Dexmedetomidine and fentanyl combination for procedural sedation in a case of Duchenne muscular dystrophy (original) (raw)
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Anesthesia and Duchenne or Becker muscular dystrophy: review of 117 anesthetic exposures
Pediatric Anesthesia, 2013
Background: Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are associated with life-threatening perioperative complications, including rhabdomyolysis, hyperkalemia, and hyperthermia. Current recommendations contraindicate use of succinylcholine and volatile anesthetics; however, the latter recommendation remains controversial. Objective: To review the perioperative outcomes of patients with DMD and BMD. Methods: We reviewed records of patients with DMD or BMD who underwent anesthetic management at our institution from January 1990 through December 2011. Results: We identified 47 patients (DMD, 37; BMD, 10) who underwent 117 anesthetic exposures (DMD, 101; BMD, 16). Volatile anesthetic agents were used 66 times (DMD, 59; BMD, 7). One patient with undiagnosed BMD received succinylcholine and developed acute rhabdomyolysis and hyperkalemic cardiac arrest. All other major complications were attributed to the procedure (i.e., large bleeding), to preexisting comorbidities (i.e., respiratory failure, cardiac disease), or to both. Conclusions: Use of succinylcholine in children with dystrophinopathy is contraindicated. These patients have significant comorbidities and are frequently undergoing extensive operations; complications related to these factors can develop, as evidenced by our series. These complications may occur with use of volatile and nonvolatile anesthetics. However, because most of our patients were older than 8 years at the time of surgery, our observation cannot be generalized to younger dystrophin-deficient children.
Duchenne muscular dystrophy: an old anesthesia problem revisited
Pediatric Anesthesia, 2007
Patients with Duchenne and Becker muscular dystrophy suffer from a progressive deterioration in muscle secondary to a defect in the dystrophin gene. As such, they are susceptible to perioperative respiratory, cardiac and other complications, such as rhabdomyolysis. Inhalational anesthetic agents have been implicated as a cause of acute rhabdomyolysis that can resemble malignant hyperthermia (MH). This article reviews perioperative 'MH-like' reactions reported in muscular dystrophy patients and groups them into three categories according to clinical presentation. The etiology and underlying pathophysiological process responsible for these reactions is discussed and recommendations are proposed for the safe anesthetic management of these patients.
Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy
Brazilian Journal of Anesthesiology (English Edition)
Background and objectives: Duchenne/Becker muscular dystrophy affects skeletal muscles and leads to progressive muscle weakness and risk of atypical anesthetic reactions following exposure to succinylcholine or halogenated agents. The aim of this report is to describe the investigation and diagnosis of a patient with Becker muscular dystrophy and review the care required in anesthesia. Case report: Male patient, 14 years old, referred for hyperCKemia (chronic increase of serum creatine kinase levels-CK), with CK values of 7,779-29,040 IU.L −1 (normal 174 IU.L −1). He presented with a discrete delay in motor milestones acquisition (sitting at 9 months, walking at 18 months). He had a history of liver transplantation. In the neurological examination, the patient showed difficulty in walking on one's heels, myopathic sign (hands supported on the thighs to stand), high arched palate, calf hypertrophy, winged scapulae, global muscle hypotonia and arreflexia. Spirometry showed mild restrictive respiratory insufficiency (forced vital capacity: 77% of predicted). The in vitro muscle contracture test in response to halothane and caffeine was normal. Muscular dystrophy analysis by Western blot showed reduced dystrophin (20% of normal) for both antibodies (C and N-terminal), allowing the diagnosis of Becker muscular dystrophy.
Hospital Pediatrics, 2016
Dexmedetomidine (DEX) is widely used in pediatric procedural sedation (PPS) by a variety of pediatric subspecialists. The objective of our study was to describe the overall rates of adverse events and serious adverse events (SAEs) when DEX is used by various pediatric subspecialists. METHODS: Patients from the Pediatric Sedation Research Consortium (PSRC) database were retrospectively reviewed and children that received DEX as their primary sedation agent for elective PPS were identified. Demographic and clinical data, provider subspecialty, and sedation-related complications were abstracted. SAEs were defined as death, cardiac arrest, upper airway obstruction, laryngospasm, emergent airway intervention, unplanned hospital admission/increased level of care, aspiration, or emergency anesthesia consult. Event rates and 95% confidence intervals (CIs) were calculated. RESULTS: During the study period, 13 072 children were sedated using DEX, accounting for 5.3% of all sedation cases entered into the PSRC. Of the sedated patients, 73% were American Society of Anesthesiologists Physical Status class 1 or 2. The pediatric providers responsible for patients sedated with DEX were anesthesiologists (35%), intensivists (34%), emergency medicine physicians (12.7%), hospitalists (1.1%), and others (17%). The overall AE rate was 466/13 072 (3.6%, 95% CI 3.3% to 3.9%). The overall SAE rate was 45/13 072 (0.34%, 95% CI 0.19% to 0.037%). Airway obstruction was the most common SAE: 35/13 072 (0.27%, 95% CI 0.19% to 0.37%). Sedations were successful in 99.7% of cases. CONCLUSIONS: We report the largest series of PPS using DEX outside the operating room. Within the PSRC, PPS performed using DEX has a very high success rate and is unlikely to yield a high rate of SAEs.
