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<p>Very Long Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD) is a rare inherited disorder of mitochondrial fatty acid β-oxidation that predisposes patients to metabolic crises, rhabdomyolysis, and cardiomyopathy. Surgical stress, fasting, and anaesthesia may precipitate metabolic decompensation. We present the case of a 65-year-old female with late-onset VLCADD, multiple comorbidities, and chronic kidney disease (CKD) stage 3 who underwent laparoscopic sleeve gastrectomy. Despite perioperative glucose infusion, normothermia, and careful anaesthetic planning, she developed postoperative rhabdomyolysis. This case highlights perioperative challenges in VLCADD, provides practical strategies for anaesthetic and nutritional management, and, to our knowledge, represents the first report of bariatric surgery in a patient with VLCADD. Long-term follow-up demonstrated sustained weight loss, improved cardiometabolic profile, and stable muscle and renal function. </p>

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Perioperative Management of a Patient With Very Long Chain Acyl-CoA Dehydrogenase Deficiency Undergoing Laparoscopic Sleeve Gastrectomy: First Report of Bariatric Surgery in VLCADD

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  • 出版模式：

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  • 发表日期：

    2025年12月5日

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  • Mirza Anwar Baig,
  • Bennedict Williams

摘要

Very Long Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD) is a rare inherited disorder of mitochondrial fatty acid β-oxidation that predisposes patients to metabolic crises, rhabdomyolysis, and cardiomyopathy. Surgical stress, fasting, and anaesthesia may precipitate metabolic decompensation. We present the case of a 65-year-old female with late-onset VLCADD, multiple comorbidities, and chronic kidney disease (CKD) stage 3 who underwent laparoscopic sleeve gastrectomy. Despite perioperative glucose infusion, normothermia, and careful anaesthetic planning, she developed postoperative rhabdomyolysis. This case highlights perioperative challenges in VLCADD, provides practical strategies for anaesthetic and nutritional management, and, to our knowledge, represents the first report of bariatric surgery in a patient with VLCADD. Long-term follow-up demonstrated sustained weight loss, improved cardiometabolic profile, and stable muscle and renal function.