Abstract

BackgroundWe aim to describe the evaluation and management of a patient with the uncommon combination of both mitochondrial myopathy and possible malignant hyperthermia susceptibility as an important source of information and as a valuable example of the role of regional anesthesia for patients with these diagnoses.Case presentationA 24 year old woman with a history of possible mitochondrial myopathy and possible malignant hyperthermia susceptibility presented for gynecologic surgery. Surgery was well tolerated with combined spinal epidural anesthesia as well as sedation with midazolam, ketamine, and fentanyl.ConclusionsAnesthetic management of patients with mitochondrial myopathy is challenging, made even more so with concurrent malignant hyperthermia susceptibility. This case adds an example to the literature of employing regional anesthesia as a safe approach to this complex care.

Highlights

  • We aim to describe the evaluation and management of a patient with the uncommon combination of both mitochondrial myopathy and possible malignant hyperthermia susceptibility as an important source of information and as a valuable example of the role of regional anesthesia for patients with these diagnoses

  • We present a case of a patient with both mitochondrial myopathy (MM) and malignant hyperthermia (MH) susceptibility managed with regional anesthesia and sedation for a gynecologic surgery

  • Discussion & Conclusions When a patient presents for surgery with a history suggestive of MM, the diagnostic features of this spectrum of disorders make anesthetic evaluation, planning, and decisions difficult

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Summary

Introduction

We aim to describe the evaluation and management of a patient with the uncommon combination of both mitochondrial myopathy and possible malignant hyperthermia susceptibility as an important source of information and as a valuable example of the role of regional anesthesia for patients with these diagnoses. Conclusions: Anesthetic management of patients with mitochondrial myopathy is challenging, made even more so with concurrent malignant hyperthermia susceptibility. This case adds an example to the literature of employing regional anesthesia as a safe approach to this complex care. Care must be taken to avoid tipping the balance of energy supply and demand, including checking baseline pH and lactate levels followed by perioperative monitoring [1, 3].

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