M. R. Bowler, J. Garbarino, J. Kenth

Royal Manchester Children's Hospital, UK


Argininosuccinic Aciduria (ASA) is an inborn error of metabolism falling into the urea cycle defects group.  It’s cause is the absence of the enzyme arginosuccinate lyase.  This catalyses the conversion of argininosuccinic acid to arginine.  Typically children develop hyperammonaemic crises typified by lethargy, vomiting and irritability.  They may also develop seizures, breathing problems, cerebral oedema and hepatomegaly(1).  Within the perioperative setting the BIMDG perioperative guidelines emphasize the importance of avoiding prolonged fasting using 10% dextrose containing fluids, avoiding certain drugs and minimising the stress response(2).  We present a 19-year experience of the anaesthetic management of these patients.


We utilised an audit format with standards identified from the BIMDG urea cycle  guidelines.  These were that all children should receive preoperative polycal drinks or dextrose containing IV fluids and all children should have dextrose containing IV fluids intraoperatively.  Data was collected from patient held upon a RMCH metabolic department database for procedures occurring between 01/05/2000 and 31/05/2019 at RMCH.  Both paper and electronic notes were reviewed by 2 independent reviewers.  Where differences in results occurred a third reviewer was used.  Results were collated straight into Microsoft Excel.


A total of 16 children had their notes reviewed of which 9 had  an operation.  Those 9 had a total of 24 operations of which 22 operative episodes were available.  The average age at operation was 2.6 years and average ASA 3.  9 of the 22 procedures were urgent, with the remainder elective.  In the perioperative period 90% of the children received either preoperative polycal drink or IV fluid preoperatively.  95% of children received an infusion of 10% dextrose with 0.45% NaCl.  All children underwent volatile anaesthesia.  13 cases used muscle relaxants for the placement of an ETT without report of prolonged neuromuscular block.  There were no reports of drug side-effects.  3 children suffered an intraoperative complication including either difficult facemask ventilation and intubation, laryngospasm and electrolyte imbalance.  5 children suffered a postoperative complication including either airway and ventilation problems, hypotension and low blood sugars.  There were no episodes of metabolic decompensation resulting from the procedure or anaesthesia.


There are currently no case reports discussing the management of ASA in the setting of anaesthesia for an operative procedure.  Our dataset whilst demonstrating that there is a higher than normal incidence of perioperative complication, identifies that these are more likely due to underlying organ dysfunction/abnormality as a result of the ASA.  The concern of causing a metabolic decompensation due to surgical stress anaesthetic drugs or the starvation process did not seem to occur.  We feel that our dataset shows that risk of decompensation in a well child with ASA is unlikely if BIMDG perioperative guidelines are observed with minimising fasting and stress.


1 - Argininosuccinic Aciduria [Internet]. Washington: NORD; 1986 [updated 2023; cited 2024 Feb 4]. Available from: https://rarediseases.org/rare-diseases/argininosuccinic-aciduria/.

2 - Surgery in urea cycle disorders [Internet]. BIMDG UK; 2017. Available from: https://www.bimdg.org.uk/store/guidelines/Management_of_surgery_in_children_with_urea_cycle_disorders__215051_09092016.pdf

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