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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.
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.
Abstract:
Muscle-eye-brain disease is a rare autosomal recessive disorder characterized by congenital muscular dystrophy, ocular abnormalities, and brain malformation. We report an intraoperative hyperkalemic cardiac arrest following the administration of succinylcholine in a child with muscle-eye-brain disease. The disease was diagnosed only after this event. Our experience suggests that preoperative determinations of serum concentrations of lactate and creatine kinase may be useful if clinical signs consistent with myopathy are present.
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