Case Report of Cardiac Arrest After Succinylcholine in a Child With Muscle-Eye-Brain Disease

Thomas Hackmann1, David L Skidmore, Brian MacManus

  • 1From the *Department of Paediatric Anaesthesia, IWK Health Centre, Halifax, Nova Scotia, Canada; †Department of Anesthesia, Pain Management and Perioperative Medicine, and ‡Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada.

A & a Case Reports
|June 13, 2017
PubMed

Insights

Muscle-eye-brain disease, a rare genetic disorder, can cause severe reactions like hyperkalemic cardiac arrest after succinylcholine administration. Preoperative lactate and creatine kinase tests may help identify at-risk patients with myopathy signs.

Area of Science:

  • Neurology
  • Genetics
  • Anesthesiology

Background:

  • Muscle-eye-brain disease is a rare autosomal recessive disorder.
  • It is characterized by congenital muscular dystrophy, ocular abnormalities, and brain malformation.

Purpose of the Study:

  • To report a case of intraoperative hyperkalemic cardiac arrest in a child with undiagnosed Muscle-eye-brain disease.
  • To suggest preoperative diagnostic markers for patients with potential myopathy.

Main Methods:

  • Case report of a patient with Muscle-eye-brain disease.
  • Review of intraoperative events and diagnostic procedures.

Main Results:

  • A child with undiagnosed Muscle-eye-brain disease experienced hyperkalemic cardiac arrest after succinylcholine administration.
  • The diagnosis of Muscle-eye-brain disease was confirmed post-event.

Conclusions:

  • Succinylcholine administration can trigger life-threatening hyperkalemic cardiac arrest in patients with Muscle-eye-brain disease.
  • Preoperative serum lactate and creatine kinase levels may serve as useful indicators for myopathy in at-risk individuals.

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