Perioperative Care and Outcomes of Bariatric Surgery in VLCADD Patient

Overview

This report describes the first known case of laparoscopic sleeve gastrectomy in a patient with Very Long Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD). Careful multidisciplinary perioperative management enabled significant weight loss and metabolic improvement without long-term complications despite initial postoperative rhabdomyolysis.

Background

VLCADD is an autosomal recessive disorder impairing mitochondrial oxidation of long-chain fatty acids, leading to energy deficiency and risk of rhabdomyolysis during catabolic stress. Patients are vulnerable to metabolic crises triggered by fasting, infection, or surgery. Bariatric surgery in VLCADD is rarely reported due to concerns about catabolic stress and complications. This case highlights the challenges and strategies for safely performing bariatric surgery in this population.

Data Highlights

Key Findings

• First reported case of laparoscopic sleeve gastrectomy in a patient with VLCADD, demonstrating feasibility with multidisciplinary planning.
• Perioperative management focused on minimizing fasting, continuous glucose infusion, normothermia, and avoidance of propofol.
• Postoperative rhabdomyolysis occurred with CK rising to 7300 U/L, managed successfully with aggressive IV fluids and glucose infusion without renal impairment.
• Readmission due to UTI triggered recurrent rhabdomyolysis (CK 13,402 U/L), treated with antibiotics and supportive care leading to recovery.
• At 3-8 months follow-up, patient achieved 25.4% weight loss, improved glycemic control (HbA1c 8.5% to 6.9%), reduced hypertension medication, and no further rhabdomyolysis episodes.
• Tailored nutritional management including moderate calorie restriction, high complex carbohydrates, low long-chain fats, and inclusion of essential fatty acids and MCTs was critical.

Clinical Implications

Bariatric surgery can be safely performed in VLCADD patients with meticulous perioperative planning involving a multidisciplinary team. Key strategies include avoiding prolonged fasting, maintaining glucose infusion, normothermia, and careful anesthetic drug selection to prevent metabolic crises. Postoperative monitoring for rhabdomyolysis and prompt management of complications such as infections are essential to optimize outcomes.

Conclusion

This novel case demonstrates that with tailored perioperative care and nutritional support, bariatric surgery is a viable option for patients with VLCADD, resulting in significant metabolic and weight-related benefits without long-term adverse events.

References

1. Author/Source/Year -- Perioperative Care for a Laparoscopic Sleeve Gastrectomy Patient with Very Long Chain Acyl-CoA Dehydrogenase Deficiency