Loco-Regional Anesthesia Alone for Emergency Abdominal Surgery in Duchenne Muscular Dystrophy: Not Always an Easy Choice

Rinaldi P, Coletta F, De Simone A, Lauro G, Porcelli ME, Tomasello A and Villani R

Published on: 2024-11-27

Abstract

Duchenne muscular dystrophy (DMD) is a recessively inherited neuromuscular disease that is linked to the X chromosome, a mutation in the gene that codes for the dystrophin, which is essential for proper muscle function. As a result of this mutation, there is multisystem involvement, affecting striated, smooth, and cardiac muscles [1].

Patients with this dystrophy undergoing surgery have an increased anaesthetic risk, both because of the underlying pathophysiological changes in the cardiac and respiratory systems caused by the disease itself, and because certain drugs used in anaesthesia can induce dangerous pathological processes that can seriously endanger the patient, such as acute rhabdomylysis, severe hyperkalemia and malignant hyperthermia [2]. The greatest risks are associated with general anaesthesia: halogenated anaesthetics and the depolarising neuromuscular blocker in particular are capable of inducing these pathological conditions, which have a poor prognosis. For this reason, these patients should be given preference for pure loco-regional anaesthesia whenever possible [2]. In fact, despite the fact that general anaesthesia could be performed safely by avoiding the use of certain trigger drugs, there are still some complications, especially respiratory, associated with the sole event of orotracheal intubation and mechanical ventilation, which could delay weaning from invasive ventilation and increase the mortality rate, particularly because in emergency situations the preoperative anaesthetic assessment cannot be thorough and the respiratory status cannot be optimised [3].