[PDF] Management of surgery in children with disorders of fatty acid oxidation

Source:
British Inherited Metabolic Disease Group
Publisher:
British Inherited Metabolic Disease Group (BIMDG)
Publication date:
01 May 2013

Abstract

Patients with disorders of fatty acid oxidation are usually well controlled but can easily decompensate during surgery, particularly if catabolism is precipitated by fasting and surgery. Elective surgery in these patients is usually best done at the hospital with the regional metabolic unit. It is important to follow an appropriate protocol, minimising catabolism by providing adequate amounts of carbohydrate. This protocol should be used in conjunction with the emergency regimens on the BIMDG website.

The guideline applies to patients with:

  • Very long chain acyl CoA dehydrogenase deficiency (VLCAD)
  • Long chain 3-hydroxy-acyl CoA dehydrogenase deficiency (LCHAD)
  • Medium chain acyl CoA dehydrogenase deficiency (MCAD)
  • Multiple acyl CoA dehydrogenase deficiency (MADD)
  • Carnitine palmitoyl transferase deficiency I & II
  • And others

Sections of the guideline include: history taking and preparation, pre-operative management, extra intra-operative instructions, post-operative procedure.