Pan_2302 100.106

Review articleDuchenne muscular dystrophy: an old anesthesiaproblem revisited JASON H AYES M D F RC P C†, F R A N C I S V E Y C K E M A N S M D*A N D B R U N O B I S S O N N E T T E MD FRCPC††The Hospital for Sick Children, Toronto, ON, Canada and*Service d’Anesthe´siologie, Clinique universitaires St Luc, Brussels, Belgium SummaryPatients with Duchenne and Becker muscular dystrophy suffer from aprogressive deterioration in muscle secondary to a defect in thedystrophin gene. As such, they are susceptible to perioperativerespiratory, cardiac and other complications, such as rhabdomyolysis.
Inhalational anesthetic agents have been implicated as a cause of acuterhabdomyolysis that can resemble malignant hyperthermia (MH).
This article reviews perioperative ‘MH-like’ reactions reported inmuscular dystrophy patients and groups them into three categoriesaccording to clinical presentation. The etiology and underlyingpathophysiological process responsible for these reactions is discussedand recommendations are proposed for the safe anestheticmanagement of these patients.
Keywords: anesthesia; Duchenne muscular dystrophy; pediatrics;rhabdomyolysis surgery without complication, perioperative adverse events are not uncommon (3–8). Acute rhabdo- Duchenne muscular dystrophy (DMD) is the most myolysis is one such event, and is believed to be common childhood muscular dystrophy, with an triggered primarily by the administration of succi- incidence of approximately one in 3500 live male nylcholine, a depolarizing muscle relaxant. Potent births (1). Patients with DMD suffer from progres- inhalational anesthetic agents have also been impli- sive degeneration of skeletal, cardiac and smooth cated as a cause of rhabdomyolysis and other muscle beginning at 3–5 years of age. The progres- perioperative metabolic reactions that resemble sion of muscle weakness is rapid, resulting in a malignant hyperthermia (MH) (9–12). Controversy failure to walk by adolescence and eventual death exists as to whether inhalational agents can be safely from respiratory failure before the end of the third administered to these patients. A hypothetical decade (1). Dilated cardiomyopathy occurs in over 50% of patients by 15 years of age (2). Although A 6 year old male diagnosed with DMD under- many of these patients undergo anesthesia and goes an adenotonsillectomy. Baseline creatinekinase (CK) levels are 900 IUÆl)1 (13) (normal Correspondence to: Dr Jason Hayes, The Hospital for Sick M5G 1X8 (email:
includes nitrous oxide in oxygen, sevoflurane, Journal compilation Ó 2007 Blackwell Publishing Ltd D U C HE N N E M U S C U L A R D Y S T R O P H Y and intravenous fentanyl. Postoperatively, the measured, ranged from 6.9 mmolÆl)1 to greater than patient voids dark, cola-colored urine and com- 12 mmolÆl)1. All patients developed massive rhab- plains of calf and heel pain. The urine is positive domyolysis with myoglobinuria and, when meas- for myoglobin (>60 lgÆl)1), and plasma creatine ured, plasma CK levels were significantly elevated kinase levels are >10 000 IUÆl)1.
(50 000–613 120 IUÆl)1). An elevated body tempera-ture and ⁄ or arterial pCO The underlying cause for these ‘MH-like’ meta- bolic reactions remains unexplained, and for manyyears the incidence of MH was assumed to beincreased in patients with DMD, presumably due to Gradual rise in temperature and heart rate the underlying myopathy. However, evidence Two articles described a total of nine patients with against this association is now available, and alter- DMD who developed unexplained hyperthermia native pathophysiological mechanisms have been (maximum 38.2°C) and tachycardia during or after anesthesia with halothane (7,23). In seven patients This article reviews perioperative metabolic reac- (ages unknown) the onset occurred within a few tions that resembled MH in DMD patients reported hours of anesthesia and resolved spontaneously (23).
over the past 40 years. Only patients administered The other two patients (ages 6 and 8 years) devel- inhalational anesthetic agents, not succinylcholine, oped intraoperative hyperthermia and tachycardia were considered. These reactions are grouped into that resolved once halothane was discontinued (7).
three categories according to clinical presentation, The arterial blood gases were normal in one patient and the etiology and underlying pathophysiological and were not measured in the others. There was no process responsible for these reactions are discussed.
