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Combined General and Regional Anesthesia for a Patient With Duchenne Muscle Dystrophy With an Implanted Left
Amr Elhamrawy1, Mauricio Arce Villalobos1,2, Grant Heydinger1,2
1Department of Anesthesiology and Pain Medicine, Nationwide Children's Hospital, Columbus, OH, USA.
Insights
Duchenne muscular dystrophy (DMD) poses significant perioperative challenges. This case highlights a novel anesthetic approach combining total intravenous anesthesia with regional blocks for a patient with DMD and a ventricular assist device.
Area of Science:
- Neurology
- Cardiology
- Anesthesiology
Background:
- Duchenne muscular dystrophy (DMD) is a severe X-linked dystrophinopathy affecting muscle, respiratory, and cardiac function.
- Perioperative management in DMD patients is complex due to high risks of adverse events like rhabdomyolysis and cardiac arrest.
Observation:
- A 22-year-old DMD patient with a left ventricular assist device (LVAD) required surgery for femur and tibia fractures.
- Anesthesia involved total intravenous anesthesia (dexmedetomidine, remimazolam) and multimodal regional anesthesia (fascia iliaca, saphenous, and popliteal nerve blocks).
Findings:
- This anesthetic strategy provided a unique approach for complex orthopedic surgery in a DMD patient with cardiac compromise.
- The combination aimed to mitigate risks associated with general anesthesia in this vulnerable population.
Implications:
- This case demonstrates the potential utility of combined anesthetic techniques in managing high-risk DMD patients.
- Further research into regional anesthesia adjuncts for DMD patients may improve perioperative outcomes and safety.
Abstract:
Duchenne muscular dystrophy (DMD) is an X-linked inherited dystrophinopathy, with an incidence of 1 in 3,600 - 5,000 male live-born infants. The leading cause of death is often cardiomyopathy-related heart failure. Given the progressive nature of the disorder with involvement of skeletal muscle, respiratory and cardiac function, perioperative care remains challenging with an increased incidence of perioperative morbidity and mortality. Perioperative care can be challenging due to life-threatening perioperative adverse events related to associated end-organ effects, as well as sensitivity to various anesthetic agents, rhabdomyolysis, hyperkalemia, hyperthermia, and cardiac arrest. We present a 22-year-old DMD patient with left ventricular assisted device (LVAD), who presented for repair of both left distal femur and tibial diaphysis fractures. Anesthetic care included the unique combination of total intravenous anesthesia with dexmedetomidine and remimazolam combined with regional anesthesia including a supra-inguinal fascia iliaca block, saphenous nerve block, and popliteal nerve block. The basics of dystrophinopathies are presented, perioperative concerns discussed, and previous reports of the use of regional anesthesia as an adjunct to general anesthesia in adult and pediatric patients with DMD are reviewed.
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