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- Chiara Viviani, Elena Buelli, Giulia Fierro, Sandra Stiffan, and Ferdinando Luca Lorini.
- From the Department of Anesthesia and Intensive Care II, Papa Giovanni Paolo XXIII Hospital, Bergamo, Italy.
- A A Pract. 2020 Aug 1; 14 (10): e01275.
AbstractAromatic 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.
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