M. Bowler1, D. Anjilivelil2

1Royal Manchester Children’s Hospital, UK

2Royal Oldham Hospital, UK

Introduction and aims

Diagnostic muscle biopsy is required for diagnosis of a number of rare conditions from a variety of pathological groups including myopathies, neuropathies, metabolic and mitochondrial disorders, seizure disorders and cardiomyopathies.  To facilitate the procedure in children, some form of deep sedation or general anaesthesia is required.  Risks of choosing the wrong technique out of a volatile or non-volatile anaesthesia can risk malignant hyperthermia (MH)/anaesthetic induced rhabdomyolysis (AIR) or propofol infusion syndrome (PRIS).  A previous study from Boston Childrens Hospital reviewed a 20 year cohort comparing pre and post biopsy diagnosis and complications(1).  Our aim was to replicate this study on our own cohort, identify non-concordance between pre and post biopsy diagnosis and any complications.  We utilised the existing guideline from Royal Melbourne Childrens Hospital as an audit standard (2).


We performed an Ormis Database search for a key-word “muscle biopsy” between 1/1/2016 and 30/12/2020 of all operations occurring at Royal Manchester Childrens Hospital.  The returned results had paper notes requested and all available notes were reviewed by senior anaesthetic doctors.  Data was entered directly into a Microsoft Excel and data analysis was also performed within Excel.


The database search returned 67 anaesthetics of which notes were available for 54 patients representing 56 procedures.  The average age of biopsy was 7.1 years.  Preoperative investigations were variable with compliance with guidelines being CK 76%, ECG 44%, ECHO 41%, lactate 44%, chest xray 16.1 and PFTs 8.9%.  A suspected preoperative diagnosis was present in 61%.  Anaesthetic technique was TIVA without volatile in 30% of cases with the remainder a volatile anaesthetic.  Airway device was predominantly LMA.  Only 4 patients suffered an identifiable complication.  These included respiratory obstruction, oxygen requirement and low postoperative heart rate.  12 patients had an inappropriate anaesthetic technique for the subsequent post biopsy diagnosis.  This included 4 children with a muscular dystrophy receiving a volatile anaesthetic (risk of AIR), 7 children receiving volatile with an RYR defect or MH linked condition (risk of MH) and 1 child with a mitochondrial myopathy receiving TIVA (risk of PRIS).  None of these children developed any of these complications.  Further analysis of the cohort revealed that 6/54 children are now deceased.  This represents a 9.2% 4 year mortality.




Blanket preoperative testing of all children undergoing muscle biopsy is likely to be an inappropriate use of resources.  However, presumptive diagnosis is important, as this will aid decision-making for relevant preoperative investigations for the presumptive diagnosis and then a safe anaesthetic technique.  Whilst our complication rate is quite low, the dangers of causing malignant hyperthermia, anaesthetic induced rhabdomyolysis, propofol infusion syndrome and other complications should not be understated.  It is important for a preoperative multi-disciplinary approach to avoid these complications.


1 Shapiro F, Athiraman U, Clendenin DJ, Hoagland M, Sethna NF. Anesthetic management of 877 pediatric patients undergoing muscle biopsy for neuromuscular disorders: a 20-year review. Paediatr Anaesth. 2016;26(7):710-721.

2 Anaesthesia for Muscle Biopsy Clinic Practice Guideline, Royal Melbourne Childrens Hospital. [accessed 30/01/2024]. Available from: https://www.rch.org.au/uploadedfiles/main/content/anaes/master_muscle_biopsy_cpg3.pdf

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