Duchenne's Muscular Dystrophy Anaesthesia Final Exam Case | #anesthesia #duchennemusculardystrophy

Thanks Dr Ralph for a great exam performance!

The Stem
13yo male presents to emergency department in a small rural hospital (2 theatres, 1 ward, no HDU/ICU) with right iliac fossa abdominal pain.

Past history
Duchenne’s muscular dystrophy
Wheelchair bound
Severe scoliosis
50kg recent weight

Vitals
HR 110 SR
BP 100/70
Sats 95% or 2L NP oxygen
Resp rate 40
Temp 37.4

Bloods
FBE 120
WCC 13 x 109/L
Plt 400 x 109/L

The surgeon is concerned about a ruptured appendix and would like to urgently go to theatre for a laparoscopic appendicectomy.

His father is very concerned about the anaesthetic.

What is Duchenne’s muscular Dystrophy and what are the concerning aspects of this disease for the anaesthetic?

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[E]

Thank you Dr. Ralph for being willing to share your off-the-cuff performance. This was very instructive and I hope I can eventually do as good a job.

[gracehinton]

A very difficult case! Well answered. I think gas induction would be too high risk for me and I would opt for further fluid bolus and then US guided IV. Call a colleague for assistance with IV in this case as it is super important!!

[tammybambini]

Nice case presentation, thank you!
Given that a tertiary care center is only 30 minutes away and he´s relatively stable I´d have opted to transfer the patient (probably after placing arterial and central venous lines) - any complications (like hyperkalemia in rhabdomyolysis) can be better treated there (dialysis instead of temporization using Ca++/Glucose-Insuline/NaBic), also they might have more experience with cases like these (compared to your local surgeon and yourself - know your and your institutions limits!).
Instead of i.o. access you might want to consider placing a central line in the (left, right side is close to planned surgical site) femoral vein - easy puncture site, especially ultrasound guided, and much less invasive - and very little chance of complications.
Please refrain from giving NaCl 0.9% solution (there´s nothing "normal" in normal saline) - that´s obsolete nowadays (may precipitate hyperchloremic acidosis). Just use balanced electrolyte solutions.
Use your usual (1.2mg/kg) dose of ROCuronium for muscle relaxation and monitor TOF - the onset of muscle relaxation in DMD is described as "variable and delayed" - and consider placing a gastric tube before RSI, if patient tolerates it. I´d discuss prolonged ventilation until NMB disappears versus reversal using sugammadex.

[yilbertcintrasombert]

X linked recessive disorder, associated with distrophin deficience, progressive muscle weakness..