mention of rhabdomyolysis in any of the patients, Lastly, based on the data available, recommenda- although plasma CK and K+ levels were not tions are proposed for the safe anesthetic manage- Acute onset of hyperkalemic cardiac arrest Significant muscle cell breakdown has been reportedin six patients following exposure to halothane, A review of the literature found a total of 13 patients sevoflurane, and enflurane (24–29). Interestingly, who had a sudden hyperkalemic cardiac arrest with although an inhalational agent was not used during no obvious preceding signs of hypermetabolism the maintenance of anesthesia in one of the six (9,10,14–22). Ages ranged from 2 to 18 years; how- patients, it was observed that the anesthesia machine ever, 12 were 8 years of age or younger. Eight had not been properly flushed prior to the admin- patients had, or were suspected of having, DMD.
istration of anesthesia (29). Five of the six patients, The remainder were diagnosed with Becker muscu- aged 15 months to 11 years, had a diagnosis of DMD lar dystrophy (BMD), a similar pathology to DMD, (24–27,29), and one patient, aged 22 years, had BMD in which dystrophin is present but dysfunctional.
(28). Rhabdomyolysis resulted in plasma CK levels For seven of the DMD and one of the BMD patients, that were elevated (12 900–105 000 IUÆl)1) from hyperkalemic cardiac arrest was the initial ‘presen- baseline in five patients (24–28). The patient who tation’ of the underlying myopathy. Halothane was received a ‘trigger-free’ anesthetic had myoglobine- administered to over half of the patients (7 ⁄ 13) in mia but did not have an elevated CK level compared this group. The others received isoflurane (two with baseline (29). One patient had an inappropriate patients), sevoflurane (one patient), or a combination sinus tachycardia and perioperative elevation in of the two (three patients). The timing of the cardiac body temperature (40.3°C) despite the use of arrest was unpredictable as it varied from 10 min dantrolene (29); otherwise there were no other signs after induction to 20 min following arrival in the recovery room. Plasma potassium (K+) levels, when Ó 2007 The AuthorsJournal compilation Ó 2007 Blackwell Publishing Ltd, Pediatric Anesthesia, 18, 100–106 States (MHAUS) in an Anesthesia Patient SafetyFoundation (APSF) newsletter (22): Cardiac arrest related to MH is usually preceded A longstanding view is that MH is the underlying by rapidly rising endtidal carbon dioxide, muscle mechanism responsible for these reactions (6,12).
rigidity, acidosis and hyperthermia, and most This is not surprising for two reasons: first, the often occurs during anesthetic administration combination of clinical signs such as tachycardia, rather than in the postoperative period. In such elevated body temperature, raised arterial pCO2 cases, the cause…is significant metabolic and ⁄ or levels, and rhabdomyolysis are suggestive of MH; respiratory acidosis rather than hyperkalemia.
second, many DMD and BMD patients have testedpositive for MH using skeletal muscle in vitro Patients in the second group would be assigned contracture tests. However, neither of these argu- an MH score of ‘somewhat less than likely’ because ments is convincing. With respect to the clinical of an ‘inappropriately rapid increase in body tem- signs of MH, the reactions described above are perature’ (15 points) and unexplained tachycardia (3 atypical. The Malignant Hyperthermia Clinical Gra- points). This is not surprising for two reasons: first, ding Scale is a method for estimating the qualitative hyperthermia is considered a late sign of MH, and likelihood of an MH reaction in a given patient using would be unlikely to precede or occur in the absence a standardized point system based on diagnostic of other signs of hypermetabolism; second, tachy- criteria grouped into six ‘processes’: muscle rigidity, cardia is a nonspecific sign of hypermetabolism. Of muscle breakdown, respiratory acidosis, tempera- note, arterial blood gases were normal when meas- ture increase, cardiac involvement and a family ured. This contradicts the diagnosis of MH as history of MH (30). The diagnostic criteria within increased CO2 production and metabolic acidosis, each process are assigned points based on their due to accelerated aerobic or anaerobic metabolism, relative severity. For example, within the muscle breakdown category, ‘elevated CK >10 000 IUÆl)1 For patients in the third group, the MH rank after anesthetic without succinylcholine’ is worth 15 would also be ‘somewhat less than likely’: five of the points, whereas ‘myoglobin in serum >170 lgÆl)1, is six developed isolated rhabdomyolysis (15 points), worth three points. The points for each criteria are and one had an inappropriate tachycardia (3 points), then added together to produce a raw score that is elevated temperature (10 points), and myoglobine- converted to an MH rank (1–6) and ‘description of mia (5 points). Again, the absence of any other likelihood’ of MH, from ‘almost never’ (rank 1) to clinical and metabolic signs of hypermetabolism is ‘almost certain’ (rank 6). If multiple criteria repre- sent a single process, only the indicator with the With respect to positive in vitro contracture tests in DMD patients, there are two problems: first, those Using this scale, patients in the first group would related to in vitro contracture tests in general, and achieve an ‘MH likelihood’ of ‘somewhat less than second, difficulties with in vitro contracture tests in likely’ (10–19 points) or ‘somewhat greater than patients with myopathies. The current ‘gold stand- likely’ (20–34 points): all had excessively elevated ard’ for in vitro contracture tests is the measurement CK levels (15 points); six had elevated arterial pCO2 of the force of contraction of viable, nonskinned levels (15 points); two patients had an ‘inappropri- muscle strips exposed to increasing levels of haloth- ately increased body temperature >38.8°C’ (10 ane or caffeine (caffeine–halothane contracture test).
