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].
Keywords
Loco-regional anaesthesia; Duchenne muscular dystrophy; Neuroaxial anaesthesia; pre-operative assessmentLetter To The Editor
We present a case in which a patient suffering from DMD with sigmoid volvulus successfully underwent exploratory laparotomy with colic resection using only loco-regional anaesthesia. The patient provided written informed consent.
A 53-year-old woman presented to our hospital complaining of severe abdominal pain (with a Numerical Rating Score of 8), nausea and abdominal distension. The CT scan showed the presence of a sigmoid colon volvulus with severe vascular compromise, which could have led to the possibility of colic resection.
Given the urgency of the situation and the need for surgery, a brief and rapid anamnesis was performed, which revealed that the patient suffered from DMD with associated dystrophinopathy-related cardiomyopathy with preserved ejection fraction and without valvular dysfunction. In view of the patient's history and the laboratory tests, which showed that her coagulation levels were within normal limits, we decided to perform a combined spinal epidural (CSE) anaesthesia [4]. Although this technique of loco-regional anaesthesia is well established in some surgical fields (especially orthopaedic and obstetric surgery), its use in abdominal surgery, especially in emergencies, as a single technique and not in combination with general anaesthesia is less common as an anaesthetic choice.
The reason for this lies in the fact that a pure loco-regional anaesthesia may not guarantee complete analgesia of the surgical region concerned and this would lead to a difficult intraoperative anaesthesiological management, especially in the patient with a full stomach. Furthermore, the occurrence of other complications, both surgical and anaesthesiological (related to the anaesthesia itself, especially emodynamic complications), would require additional management during surgery, which could be even more difficult [5, 6].
For all these reasons, the most widely used anaesthetic choice in emergency abdominal surgery today is undoubtedly general or blended anaesthesia [7].
In the operating room, multiparametric monitoring of vital parameters (SpO2%, NIBP, ECG) was performed and the patient was premedicated with Paracetamol 1 g and Dexamethasone 4 mg.
We then inserted a nasogastric tube, which helped to reduce the gastric tension caused by the volvulus, which immediately benefited the patient with the disappearance of nausea. CSE anaesthesia was performed using separate needles in two different interspinous spaces: spinal anaesthesia was performed in the T12-L1 interspinous space using 2 ml of 0.5% hyperbaric bupivacaine and epidural anaesthesia was performed in the T11-T12 interspinous space, leaving the epidural catheter in place, which was used both to supplement and prolong the spinal anaesthesia with intraoperative boluses of 10 ml of a mixture of 0.5% ropivacaine and sufentanil 10 mcg and for postoperative pain relief with boluses of 10 ml of a mixture of 0.2% ropivacaine. The analgesic coverage was satisfactory, allowing the operation to be completed with colic resection of the volvulus (Figure 1) and vital parameters to remain stable without any cardio-respiratory complications and with complete absence of postoperative pain (NRS 0).
Figure 1: Resected volvulus.
Loco-regional anaesthesia has also been shown to be safe and effective in this case of urgent abdominal surgery, as well as in other elective surgical scenarios [8], avoiding general anaesthesia and the cardio-respiratory complications, rhabdomyolysis and malignant hyperthermia that DMD can cause.
However, safely performed general anaesthesia remains the most reliable technique when loco-regional anaesthesia is not possible, either because of the location of the surgery or because the airway is not safe. In addition, not all anaesthetists are equally skilled in loco-regional anaesthetic techniques: the best anaesthetic plan also depends on the skills of the anaesthetist. Finally, also in view of the limitations and complications described for neuraxial anaesthesia, it is worth a try, if possible, if the risks associated with general anaesthesia are high, as in this case.
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