Clinical use of Dexmedetomidine for Sedation
2017
Current increase of invasive and noninvasive clinical procedures creates great demand for sedation. This sedation may also provide analgesia, anxiolysis, and hypnosis. Protection of respiratory and cardiovascular system is very crucial in anesthesia and intensive care procedures. Thus appropriately given sedation may reduce the duration of surgical procedures, create excellent pain management, improve the quality of technique and increase patient comfort. Moreover, selection, dosing, combination and administration of proper anesthetic medications are important for appropriate sedation and anesthesia.
Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by extra-pyramidal symptoms, cerebellar ataxia, and autonomic dysfunction. The perioperative management of patients with MSA is challenging primarily due to autonomic dysfunction and vocal cord paralysis. Dexmedetomidine (DEX) is a highly selective α2-adrenoreceptor agonist that is used as a sedative, causing minimal depression of the respiratory function. We administered DEX continuously for sedation during spinal anesthesia for bipolar hip arthroplasty in a patient with MSA, without any cardiovascular or respiratory complications. This is the first case report of the efficacy of DEX for the sedation of a patient with MSA during spinal anesthesia. ABSTRACT
Role of dexmedetomidine in pediatric dental sedation
Journal of Dental Anesthesia and Pain Medicine
Dexmedetomidine is a highly selective α2-adrenoceptor agonist with a vast array of properties, making it suitable for sedation in numerous clinical scenarios. Its use was previously restricted to the sedation of intensive care unit patients. However, its use in pediatric dental sedation has been gaining momentum, owing to its high suitability when compared with conventional pediatric sedatives. Its properties range from sedation to anxiolysis to analgesia, due to its sympatholytic properties and minimal respiratory depression ability. Because dexmedetomidine is an efficacious and safe drug, it is gaining importance in pediatric sedation. Thus, the aim of this review is to highlight the properties of dexmedetomidine, its administration routes, its advantages over the commonly used pediatric sedatives, and especially its role as an alternative pediatric sedative.
Journal of medical cases, 2024
Duchenne muscular dystrophy (DMD) is an X-linked inherited dystrophinopathy, with an incidence of 1 in 3,600-5,000 male liveborn infants. The leading cause of death is often cardiomyopathyrelated heart failure. Given the progressive nature of the disorder with involvement of skeletal muscle, respiratory and cardiac function, perioperative care remains challenging with an increased incidence of perioperative morbidity and mortality. Perioperative care can be challenging due to life-threatening perioperative adverse events related to associated end-organ effects, as well as sensitivity to various anesthetic agents, rhabdomyolysis, hyperkalemia, hyperthermia, and cardiac arrest. We present a 22-year-old DMD patient with left ventricular assisted device (LVAD), who presented for repair of both left distal femur and tibial diaphysis fractures. Anesthetic care included the unique combination of total intravenous anesthesia with dexmedetomidine and remimazolam combined with regional anesthesia including a supra-inguinal fascia iliaca block, saphenous nerve block, and popliteal nerve block. The basics of dystrophinopathies are presented, perioperative concerns discussed, and previous reports of the use of regional anesthesia as an adjunct to general anesthesia in adult and pediatric patients with DMD are reviewed.
Dexmedetomidine: An Ideal Drug for Procedural Sedation Outside Operation Theatre
Journal of Evolution of medical and Dental Sciences, 2015
INTRODUCTION: Procedural sedation and analgesia (PSA) is a method of administering sedatives or dissociative agents to patients undergoing unpleasant procedures. It has become the international standard of care for managing acute anxiety and pain, practiced by multiple specialists in varying settings outside the operating room. The aim of procedural sedation is to enable painful procedures to be performed safely and effectively with minimal discomfort to the patient. It is important that the depth of sedation is controlled to achieve these aims without compromising the patient’s airway or causing haemodynamic instability.1 PSA for children is the use of sedative, analgesic, and/or dissociative agents to relieve anxiety and pain associated with diagnostic and therapeutic procedures.2 Various drugs are being used for the procedural sedation e.g. Propofol, midazolam, ketamine and fentanyl. The respiratory depressant effects of opioids, benzodiazines, Propofol and ketamine create the ne...