[PDF] Management of surgery in children with urea cycle disorders

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


Patients with urea cycle disorders who are usually well controlled 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 following instructions apply to patients with:

  • Ornithine carbamyl transferase (OCT or OTC) deficiency
  • Citrullinaemia (Argininosuccinic acid synthase deficiency)
  • Argininosuccinic aciduria (ASA, Argininosuccinic acid lyase deficiency)
  • Arginase deficiency
  • Carbamyl phosphate synthase deficiency
  • N-acetylglutamate synthetase deficiency

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