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

Abstract

Abstract Background Methylmalonic acidemia (MMA) is a metabolic disorder of organic acids and is characterized by the accumulation of methylmalonic acids. Case presentation The patient was a 19-year-old female diagnosed with severe MMA at 3 days of age, who was scheduled for renal replacement therapy. Preoperatively, there was no evidence of metabolic acidosis or electrolyte abnormalities. Glucose was administered preoperatively following a 6-h fast. Anesthesia was administered using thiamylal, remifentanil, rocuronium, and sevoflurane. After tracheal intubation, the patient underwent an ultrasound-guided bilateral rectus sheath block with ropivacaine. A drop in blood sugar level was treated with 5% glucose. Extubation was performed after intravenous administration of sugammadex. Conclusions We report the anesthetic management of a patient with MMA using a combination of general anesthesia and rectus sheath block.

Keywords

• Methylmalonic acidemia
• Methylmalonyl coenzyme A mutase
• Metabolic acidosis