Anesthetic Hypersensitivity in a Case-Controlled Series of Patients With Mitochondrial Disease.

BACKGROUND: Children with mitochondrial disease undergo anesthesia for a wide array of surgical procedures. However, multiple medications used for their perioperative care can affect mitochondrial function. Defects in function of the mitochondrial electron transport chain (ETC) can lead to a profound hypersensitivity to sevoflurane in children. We studied the sensitivities to sevoflurane, during mask induction and maintenance of general anesthesia, in children presenting for muscle biopsies for diagnosis of mitochondrial disease. METHODS: In this multicenter study, 91 children, aged 6 months to 16 years, presented to the operating room for diagnostic muscle biopsy for presumptive mitochondrial disease. General anesthesia was induced by a slow increase of inhaled sevoflurane concentration. The primary end point, end-tidal (ET) sevoflurane necessary to achieve a bispectral index (BIS) of 60, was recorded. Secondary end points were maximal sevoflurane used to maintain a BIS between 40 and 60 during the case, and maximum and minimum heart rate and blood pressures. After induction, general anesthesia was maintained according to the preferences of the providers directing the cases. Primary data were analyzed comparing data from patients with complex I deficiencies to other groups using nonparametric statistics in SPSS v.27. RESULTS: The median sevoflurane concentration to reach BIS of 60 during inductions (ET sevoflurane % [BIS = 60]) was significantly lower for patients with complex I defects (0.98%; 95% confidence interval [CI], 0.5-1.4) compared to complex II (1.95%; 95% CI, 1.2-2.7; P < .001), complex III (2.0%; 95% CI, 0.7-3.5; P < .001), complex IV (2.0%; 95% CI, 1.7-3.2; P < .001), and normal groups (2.2%; 95% CI, 1.8-3.0; P < .001). The sevoflurane sensitivities of complex I patients did not reach significance when compared to patients diagnosed with mitochondrial disease but without an identifiable ETC abnormality (P = .172). Correlation of complex I activity with ET sevoflurane % (BIS = 60) gave a Spearman's coefficient of 0.505 (P < .001). The differences in sensitivities between groups were less during the maintenance of the anesthetic than during induction. CONCLUSIONS: The data indicate that patients with complex I dysfunction are hypersensitive to sevoflurane compared to normal patients. Hypersensitivity was less common in patients presenting with other mitochondrial defects or without a mitochondrial diagnosis.

Anesthetic considerations in patients with mitochondrial defects.

Mitochondrial disease, once thought to be a rare clinical entity, is now recognized as an important cause of a wide range of neurologic, cardiac, muscle, and endocrine disorders . The incidence of disorders of the respiratory chain alone is estimated to be about 1 per 4-5000 live births, similar to that of more well-known neurologic diseases . High-energy requiring tissues are uniquely dependent on the energy delivered by mitochondria and therefore have the lowest threshold for displaying symptoms of mitochondrial disease. Thus, mitochondrial dysfunction most commonly affects function of the central nervous system, the heart and the muscular system . Mutations in mitochondrial proteins cause striking clinical features in those tissues types, including encephalopathies, seizures, cerebellar ataxias, cardiomyopathies, myopathies, as well as gastrointestinal and hepatic disease. Our knowledge of the contribution of mitochondria in causing disease or influencing aging is expanding rapidly . As diagnosis and treatment improve for children with mitochondrial diseases, it has become increasingly common for them to undergo surgeries for their long-term care. In addition, often a muscle biopsy or other tests needing anesthesia are required for diagnosis. Mitochondrial disease represents probably hundreds of different defects, both genetic and environmental in origin, and is thus difficult to characterize. The specter of possible delayed complications in patients caused by inhibition of metabolism by anesthetics, by remaining in a biochemically stressed state such as fasting/catabolism, or by prolonged exposure to pain is a constant worry to physicians caring for these patients. Here, we review the considerations when caring for a patient with mitochondrial disease.

