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Anesthetic management of a patient with methylmalonic acidemia: a case report

Yuta Uemura1, Nami Kakuta1, Katsuya Tanaka1

  • 1Department of Anesthesiology, Tokushima University, 3-18-15, Kuramoto, Tokushima, 770-8503, Japan.

JA Clinical Reports
|February 7, 2020
PubMed
Abstract

Insights

Anesthetic management for a patient with methylmalonic acidemia (MMA) involved general anesthesia and a rectus sheath block. This approach ensured safe surgical preparation for renal replacement therapy in this metabolic disorder patient.

Area of Science:

  • Anesthesiology
  • Metabolic Disorders
  • Organic Acidemias

Background:

  • Methylmalonic acidemia (MMA) is a severe organic acid metabolic disorder.
  • Characterized by the accumulation of methylmalonic acids.
  • Requires careful management, especially perioperatively.

Purpose of the Study:

  • To describe the anesthetic management of a patient with severe MMA.
  • To highlight the use of a combined general anesthesia and rectus sheath block technique.
  • To ensure safe surgical preparation for renal replacement therapy.

Main Methods:

  • General anesthesia with thiamylal, remifentanil, rocuronium, and sevoflurane.
  • Ultrasound-guided bilateral rectus sheath block with ropivacaine post-intubation.
  • Intraoperative glucose administration for hypoglycemia and sugammadex for extubation.

Main Results:

  • Successful anesthetic management for renal replacement therapy in a 19-year-old female with severe MMA.
  • No preoperative metabolic acidosis or electrolyte abnormalities observed.
  • Managed intraoperative hypoglycemia with glucose infusion.

Conclusions:

  • Combined general anesthesia and rectus sheath block is a viable anesthetic strategy for MMA patients.
  • This technique facilitated safe surgery for renal replacement therapy.
  • Highlights the importance of tailored anesthetic plans for rare metabolic disorders.
Keywords:
Metabolic acidosisMethylmalonic acidemiaMethylmalonyl coenzyme A mutase

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