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Background: Duchenne muscular dystrophy (DMD) possesses many potential challenges for anesthetic care. Invasive and noninvasive procedures with corresponding sedation or general anesthesia are frequent and necessary for affected patients. There remains a need for a better agent or agents for procedural sedation in patients with comorbid diseases. This study prospectively evaluated a combination of ketamine with two different doses of dexmedetomidine for sedation during muscle biopsy in patients with DMD., Methods: Dexmedetomidine 1.0 or 0.5 μg·kg(-1) was administered as a loading dose over 3 min followed by a continuous infusion of 1.0 or 0.5 μg·kg·h(-1). Ketamine (1 mg·kg(-1)) was administered along with the dexmedetomidine loading dose. As the procedure commenced, additional doses of ketamine (0.5 mg·kg(-1)) were administered as needed. Sedation scores, hemodynamic data, operative times, and recovery times were recorded., Results: The study cohort included a total of 53 bicep, deltoid, or anterior tibialis muscle biopsies in 19 boys including 24 in the dexmedetomidine 1.0 μg·kg(-1) group and 29 in the dexmedetomidine 0.5 μg·kg(-1) group. Mean age and weight were 9.7 ± 1.4 years and 33.3 ± 7.7 kg in the dexmedetomidine 1.0 μg·kg(-1) group and 8.8 ± 1.8 years and 30.2 ± 10.8 kg in the dexmedetomidine 0.5 μg·kg(-1) group. No significant changes in blood pressure were noted. A decrease in heart rate (HR) occurred after the loading dose of dexmedetomidine in both groups. The HR was significantly lower in the dexmedetomidine 1.0 μg·kg(-1) group compared with the dexmedetomidine 0.5 μg·kg(-1) group. Total recovery time to discharge was significantly shorter in the dexmedetomidine 0.5 μg·kg(-1) group than the dexmedetomidine 1.0 μg·kg(-1) group (146 ± 65 vs 174 ± 58 min; P = 0.03), although the total ketamine dose was significantly greater in the dexmedetomidine 0.5 μg·kg(-1) group (3.7 ± 1.0 vs 2.0 ± 0.5 mg·kg(-1); P < 0.01). There were no episodes of apnea or hypoventilation; however, a jaw thrust was needed in one patient in the dexmedetomidine 1.0 μg·kg(-1) group., Conclusion: The combination of dexmedetomidine and ketamine is safe and effective for moderately painful procedures with limited respiratory and cardiovascular effects in a high-risk patient population. Dexmedetomidine 0.5 μg·kg(-1) as a loading dose with ketamine followed by a continuous infusion of dexmedetomidine at 0.5 μg·kg(-1) ·h(-1) achieved an adequate sedation level with shorter total recovery times in the perioperative unit compared with a higher dose regimen of dexmedetomidine (1.0 μg·kg(-1) loading dose followed by an infusion at 1.0 μg·kg(-1) ·h(-1))., (© 2014 John Wiley & Sons Ltd.)

Background Mucopolysaccharidosis type III is a progressive disease with worsening airway, pulmonary, and cardiac involvement that may complicate anesthetic care. Aim To prospectively evaluate the incidence of airway issues and complications during magnetic resonance imaging (MRI) and lumbar puncture (LP) during general anesthesia with a native airway for patients with mucopolysaccharidosis type III. Method The study was a part of the natural history study. Anesthesia was induced with sevoflurane, which was discontinued after intravenous access was obtained. General anesthesia with a native airway was provided by dexmedetomidine and propofol. Dexmedetomidine (0.5 μg·kg−1) was administered over 5 min followed by a continuous infusion at 0.5 μg·kg−1·h−1. A continuous infusion of propofol was started at 150 μg·kg−1·min−1. A bolus dose of propofol (1 mg·kg−1) was administered and the propofol infusion was increased as needed. Airway management and vital signs were recorded for the entire procedure until discharge. Results Twenty-five patients (6.9 ± 3.1 years) received total of 43 MRI and LP procedures in the cohort. No patient failed sedation. Although mask induction with sevoflurane was not clinically problematic, upper airway obstruction was noted during 14 procedures (33%). This required the application of continuous positive airway pressure, temporary oral airway placement, jaw thrust, or shoulder roll. Airway dynamics improved once the anesthesia was transitioned to intravenous anesthetic agents. Although a small shoulder roll was needed to improve airway patency for 11 cases (26%), a large shoulder roll tended to make the upper airway obstruction worse. Oxygen desaturation (≤90%) was noted during MRI in three cases (7%). Conclusion A combination of dexmedetomidine and propofol provided effective general anesthesia with a native airway during the procedures. Although upper airway obstruction was noted, it resolved with simple airway maneuvers without further airway intervention. [ABSTRACT FROM AUTHOR]

Sedation during invasive procedures not only provides appropriate humanitarian care for patients, but also facilitates the completion of invasive procedures. Although generally safe and effective, adverse effects may occur especially in patients with co-morbid diseases. We present the successful use of a combination of dexmedetomidine and ketamine to provide sedation and analgesia in a 21-year-old patient with Duchenne muscular dystrophy (DMD) undergoing bone marrow aspiration and biopsy. Comorbidities included both depressed myocardial function and impaired respiratory function. Dexmedetomidine was administered as a loading dose of 1 μg/kg over 5 min followed by an infusion of 1 μg/kg/h. Ketamine (20 mg) was administered along with the dexmedetomidine loading dose. An additional 10 mg of ketamine was administered to treat the pain experienced during the placement of the local anesthetic agent prior to the procedure. No clinically significant hemodynamic or respiratory changes were noted. The patient tolerated the procedure well and was discharged home. A review of previously published reports of dexmedetomidine and ketamine for procedural sedation are reviewed. [ABSTRACT FROM AUTHOR]