[PDF] Management of surgery in children with an organic acidiaemia and MSUD

British Inherited Metabolic Disease Group
British Inherited Metabolic Disease Group (BIMDG)
Publication date:
22 May 2013


Patients with organic acidaemias who are usually well controlled can easily decompensate during surgery, precipitated by a combination of stress and fasting. 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 that are also available on the BIMDG website.
The guideline applies  to patients with:

  • Methylmalonic acidaemia
  • Propionic acidaemia
  • Isovaleric acidaemia
  • Glutaric acidaemia type I
  • Maple syrup urine disease
  • Other less common disorders

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