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Abstract |
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Implications: Children with Duchenne’s muscular dystrophy should not be exposed to succinylcholine because of the risk of hyperkalemic cardiac arrest and rhabdomyolysis. This report describes the response to rapacuronium bromide in two patients with Duchenne’s muscular dystrophy. Both patients had a recovery index 2 times longer than that reported in children with normal neuromuscular function.
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Introduction |
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TopAbstractIntroductionCase ReportsDiscussionReferences |
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Duchenne’s muscular dystrophy (DMD) is the most common myopathy occurring in pediatric patients. Children who have this disease often require anesthetic care during muscle biopsy or correction of progressive orthopedic deformities. One of the major issues related to the anesthetic conduct of such patients is the choice of neuromuscular blocking agent (NMBA). Succinylcholine is contraindicated because of the risks of rhabdomyolysis, hyperkalemia, and cardiac arrest (1–3). Rapacuronium is a recently approved NMBA that has the properties of rapid onset and recovery, which may be desirable in patients with DMD. We report our experience with the administration of rapacuronium to two children with DMD.
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Case Reports |
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TopAbstractIntroductionCase ReportsDiscussionReferences |
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A 10-yr-old, 54-kg boy presented for heel cord lengthening. The diagnosis of DMD was made when the boy was 7 yr old, after a muscle biopsy. The patient’s only medication was prednisone (5 mg by mouth, every other day). Physical examination showed a normally developed boy with bilateral lower extremity hypertrophy and contractures at the Achilles tendon.
The second patient was a 20-kg, 9-yr-old boy presenting for the same procedure. His medical history was significant for a muscle biopsy 2 yr earlier that was diagnostic for DMD. He had been a wheelchair user for the past 4 mo.
Neither patient had other associated conditions, such as scoliosis, cardiomyopathy, or recurrent pneumonias. A nontriggering anesthetic was conducted in both cases. Midazolam (1.5–2 mg) was given after IV cannulation. Standard monitors were placed, the patients’ lungs were preoxygenated, and general anesthesia was induced with the following drugs: sodium thiopental (1–2 mg/kg), lidocaine hydrochloride (1.5–2.0 mg/kg), fentanyl (2–5 µg/kg), and increments of propofol (2–4 mg/kg). Once adequate mask ventilation was established, rapacuronium (2.0 mg/kg) was administered. Rapacuronium was chosen with the supposition that it would provide a rapid onset with the potential for a brief duration even in patients with underlying muscle pathology. Approximately 1 min after the administration of rapacuronium, each patient’s trachea was intubated and the endotracheal tube secured. Mechanical ventilation with 30% oxygen and 70% nitrous oxide was begun, maintaining an end-tidal carbon dioxide at 35–40 mm Hg. General anesthesia was maintained with propofol and remifentanil infusions at rates of 45–150 µg · kg-1 · min-1 and 0.12–0.2 µg · kg-1 · min-1, respectively. Volatile anesthetics were not used. Temperature was maintained at 36°–37°C. The surgical procedures lasted 2.5–3 h. The intraoperative and postoperative courses of these two patients were uneventful.
Neuromuscular transmission was monitored with an EMG Datex Relaxograph® (NMT-100, Helsinki, Finland) delivering stimuli to the left ulnar nerve and measuring evoked potentials of the abductor digiti minimi muscle. The EMG was placed on the side opposite the IV infusion and blood pressure cuff. After an initial baseline recording was obtained, train-of-four stimulation was conducted every 20 s (2 Hz, 100-µs pulse width). Monitoring was continued until complete clinical recovery (a plateau phase on the T1 and train-of-four) was achieved. Repeat dosing of rapacuronium was not required. The clinical data are summarized in Table 1.
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Discussion |
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TopAbstractIntroductionCase ReportsDiscussionReferences |
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Rapacuronium is an NMBA with a clinical onset time of approximately one minute. Clinical duration (defined as the time to return of 25% of T1) is 13.8 ± 7.2 minutes after the administration of 2 mg/kg in the pediatric population (4). However, the initial studies with rapacuronium in pediatric patients have included only patients with normal neuromuscular function. We present the first information concerning recovery characteristics in two patients with DMD. After the administration of 2 mg/kg of rapacuronium to our two patients with DMD, we noted a clinical duration (return of T1 to 25% baseline) that was slightly longer than the range of normal published values, a recovery index (T1 25%–75%) that was prolonged twofold and a time to 70% T4/T1 ratio that was approximately twice as long as that noted in the normal population.
