Anesthetic Mx of a patient with methylmalonic acidemia
Methylmalonic acidemia (MMA) is a metabolic disorder of organic acids and is characterized by the accumulation of methylmalonic acids.

Published in the JA Clinical Reports, the authors describe anesthetic management of a patient with MMA using a combination of general anesthesia and rectus sheath block.

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.

Read in detail here: https://jaclinicalreports.springeropen.com/articles/10.1186/s40981-018-0209-7
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