Comparison of the Truview PCD™ and the GlideScope(®) video laryngoscopes with direct laryngoscopy in pediatric patients: a randomized trial.

INTRODUCTION: The GlideScope(®) video laryngoscope has a 60° angled blade and the blade of the Truview PCD™ video laryngoscope has an optical lens that provides a 46° refraction of the viewing angle. Despite successful results using the GlideScope in adults, few studies have been published regarding its use in pediatric patients. We therefore tested our joint primary hypothesis that the GlideScope and the Truview PCD video laryngoscopes provide superior visualization to direct laryngoscopy and are non-inferior regarding time to intubation. METHODS: One hundred thirty-four patients (neonate to ten years of age, American Society of Anesthesiologists physical status I-III) scheduled for general surgical procedures were randomized to tracheal intubation using the Truview PCD or GlideScope video laryngoscope or direct laryngoscopy (Macintosh blade). The laryngoscopic view was scored using the Cormack-Lehane scale. Time to intubation (defined as the time from the moment the device entered the patient's mouth until end-tidal CO2 was detected) and the number of attempts were recorded. RESULTS: The Cormack-Lehane views attained using the GlideScope (P > 0.99) and Truview PCD (P = 0.18) were not superior to the views attained with direct laryngoscopy. Furthermore, the view attained using the GlideScope was significantly worse than that attained using direct laryngoscopy (P < 0.001). Fewer patients showed Cormack-Lehane grade I views with the GlideScope than with the Truview PCD (14% vs 82%, respectively; 95% confidence interval [CI] -91% to -46%). The observed median [Q1, Q3] times to intubation were: 39 [31, 59] sec, 44 [28, 62] sec, and 23 [21, 28] sec with the GlideScope, Truview PCD, and direct laryngoscopy, respectively, with median differences of 14 sec (95% CI 7 to 26, GlideScope - direct laryngoscopy) and 17 sec (95% CI 6 to 28, Truview PCD - direct laryngoscopy). CONCLUSION: The Cormack-Lehane views attained using the GlideScope and the Truview PCD video laryngoscopes were not superior to views attained using direct laryngoscopy. Visualization with the GlideScope was significantly worse than with direct laryngoscopy. Use of the GlideScope and Truview PCD systems should be restricted to patients with specific indications.

Surgery for catastrophic epilepsy in infants 6 months of age and younger.

OBJECT: Few data are available concerning efficacy and safety of surgery for catastrophic epilepsy in the first 6 months of life. METHODS: The authors retrospectively analyzed epilepsy surgeries in 15 infants ranging in age from 1.5 to 6 months (median 4 months) and weight from 4 to 10 kg (median 7 kg) who underwent anatomical (4 patients) or functional (7 patients) hemispherectomy, or frontal (1 patient), frontoparietal (2 patients), or parietooccipital (1 patient) resection for life-threatening catastrophic epilepsy due to malformation of cortical development. RESULTS: No patient died. Intraoperative complications included an acute ischemic infarction with hemiparesis in our youngest, smallest infant. The most frequent complication was blood loss requiring transfusion, which was encountered in every case. The estimated blood loss was 3-214% (median 63%) of the total blood volume. At maximum follow-up of 6-121 months (median 60 months), 46% were seizure free. CONCLUSIONS: Epilepsy surgery may be effective in young infants as it is in older children. However, intraoperative blood loss and risk of permanent postoperative neurological deficits present significant challenges.

Epidural analgesia for postoperative pain control after adolescent spinal fusion procedures which violated the epidural space.

