Anesthesia Management for Cesarean Delivery in a Woman With Aromatic l-Amino Acid Decarboxylase Deficiency: A Case Report

Abstract

Aromatic l-amino acid decarboxylase deficiency (AADCD) is a rare autosomal recessive disorder of neurotransmitter synthesis with lack of sympathetic autoregulation. Owing to hemodynamic regulatory dysfunction and impairment of sympathetic regulation of heart rate, anesthesia is challenging. We report the successful management of anesthesia in a 26-year-old pregnant woman presenting with a mild phenotype of AADCD. Neuraxial anesthesia was administered, as she had not developed complications previously. Thus, neuraxial anesthesia can also be used safely for a cesarean delivery with appropriate anticipation of potential autonomic disturbances and lack of adrenergic neurotransmission.

Anaesthesia management of a child with aromatic L‐amino acid decarboxylase deficiency

“…Similarly, a letter also published in 2006 outlined the management of a 4‐year‐old child using combined regional and general anaesthesia [4]. A more recent case report from 2020 discussed successful spinal anaesthesia for caesarean delivery in an adult with the disorder [5], demonstrating that patients with this disease can live to adulthood and become pregnant. Further advances in treatment for this disease may increase the likelihood of it being encountered in adult patients presenting for surgery (Figure 1).…”