points). It is important to note that elevated arterial Two caffeine–halothane contracture test protocols, a pCO2 levels always occurred in the context of a low North American and a European, exist (32). The cardiac output state and no patients had evidence of European protocol uses more increments in the excessive CO2 production or hypermetabolism prior caffeine and halothane concentrations than the to the event. Additionally, the acuity of the cardiac North American protocol, resulting in lower diag- arrest with no preceding signs of hypermetabolism nostic thresholds (32,33). Sensitivity thresholds are is very unusual. This point was emphasized by the intentionally kept high at the sacrifice of specificity Malignant Hyperthermia Association of the United to avoid false-negative results (32). The sensitivity is Journal compilation Ó 2007 Blackwell Publishing Ltd, Pediatric Anesthesia, 18, 100–106 D U C HE N N E M U S C U L A R D Y S T R O P H Y 97–99% for both protocols, whereas the specificity is The concept of AIR has been discussed in the 80–85% for the North American protocol and 90% literature since 1985, recognizing that rhabdomyoly- for the European (32). Therefore, a positive caffeine– sis not associated with MH can occur in DMD halothane contracture test is not a guarantee that a patients after exposure to potent inhalational agents With respect to muscular dystrophy patients, (10,20,27,36,39,45,46). The supporting evidence for some have been deemed MH susceptible using AIR is the same as that against MH: the reactions are invalidated in vitro contracture tests methods, such atypical for MH despite some similar characteristics, as Ca2+ uptake and ATPase activity (9,29), and and most caffeine–halothane contracture test results skinned muscle fibers (the sarcolemma is removed are negative, and probably unreliable, in muscular chemically or mechanically before exposure to the dystrophy patients. There is also indirect evidence agent) (11,34). The results of caffeine–halothane that supports the notion that the lack of dystrophin contracture tests in muscular dystrophy patients is the root cause of rhabdomyolysis after exposure to are conflicting. A few case reports have described inhalational anesthetic agents. Patients with muscle patients as MH susceptible on the basis of positive disorders, such as myotonic dystrophy, have normal contracture tests with halothane alone (12,35,36).
dystrophin and thus stable sarcolemma. Despite the This would be considered an MH-equivocal result presence of massive contractures following the by others (37). Abnormal contractures to both administration of succinylcholine, there is no signi- halothane and caffeine have been documented in ficant rhabdomyolysis observed in these patients.
both BMD (10,37) and DMD patients (38), although Why a small minority of muscular dystrophy the latter reference did not use an established patients suffer AIR after exposure to inhalational caffeine–halothane contracture test protocol. Numer- anesthetic agents remains unknown. Susceptibility ous reports have demonstrated negative contracture may be, in part, related to the relative amount of tests in both DMD and BMD patients (37,39–41) muscle ‘at risk’. In DMD patients under 8 years of using both the North American (39) and European age, muscle fibers are attempting to regenerate, and protocols (40). Moreover, contracture tests of dys- are more prone to rhabdomyolysis (47–49). As the trophic muscle may be unreliable for two reasons: patient ages, greater proportions of muscle fibers first, the underlying defect of raised intracellular stop regenerating and become fibrotic (47,49,50).
Ca2+ levels may produce abnormal contractures This observation may explain why the majority of (32,39) and thus a greater incidence of false-positive AIR reactions occur in preadolescent patients, or results; second, the muscle specimens are often of older patients with BMD, which progresses more poor quality because of progressive fibrosis (42).
A dystrophin-deficient mouse model (mdx) has been used to provide additional insight into this issue (43). The mdx mouse myocytes, like human DMDmyocytes, lack dystrophin and have abnormal intra- Duchenne muscular dystrophy is an X-linked reces- cellular Ca2+ homeostasis. However, the cells are sive disease characterized by a lack of dystrophin much less dystrophic and the mice exhibit only a mild because of an abnormal dystrophin gene located on myopathy. MH testing of mdx muscle using the the short arm of the X chromosome (Xp21 position) European protocol produced normal responses, sug- (45). The majority (65%) of mutations of the dystro- gesting that dystrophin deficiency and abnormal phin gene are large-scale deletions and approxi- Ca2+ homeostasis per se, do not predispose to MH (43).
mately 5% are because of duplications (51).
