Death caused by perioperative fasting and sedation in a child with unrecognized very long chain acyl-coenzyme A dehydrogenase deficiency

An adopted 31/2-year-old girl with no prior medical problems died after a r outine dental procedure. More than 2 years later, acylcarnitine analysis of dried blood found on her bedding revealed she had very long chain acyl-coe nzyme A dehydrogenase (VLCAD) deficiency. Perioperative oral fasting, witho ut intravenous administration of glucose, may be detrimental to children wi th certain metabolic and endocrine disorders. Newborn screening by tandem m ass spectrometry will detect disorders of fatty acid oxidation such as VLCA D and allow early and preventive treatment.