Anesthetic Management of Children With Propionic Acidemia Undergoing Esophagogastroduodenoscopy

Abstract

Propionic acidemia is a rare genetic disorder of metabolism that predisposes patients to metabolic acidosis, lethargy, neurologic dysfunction, developmental delays, and cardiomyopathy. Perioperative anesthetic management is guided toward mitigating the effects of preoperative fasting times, maintaining normovolemia, and preventing cardiovascular complications secondary to underlying cardiomyopathy. Commonly used anesthetic agents may have undesirable side effects in these patients. Propofol, the lactate in Lactated Ringer's, and neuromuscular blocking agents that undergo ester hydrolysis are poorly metabolized and can lead to metabolic acidosis. Opioids, such as fentanyl and morphine, should be used judiciously in patients with coexisting developmental delays to avoid oversedation and delayed time to resuming oral intake postanesthesia. In addition, inhaled anesthetics have direct myocardial depressive effects and can compromise cardiac function in the setting of pre-existing cardiomyopathy. The perioperative period represents a critical time in this population and appropriate planning is crucial to prevent perioperative morbidity. We present a case of an eight-year-old child undergoing esophagogastroduodenoscopy under general anesthesia and describe the anesthetic concerns we addressed to provide a safe perioperative course.

Keywords: anesthesia; esophagogastroduodenoscopy (egd); propionic acidemia; propofol.

Figure 1. The enzyme defect in propionic acidemia and its effects.

CoA, coenzyme A; TCA, tricarboxylic acid. Reprinted with permission from the New England Consortium of Metabolic Programs. Propionic Acidemia. Accessed: September 2, 2020: https://www.newenglandconsortium.org/propionic-acidemia.