Despite its prevalence in the general population, there is limited information concerning the use of NMBAs in children with DMD. In two children with DMD, sensitivity to vecuronium was unchanged whereas recovery time was 3–6 times longer when compared with patients with normal neuromuscular function (5). In contrast, a matched control study indicated that patients with DMD were more sensitive to vecuronium (with prolonged recovery times) (6).
The use of atracurium has been reported in DMD patients, but data are lacking as to whether there is an appreciable effect on recovery time (7). Mivacurium, a short-acting NMDA, was thought to offer advantages by having a significantly shorter recovery time. Two studies have indicated that DMD patients receiving mivacurium have an increased sensitivity to the drug and a prolonged recovery index (2–3 times normal) (8,9). Both mivacurium and atracurium have the disadvantages of histamine release and slow onset.
We chose not to use reversal drugs in our two patients. Although early reversal of rapacuronium bromide has been studied as a means of achieving recovery times similar to that of succinylcholine (10,11), altered responses to reversal of neuromuscular blockade have been reported in adult patients with neuromuscular disorders other than DMD (12). Although the pharmacological antagonism of acetylcholine breakdown has been thought to precipitate rhabdomyolysis in patients with DMD (5), reversal of nondepolarizing neuromuscular blockade in patients with DMD has been reported without adverse sequelae (6,9).
We have demonstrated that neuromuscular recovery from rapacuronium in two patients with DMD is prolonged. Depending on the duration of the anticipated procedure, this effect may or may not be clinically significant. In comparison to other available nondepolarizing NMBAs, rapacuronium has a clinically useful recovery time in patients with DMD. Further studies are necessary to assess our preliminary observations.
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References |
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TopAbstractIntroductionCase ReportsDiscussionReferences |
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- Larach MG, Rosenberg H, Gronert GA, Allen GC. Hyperkalemic cardiac arrest during anesthesia in infants and children with occult myopathies. Clin Pediatr 1997;36:9–16.
- Larsen UT, Juhl B, Hein-Sorensen O, de Fine O. Complications during anaesthesia in patients with Duchenne’s muscular dystrophy (a retrospective study). Can J Anaesth 1989;36:418–22.[Web of Science][Medline]
- Sullivan M, Thompson WK, Hill GD. Succinylcholine-induced cardiac arrest in children with undiagnosed myopathy. Can J Anaesth 1994;41:497–501.[Web of Science][Medline]
- Brandom BW, Margolis JO, Bikhazi GB, et al. Neuromuscular effects of rapacuronium in pediatric patients during nitrous oxide-halothane anesthesia: comparison with mivacurium. Can J Anaesth 2000;47:143–9.[Web of Science][Medline]
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Buzello W, Huttarsch H. Muscle relaxation in patients with Duchenne’s muscular dystrophy: use of vecuronium in two patients. Br J Anaesth 1988;60:228–31.
[Abstract/Free Full Text] - Ririe DG, Shapiro F, Sethna NF. The response of patients with Duchenne’s muscular dystrophy to neuromuscular blockade with vecuronium. Anesthesiology 1998;88:351–4.[Web of Science][Medline]
- Rosewarne FA. Anaesthesia, atracurium and Duchenne muscular dystrophy. Can Anaesth Soc J 1986;33:250–1.[Medline]
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Uslu M, Mellinghoff H, Diefenbach C. Mivacurium for muscle relaxation in a child with Duchenne’s muscular dystrophy. Anesth Analg 1999;89:340–1.
[Free Full Text] - Tobias JD, Atwood R. Mivacurium in children with Duchenne muscular dystrophy. Paediatr Anaesth 1994;4:57–60.
- Purdy R, Bevan DR, Donati F, Lichtor JL. Early reversal of rapacuronium with neostigmine. Anesthesiology 1999;91:51–7.[Web of Science][Medline]
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Wierda JM, van den Broek L, Proost JH, et al. Time course of action and endotracheal intubating conditions of Org 9487, a new short-acting steroidal muscle relaxant; a comparison with succinylcholine. Anesth Analg 1993;77:579–84.
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Buzello W, Krieg N, Schlickewei A. Hazards of neostigmine in patients with neuromuscular disorders: report of two cases. Br J Anaesth 1982;54:529–34.
[Abstract/Free Full Text]
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  J. Schmidt, T. Muenster, S. Wick, J. Forst, and H. J. Schmitt Onset and duration of mivacurium-induced neuromuscular block in patients with Duchenne muscular dystrophy Br. J. Anaesth., December 1, 2005; 95(6): 769 - 772. [Abstract] [Full Text] [PDF]
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