Dystrophin, a large intracellular protein, and dys- trophin-related glycoproteins form a complex thatconnects the subsarcolemmal cytoskeleton to the The lack of evidence to support an association extracellular matrix. The absence of dystrophin prevents either the assembly or integration of the proposal of an alternative mechanism termed ‘an- components of the glycoprotein complex into the esthesia-induced rhabdomyolysis’ (AIR) (27,44,45).
muscle cell membrane (sarcolemma) or accelerates Ó 2007 The AuthorsJournal compilation Ó 2007 Blackwell Publishing Ltd, Pediatric Anesthesia, 18, 100–106 degradation (51). The absence of the dystrophin– should be carefully monitored for signs of rhabdo- glycoprotein complex results in instability and myolysis (serum K+ level) because, even if the risk is increased permeability of the sarcolemma and low, its occurrence is unpredictable.
increased intracellular calcium levels (45). Addition- In the event that AIR is suspected, the inhalational ally, chronically elevated intracellular Ca2+ levels anesthetic agent should be discontinued immedi- may result in the activation of enzymes that pro- ately. Serial serum potassium levels should be teolyze the cytoskeletal components and further measured and immediately treated if greater than degrade the structural stability of the sarcolemma 5.5 mmolÆl)1. To shift potassium back into the muscle (52). Exposure of the sarcolemma to a potent cells, intravenous sodium bicarbonate and insulin inhalational agent (or succinylcholine) stresses the with 10% dextrose should be administered and the muscle cell membrane and further increases the patient hyperventilated to produce a respiratory instability and permeability. Consequently, intracel- alkalosis. Serial plasma CK, plasma myoglobin and lular Ca2+ levels increase further and cell contents, urine myoglobin levels should be measured to detect such as K+ and CK, leak out. A compensatory rhabdomyolysis. If present, the patient should be hypermetabolic response occurs in an attempt to treated with intravenous hydration and mannitol to reestablish membrane stability and prevent Ca2+ maintain the urine output greater than 1 mlÆkg)1Æh)1 fluxes (39). This proposed mechanism may explain and minimize the risk of renal impairment.
the hyperkalemia, hyperthermia, tachycardia and Perioperative hyperkalemic cardiac arrest in an rhabdomyolysis observed in these patients.
asymptomatic young male patient may be the initialpresentation of occult muscular dystrophy. In suchan event, the American Heart Association (AHA) Guidelines recommend the immediate administra- tion of intravenous calcium chloride to antagonize Although only a small proportion of DMD patients the myocardial effects of hyperkalemia and help develop AIR after exposure to inhalational anes- restore a spontaneous cardiac rhythm (55). The thetic agents, the question is: should we continue to protective effect of Ca2+ on myocardial cells is most use inhalational anesthetic agents when total intra- likely related to the influx of Ca2+ into the cell, which venous anesthesia (TIVA) is a safe and readily raises intracellular Ca2+ levels and transiently available alternative? The opinion in the literature decreases the resting potential of the cell membrane has shifted over the last decade from ‘yes’ (1,53) to (56,57). This reduces the potassium-related hyperex- ‘no’ (49,54). Not only do we agree with this, we also citability of the myocardium and maintains a spon- suggest that a ‘trigger-free’ anesthetic and ‘clean’ taneous cardiac rhythm. Intracellular Ca2+ levels are anesthesia machine be used, similar to that for MH- elevated in skeletal muscle cells of DMD patients susceptible patients. This recommendation is based and in the myocardium of older, but not necessarily on the fact that the minimum triggering concentra- younger, mdx mice (52,58,59). Therefore, the admin- tion of inhalational agent remains unknown. Many istration of calcium chloride may not depress the of the most severe AIR reactions occur in the myocardial membrane resting potential to the same recovery room when drug concentrations are low degree, and thus may not be as effective for the (49). For instance, rhabdomyolysis has been reported treatment of hyperkalemic cardiac arrest. Nonethe- in a 3-year-old patient who received a trigger-free less, the AHA Guidelines regarding the administra- anesthetic but with an anesthesia machine that had tion of calcium chloride for hyperkalemic cardiac not been flushed prior to this procedure (29).
arrest should be adhered to. However, initial treat- In certain clinical situations, such as a DMD patient ment should also focus on measures to shift potas- with the potential for difficult airway management, sium back into muscle cells, as sinus rhythm cannot and where an intravenous technique is not believed to be reestablished until the serum potassium levels are be an option, a short exposure to an inhalational agent until the airway has been secured can be supported.
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