a Laparoscopic Sleeve Gastrectomy Patient with Very Long Chain Acyl-CoA Dehydrogenase Deficiency: A Novel Case of Bariatric Surgery in VLCADD

At a Glance

Key Highlights

• VLCADD patients are at high risk of rhabdomyolysis and metabolic decompensation triggered by fasting, infection, surgery, or catabolic stress.
• Perioperative management requires minimising fasting, continuous glucose infusion, normothermia, and avoidance of propofol and succinylcholine.
• Bariatric surgery is feasible in VLCADD with tailored nutritional support, close metabolic monitoring, and multidisciplinary coordination.

Guideline-Based Recommendations

Diagnosis

• Recognize VLCADD phenotypes: infantile cardiomyopathy, childhood hypoketotic hypoglycemia, adult myopathic form with exertional myalgia.
• Monitor creatine kinase (CK) levels pre- and postoperatively to detect rhabdomyolysis.
• Assess renal function and cardiac status preoperatively.

Management

• Avoid prolonged fasting; allow carbohydrate drinks up to 2 hours preoperatively.
• Initiate intravenous 10% dextrose with variable rate insulin infusion perioperatively to maintain glucose levels.
• Use volatile anesthetics (sevoflurane, desflurane) and remifentanil; avoid propofol infusions and succinylcholine.
• Maintain normothermia with active warming and warmed IV fluids.
• Employ non-depolarizing muscle relaxants such as rocuronium; reverse with sugammadex.
• Provide tailored nutritional management with moderate calorie restriction (>1200 kcal/day), high complex carbohydrates, low long-chain fats, and supplementation with essential fatty acids and medium-chain triglycerides (MCTs).
• Aggressively manage rhabdomyolysis with IV fluids to prevent pigment nephropathy.

Monitoring & Follow-up

• Continuous blood glucose monitoring intra- and postoperatively.
• Serial creatine kinase and renal function tests to detect and manage rhabdomyolysis and renal injury.
• Electrolyte monitoring, especially potassium and phosphate.
• Close observation in a high dependency or intensive care setting postoperatively.

Risks

• Rhabdomyolysis triggered by catabolic stress, fasting, hypothermia, or inappropriate anesthetic agents.
• Metabolic acidosis and renal injury secondary to muscle breakdown.
• Postoperative infections such as urinary tract infections can precipitate metabolic crises.
• Rapid weight loss and caloric restriction post-bariatric surgery may exacerbate metabolic instability.

Patient & Prescribing Data

Adult VLCADD patient with obesity, type 2 diabetes, hypertension, and chronic kidney disease undergoing laparoscopic sleeve gastrectomy.

Multidisciplinary perioperative planning with avoidance of fasting, glucose infusion, careful anesthetic selection, and tailored nutritional support enabled safe surgery and improved metabolic and weight outcomes.

Clinical Best Practices

• Engage a multidisciplinary team including bariatric surgeons, anesthetists, dietitians, internal medicine, cardiology, and metabolic specialists.
• Minimize fasting periods and provide carbohydrate drinks up to 2 hours before surgery.
• Use remifentanil and volatile anesthetics; avoid propofol infusions and succinylcholine to reduce rhabdomyolysis risk.
• Maintain normothermia intra- and postoperatively with active warming techniques.
• Monitor CK, renal function, electrolytes, and glucose closely perioperatively and postoperatively.
• Implement aggressive IV fluid therapy promptly if rhabdomyolysis occurs to prevent renal complications.
• Provide individualized nutritional plans emphasizing moderate calorie restriction, high complex carbohydrates, low long-chain fats, and supplementation with essential fatty acids and MCTs.
• Plan postoperative care in a high dependency unit for close monitoring and early intervention.

References

• VLCADD Phenotypes and Pathophysiology
• Rhabdomyolysis in VLCADD
• Perioperative Management in Fatty Acid Oxidation Disorders
• Anesthetic Considerations in VLCADD
• Bariatric Surgery and Metabolic Risks in VLCADD
• Nutritional Management in VLCADD Post-Bariatric Surgery
• Dietary Recommendations for VLCADD