Peri-operative Management of a Child with Maple Syrup Urine Disease in a Non-tertiary Paediatric Hospital

Abstract

Introduction: Maple Syrup Urine Disease (MSUD) is a rare, autosomal recessive aminoacidopathy. We report the peri-operative management of an eleven year old girl, with well controlled MSUD who presented to a nonpaediatric, tertiary hospital for a non-emergency procedure, under general anaesthetic. Case Presentation: An 11 year old girl presented to the Emergency Department with a history of nasal bone trauma. She was admitted for a manipulation of nasal bones (MNB) under general anaesthetic (GA). She had a history of MSUD diagnosed on day three of life. The patient was neurologically intact and had leucine levels within the accepted range prior to her surgery. In times of illness her leucine levels were generally well controlled. The patient was admitted the night prior to surgery following her normal evening meal. Her urine was checked for ketones on admission and this was negative. Overnight carbohydrate management was instituted. Her surgery was performed first on the theatre list. The procedure lasted 14 min and was uneventful. Oral intake was recommenced at 10.00 am. Her urine was again checked for ketones post-operatively and was negative. The patient was well over the weekend and her leucine level post-surgery were within normal limits. Discussion: MSUD is a rare disease. There is limited experience of the conduct of anaesthesia in these patients outside of specialist paediatric centers. Most reports advised transfer to a tertiary paediatric center. We demonstrated that low risk surgery can be carried out safely in these patients.