STUDY DESIGN: Retrospective chart review. OBJECTIVE: To determine the effectiveness of epidural analgesia in scoliosis patients with an opened (violated) spinal canal. BACKGROUND: Postoperative continuous epidural analgesia using local anesthetics and/or opioids has been described with good success after posterior scoliosis correction. One of the concerns with the use of epidural catheters for pain control is the reliability and distribution of analgesic medication when the epidural space is violated intraoperatively by Smith-Petersen osteotomies or placement of multiple sublaminar wires. METHODS: The medical records of 55 patients who underwent pediatric deformity surgery for scoliosis at a single university center over 1-year period (January 2007 to January 2008) were reviewed. Patient demographic, surgical, and postoperative data were collected. A chi test was used to make comparisons on the basis of categorical data, whereas analysis of variance tests were used to make comparisons on the basis of means. Statistical significance was defined as a probability value of <0.5. RESULTS: Twenty-nine patients were managed with epidural analgesia, whereas 26 patients were managed with intravenous patient-controlled analgesia (IV PCA). Seven of the 29 patients with epidural analgesia had a violation of their epidural space during their surgery, which did not alter the efficacy of their postoperative analgesia with an epidural. Epidural analgesia provided a lower mean pain score (P=0.022) over the first 24 hours and fewer pain scores over 4 (P=0.050) during that same time period than IV PCA for all patients as well as those with a violated spinal canal. There was no statistical difference in the rates of pruritus, nausea, ileus, constipation, urinary retention, change in neurologic examination, or rate of infection between the patients with a violated spinal canal and those with an intact spinal canal. CONCLUSIONS: Overall, patients managed with an epidural had significantly lower pain scores than those managed with IV PCA despite violation of the epidural space.

Dexmedetomidine for deep brain stimulator placement in a child with primary generalized dystonia: case report and literature review.

Dexmedetomidine, which is a relatively selective alpha2-adrenoceptor agonist, is used for sedation and analgesia in intensive care unit patients, during awake craniotomies in pediatric and adult patients, and during magnetic resonance imaging, with minimal depression of respiratory function. The successful use of dexmedetomidine in a pediatric patient undergoing bilateral deep brain stimulator placement for the treatment of generalized dystonia, is presented.

Anesthesia for minimally invasive cranial and spinal surgery.

The field of minimally invasive neurosurgery has evolved rapidly in its indications and applications over the last few years. New, less invasive techniques with low morbidity and virtually no mortality are replacing conventional neurosurgical procedures. Providing anesthesia for these procedures differs in many ways from conventional neurosurgical operations. Anesthesiologists are faced with the perioperative requirements and risks of newly developed procedures. This review calls attention to the anesthetic issues in various minimally invasive neurosurgical procedures for cranial and spinal indications. Among the procedures specifically discussed are endoscopic third ventriculostomy, endoscopic transsphenoidal hypophysectomy, endoscopic strip craniectomy, deep brain stimulation, video-assisted thorascopic surgery, vertebroplasty and kyphoplasty, cervical discectomy and foraminectomy, and laparoscopically assisted lumbar spine surgery.

Shock-wave lithotripsy of renal stones situated adjacent to electronic nerve stimulators.

Shock-wave lithotripsy is commonly employed for the treatment of nephrolithiasis in the pediatric patient population. This report describes such treatment of a patient with electronic nerve stimulators that were located in close proximity to bilateral renal pelvic stones. Precautions and considerations of shock-wave lithotripsy in this context are discussed.

Unexpected ST segment changes in children--a case report.

In children, myocardial ischemic changes during anesthesia are a rare event unless there is underlying pathology. The patient in this case report was an apparently healthy child scheduled for adenoidectomy and bilateral tympanostomy. Occurrence of significant ST changes as well as intraoperative and postoperative hypoxemia required further diagnostic work-up. Postoperative echocardiographic findings were suspicious of intrapulmonary right to left shunting. The pulmonary arteriovenous fistula is probably the major pathophysiological factor for the development of hypoxemia and paradoxical air embolism especially during positive pressure ventilation in our patient. Unexpected ST segment changes might also occur in patients with anomalous origin of coronary arteries. Although diagnostic work-up was inconclusive, it is necessary to rule out any underlying pathological process. Further follow-up is also important in order to learn more about these disease states that often have subclinical, but potentially fatal presentation.