A retrospective observational cohort study of the anesthetic management and outcomes of pediatric patients with Alexander disease undergoing lumbar puncture or magnetic resonance imaging.

BACKGROUND: Alexander disease is a rare, progressive leukodystrophy, which predisposes patients to complications under general anesthesia due to clinical manifestations including developmental delay, seizures, dysphagia, vomiting, and sleep apnea. However, study of anesthetic outcomes is limited. AIMS: Our aim was to describe patient characteristics, anesthetic techniques, and anesthesia-related complications for Alexander disease patients undergoing magnetic resonance imaging and/or lumbar puncture at a quaternary-care children's hospital. METHODS: We performed a retrospective review of anesthetic outcomes in patients with Alexander disease enrolled in a prospective observational study. Included patients had diagnosed Alexander disease and underwent magnetic resonance imaging and/or lumbar puncture at our institution. We excluded anesthetics for other procedures or at outside institutions. Collected data included patient characteristics, anesthetic techniques, medications, and complications under anesthesia and in the subsequent 24 h. We performed descriptive statistics as appropriate. RESULTS: Forty patients undergoing 64 procedures met inclusion criteria. Fifty-six procedures (87.5%) required general anesthesia or monitored anesthesia care (MAC) and eight (12.5%) did not. The general anesthesia/MAC group tended to be younger than nonanesthetized patients (median age 6 years [IQR 3.8; 9] vs. 14.5 years [IQR 12.8; 17.5]). In both groups, dysphagia (78.6% vs. 87.5%, respectively), seizures (62.5% vs. 25%), and recurrent vomiting (17.9% vs. 25%) were frequently reported preprocedure symptoms. Inhalational induction was common (N = 48; 85.7%), and two (3.6%) underwent rapid sequence induction. Serious complications were rare, with no aspiration or seizures. Hypotension resolving with ephedrine occurred in eight cases (14.3%). One patient each (1.8%) experienced postprocedure emergence agitation or vomiting. Fifty-three (94.6%) were ambulatory procedures. No inpatients required escalation in acuity of care. CONCLUSIONS: In this single-center study, patients with Alexander disease did not experience frequent or irreversible complications while undergoing general anesthesia/MAC. Co-morbid symptoms were not increased postanesthesia. Some patients may not require anesthesia to complete short procedures.

A retrospective comparison of thoracic epidural infusion and multimodal analgesia protocol for pain management following the minimally invasive repair of pectus excavatum.

BACKGROUND: Pain management following minimally invasive repair of pectus excavatum is variable. We recently adopted a comprehensive multimodal analgesic protocol that standardizes perioperative analgesic management. We hypothesized that patients managed with this protocol would use more opioids postoperatively, have similar pain control, and shorter length of stay compared to patients managed with thoracic epidural infusion. AIMS: We retrospectively compared opioid consumption, pain scores, and length of stay between a cohort of patients managed with our multimodal analgesic protocol and a cohort managed with a thoracic epidural infusion. METHODS: This retrospective cohort comparison includes patients, 8 to 21 years of age, managed with either thoracic epidural infusion (n = 21) or multimodal analgesic protocol (n = 29) following minimally invasive repair of pectus excavatum from January 1, 2011 through September 15, 2015. The primary outcome, total daily opioid consumption in morphine equivalents, is presented as an average by postoperative day. Secondary outcomes included median daily pain score and length of stay. RESULTS: Patients were similar in age, weight, sex, and physical status. Patients managed with thoracic epidural infusion received less opioid (morphine equivalents-mg/kg) intraoperatively compared to multimodal analgesic protocol (difference of mean [95% confidence interval] 0.22 [0.16-0.28] P ≤ .01) but required more total opioid through postoperative day 3 (difference of mean [95% confidence interval] 1.2 [0.26-2.14] P = .01). We did not observe a difference in pain scores. Median length of stay was 1 day less in patients managed with multimodal analgesic protocol (difference of median [95% confidence interval] 1 [0.3-1.7] P = .003). CONCLUSION: Implementation of a standardized comprehensive multimodal analgesic protocol following minimally invasive repair of pectus excavatum resulted in equivalent analgesia with a modest reduction in length of stay when compared to thoracic epidural. We did not observe an opioid sparing effect in our thoracic epidural which may reflect technique variability.

Do pediatric chief residents recognize details of prepubertal female genital anatomy: a national survey.

OBJECTIVE: To evaluate how well a group of recently trained pediatric chief residents could label anatomic structures on two different photographs of female prepubertal genitalia. Additionally, the study sought to explore aspects of pediatric training in sexual abuse and clinical practice issues surrounding the routine genital examination. METHOD: A 38-item questionnaire was mailed to pediatric chief residents at all of the officially listed pediatric residency-training programs in the continental US. Comparisons were made between this study and the responses to two previous surveys, which asked a more heterogeneous group of physicians to label one of the photographs used in the study. The second photograph was added because of its improved clarity of each anatomic structure when compared to the first photograph used in the previous studies. The study also asked about clinical practice issues surrounding the prepubertal genital examination. RESULTS: An overall response rate of 73% was achieved and analysis was done on 139 respondents. One-half of chief residents thought that their training during residency on sexual abuse was inadequate for practice. Sixty-four percent of chief residents correctly labeled the hymen on the photograph used in the previous studies, which was not significantly different from the 62% and 59% of physicians who correctly labeled the hymen in the previous surveys. In the second photograph, which more clearly displayed the various anatomic structures, 71% correctly labeled the hymen. CONCLUSION: Pediatric chief residents reported variable amounts of training on issues pertaining to child sexual abuse during residency, think that this time was inadequate, and, while doing slightly better than a more diverse group of previously studied physicians, did not achieve 100% accuracy in identifying basic genital structures correctly on two different photographs.