Anesth Pain Med.  2023 Jul;18(3):270-274. 10.17085/apm.23034.

Perioperative considerations of pyruvate dehydrogenase complex deficiency: a case report of two consecutive anesthesia

• Lim WY
• Lee HJ
• Park EJ
• Jeon S
• Do W
• Kim HJ
• Lee D
• Hong JM

• KMID: 2547035
• DOI: http://doi.org/10.17085/apm.23034

Abstract

Background
Pyruvate dehydrogenase complex (PDHC) deficiency is a rare mitochondrial disorder caused by a genetic mutation affecting the activity of the PDHC enzyme, which plays a major role in the tricarboxylic cycle. Few cases of surgery or anesthesia have been reported. Moreover, there is no recommended anesthetic method. Case: A 24-month-old child with a PDHC deficiency presented to the emergency room with respiratory failure, mental decline, systemic cyanosis, and lactic acidosis. During hospitalization period, the patient presented with pneumothorax, pneumoperitoneum, and multiple air pockets in the heart. Two surgeries were performed under general anesthesia using an inhalational anesthetic agent. The patient was discharged with home ventilation.
Conclusions
Anesthesiologists should be wary of multiple factors when administering anesthesia to patients with PDHC deficiency, including airway abnormalities, acid-base imbalance, intraoperative fluid management, selection of appropriate anesthetics, and monitoring of lactic acid levels.

Keyword

Acid-base imbalance; Anesthesia, inhalation; Lactic acidosis; Mitochondrial disease; Pyruvate dehydrogenase complex deficiency disease

Figure

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  Fig. 1. Computed tomography image of the patient. Pneumothorax, pneumomediastinum, and multiple air pockets in the heart are detected.

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