Anesthetic management of 877 pediatric patients undergoing muscle biopsy for neuromuscular disorders: a 20-year review.

BACKGROUND: The objectives are to review the anesthetic management and anesthetic-related adverse events in patients undergoing muscle biopsy for a broad spectrum of neuromuscular disorders (NMD). AIM: The study aims to assess the hypothesis that perceived awareness of potential anesthesia-induced hyperkalemia and MH in patients with NMD reduces the frequency of such events. METHODS: A 20-year retrospective review of 877 consecutive patients undergoing muscle biopsy to establish diagnoses of NMD has been performed. Patients were categorized prebiopsy into six groups: M (myopathy and muscular dystrophy), MM (mitochondrial or metabolic myopathy), N (neurodegenerative, peripheral neuropathy or spinal muscular atrophy disorder), D (dermatomyositis), C (cardiomyopathy), or S (seizure disorder). Data were collected for demographics, anesthetic management, pre- and postoperative anesthesia-induced muscle injury, postbiopsy histopathologic diagnosis, and concordance comparisons between pre- and postbiopsy diagnoses. RESULTS: There were 513 males (58.5%) and 364 females (41.5%) (1.4:1) with 137 individuals (15.6%) operated on under 1 year of age and two-thirds by 6 years of age. NMD diagnosis was reached in 409 (46.6%) while 468 (53.4%) had no specific pathology. No patients exhibited signs of anesthesia-induced muscle injury (malignant hyperthermia, rhabdomyolysis, cardiac arrest, or postoperative deterioration of weakness). MM was the largest group pre biopsy (367, 41.8%). Anesthetic agents were: nitrous oxide in 657 (74.9%); volatile agents in 139 (15.8%); intravenous agents in 836 (95.3%) (primarily propofol, midazolam, and fentanyl); nondepolarizing muscle relaxants in 404 (46.1%); and regional anesthesia in 112 (12.8%) [most commonly spinal anesthesia in 80 (71.4%)]. Comparing preoperative diagnostic category with postoperative diagnosis, there was a concordance of 78% (319/409) between the two for cases with a definitive diagnosis and 89.7% (787/877) for all cases. CONCLUSIONS: In this retrospective study, no patient exhibited signs or symptoms of hyperkalemia or MH probably because the incidence is very low and becomes even less likely due to the selection of the various anesthetic agents and strategies administered.

Ultrasonography-guided bilateral rectus sheath block vs local anesthetic infiltration after pediatric umbilical hernia repair: a prospective randomized clinical trial.

IMPORTANCE: Regional anesthetic techniques can be used to alleviate postoperative pain in children undergoing pediatric surgical procedures. Use of ultrasonographic guidance for bilateral rectus sheath block (BRSB) has been shown to improve immediate pain scores and reduce use of postoperative analgesia in the postanesthesia care unit (PACU). OBJECTIVE: To compare efficacy of ultrasonography-guided BRSB and local anesthetic infiltration (LAI) in providing postoperative analgesia after pediatric umbilical hernia repair. DESIGN: Prospective, observer-blinded, randomized clinical trial. SETTING: Tertiary-referral urban children's hospital. PARTICIPANTS: Eligible children 3 to 12 years of age undergoing elective umbilical hernia repair from November 16, 2009, through May 31, 2011. INTERVENTIONS: Ropivacaine hydrochloride administered at the conclusion of surgery as LAI by the surgeon (n = 25) or as ultrasonography-guided BRSB by the anesthesiologist (n = 27). MAIN OUTCOMES AND MEASURES: Scores on the FACES Pain Rating Scale measured at 10-minute intervals and all use of analgesic medications in the PACU. RESULTS: Median FACES scores in the PACU were lower in the BRSB group compared with the LAI group at 10 minutes (0 vs 1; P = .04), 30 minutes (0 vs 1; P = .01), and 40 minutes or later (0 vs 1; P = .03). Fewer doses of opioid and nonopioid medications were given to the BRSB group compared with the LAI group (5 vs 11 doses for opioids; 5 vs 10 for nonopioids). CONCLUSIONS AND RELEVANCE: In the PACU, ultrasonography-guided BRSB after umbilical hernia repair in children is associated with lower median FACES scores and decreased use of opioid and nonopioid medications compared with LAI. Future studies could examine the use of longer-acting anesthetic agents with ultrasonography-guided BRSB. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT01015053.

Accuracy of preoperative electrocardiographic and chest radiographic screening for prediction of left ventricular dysfunction in patients with suspected neuromuscular disorders.

BACKGROUND: We sought to determine the reliability of electrocardiography (ECG) and chest radiography (CXR) in predicting left ventricular (LV) dysfunction in patients with suspected neuromuscular disorders (NMDs) undergoing preanesthetic evaluation for muscle biopsy. METHODS: In this retrospective study, 255 patients with a preliminary diagnosis of NMDs based on history, physical examination, and laboratory testing underwent preanesthetic screening before muscle biopsy. The screening included various combinations of ECG, CXR, and transthoracic echocardiography (Echo) to assess perioperative risk associated with potentially undiagnosed LV dysfunction. Multivariate logistic regression analysis was applied to ascertain whether CXR and ECG were independently predictive of LV dysfunction. In addition, receiver-operating characteristic curve analysis was used to assess the diagnostic accuracy of each test and the combination of CXR and ECG in differentiating LV dysfunction from normal function based on Echo "gold standard" data. RESULTS: The study consisted of 255 patients who had a transthoracic Echo, and among these patients, 235 had CXR and 237 had ECG. Forty-four patients were diagnosed by transthoracic Echo to have LV dysfunction (17.3%). Of the 255 patients in the study population, 24 were found to have mild LV dysfunction (9.4%) and 20 had moderate to severe LV dysfunction (7.8%) on Echo. With Echo providing the definitive standard for the diagnosis of LV dysfunction, we found that a CXR alone was predictive in 37% of cases of LV dysfunction, an ECG alone was predictive in 14% of cases, and the combination of both was predictive in 81% of cases. The combination of ECG and CXR test offered the highest diagnostic accuracy (area under the curve of 0.95, P < 0.0001) for differentiating moderate to severe LV dysfunction from normal LV function. CONCLUSIONS: In patients with suspected neuromuscular disease, CXR and ECG provided low independent diagnostic prediction for the presence or absence of LV dysfunction. The combination of both tests can identify cardiomyopathy with relatively high accuracy in children with suspected NMDs independent of age and gender, particularly in patients with moderate to severe LV dysfunction. Although our findings suggest that combination ECG and CXR screening is a reliable means of detecting LV dysfunction, this approach fails to differentiate the severity or type of cardiomyopathy that may exist. Therefore, the decision to obtain a perioperative Echo before muscle biopsy should involve careful consideration of the disease suspected, ECG and CXR results, laboratory studies, patient age, physical examination, and family history.