Pediatric Charcot-Marie-Tooth disease and peripheral nerve blocks: a retrospective cohort study of 25 patients.

PURPOSE: Charcot-Marie-Tooth (CMT) disease is an inherited neurologic disorder characterized by progressive peripheral neuropathies. The use of peripheral nerve blocks (PNB) in patients with CMT disease has been controversial because of concerns about exacerbating existing neurologic impairments and the "double hit" hypothesis. We aimed to assess the use of PNB in pediatric patients with CMT disease undergoing orthopedic surgery to address the limited data available in the literature on this topic. METHODS: In this retrospective cohort study, we included all pediatric patients with CMT disease scheduled for orthopedic surgery receiving PNB at our centre. All of the patients had preoperative neurologic exams and received one or more ultrasound-guided regional anesthesia techniques. Data extracted included details of anesthesia technique, surgical procedure, opioid consumption, and pain scores during the first three postoperative days. We also reviewed any complications such as neurologic deficits and local anesthetic toxicity. We used descriptive statistics to summarize the findings. RESULTS: We included 25 patients, 14 of whom (56%) presented with pre-existing neurologic deficits, primarily in the lower extremities. Postoperative assessments revealed no new neurologic impairments in 24/25 (96%) patients, with only one patient experiencing a nerve injury possibly related to the surgical procedure. Opioid consumption was low in the postanesthesia care unit and on the day of surgery. No additional complications were noted in the first 72 hr after surgery. CONCLUSION: Despite concerns, PNB showed favourable outcomes in a pediatric cohort with CMT disease, with low opioid consumption and pain scores and minimal complications during follow-up. These findings match previous reports of adult patients with CMT disease and suggest that the benefits of PNB may outweigh the perceived risks in pediatric patients with CMT disease.

RéSUMé: OBJECTIF: La maladie de Charcot-Marie-Tooth (CMT) est une maladie neurologique héréditaire caractérisée par des neuropathies périphériques progressives. L'utilisation de blocs nerveux périphériques (BNP) chez la patientèle atteinte de CMT est controversée en raison des inquiétudes concernant l'exacerbation des déficiences neurologiques existantes et de l'hypothèse d'une « double insulte ¼. Notre objectif était d'évaluer l'utilisation de BNP chez les patient·es pédiatriques atteint·es de CMT bénéficiant d'une chirurgie orthopédique afin de pallier les données limitées disponibles dans la littérature à ce sujet. MéTHODE: Dans cette étude de cohorte rétrospective, nous avons inclus tou·tes les patient·es pédiatriques atteint·es de CMT devant bénéficier d'une chirurgie orthopédique et recevant un BNP dans notre centre. Tou·tes ont bénéficié d'examens neurologiques préopératoires et ont reçu une ou plusieurs techniques d'anesthésie régionale échoguidées. Les données extraites comprenaient des détails sur la technique d'anesthésie, l'intervention chirurgicale, la consommation d'opioïdes et les scores de douleur au cours des trois premiers jours postopératoires. Nous avons également examiné toutes les complications telles que les déficits neurologiques et la toxicité des anesthésiques locaux. Nous avons utilisé des statistiques descriptives pour résumer les résultats. RéSULTATS: Nous avons inclus 25 patient·es, dont 14 (56 %) présentaient des déficits neurologiques préexistants, principalement dans les membres inférieurs. Les évaluations postopératoires n'ont révélé aucune nouvelle déficience neurologique chez 24 patient·es sur 25 (96 %), une seule personne ayant subi une lésion nerveuse possiblement liée à l'intervention chirurgicale. La consommation d'opioïdes était faible en salle de réveil et le jour de l'opération. Aucune complication supplémentaire n'a été notée dans les 72 premières heures après la chirurgie. CONCLUSION: Malgré les inquiétudes, le BNP a montré des résultats favorables dans une cohorte pédiatrique atteinte de CMT, avec une faible consommation d'opioïdes et des scores de douleur et des complications minimes pendant le suivi. Ces résultats correspondent à des comptes rendus antérieurs de patient·es adultes atteint·es de CMT et suggèrent que les avantages des BNP pourraient l'emporter sur les risques perçus chez la patientèle pédiatrique atteinte de CMT.

Validity of claims-based algorithms to identify neurodevelopmental disorders in children.

PURPOSE: To validate healthcare claim-based algorithms for neurodevelopmental disorders (NDD) in children using medical records as the reference. METHODS: Using a clinical data warehouse of patients receiving outpatient or inpatient care at two hospitals in Boston, we identified children (≤14 years between 2010 and 2014) with at least one of the following NDDs according to claims-based algorithms: autism spectrum disorder/pervasive developmental disorder (ASD), attention deficit disorder/other hyperkinetic syndromes of childhood (ADHD), learning disability, speech/language disorder, developmental coordination disorder (DCD), intellectual disability, and behavioral disorder. Fifty cases per outcome were randomly sampled and their medical records were independently reviewed by two physicians to adjudicate the outcome presence. Positive predictive values (PPVs) and 95% confidence intervals (CIs) were calculated. RESULTS: PPVs were 94% (95% CI, 83%-99%) for ASD, 88% (76%-95%) for ADHD, 98% (89%-100%) for learning disability, 98% (89%-100%) for speech/language disorder, 82% (69%-91%) for intellectual disability, and 92% (81%-98%) for behavioral disorder. A total of 19 of the 50 algorithm-based cases of DCD were confirmed as severe coordination disorders with functional impairment, with a PPV of 38% (25%-53%). Among the 31 false-positive cases of DCD were 7 children with coordination deficits that did not persist throughout childhood, 7 with visual-motor integration deficits, 12 with coordination issues due to an underlying medical condition and 5 with ADHD and at least one other severe NDD. CONCLUSIONS: PPVs were generally high (range: 82%-98%), suggesting that claims-based algorithms can be used to study NDDs. For DCD, additional criteria are needed to improve the classification of true cases.

Extremity fracture pain after emergency department reduction and casting: predictors of pain after discharge.

STUDY OBJECTIVE: The aims of this study are to determine the prevalence of pediatric extremity fracture pain after emergency department (ED) discharge, compare pain severity between fractures requiring simple casting versus sedated reduction and casting, and explore predictors of postdischarge pain. METHODS: This is a prospective observational study of children aged 4 to younger than 18 years and presenting to the ED with extremity fracture from May 2010 to February 2011. The Parents' Postoperative Pain Measure, which scores pain according to 15 behavior-related questions, was completed 48 to 72 hours after discharge. A score greater than or equal to 6 of 15 indicates clinically meaningful pain. Univariate tests and multivariable regression analyses were used to compare Parents' Postoperative Pain Measure scores between cohorts. RESULTS: Two hundred fifty-seven patients were enrolled; 202 (79%) had Parents' Postoperative Pain Measure scores for analysis. Pain scores greater than or equal to 6 were reported by 37 of 102 (36%) of the simple casted and 44 of 100 (44%) of the reduced casted children. There was no difference in scores between the simple (median 4.0) and reduced casted (median 5.0) cohorts (difference 16.7%; 95% confidence interval [CI] -3.0% to 40%). In the multivariate analysis, ED narcotic administration was associated with 24% higher Parents' Postoperative Pain Measure scores (95% CI 0.95% to 53.6%). Children receiving ED narcotics had more than 2 times increased odds of pain scores greater than or equal to 6 after discharge (95% CI 1.24 to 5.39). CONCLUSION: Children in both simple casted and reduced casted groups had clinically meaningful pain after ED discharge. Identifying these children is important to improving pain management and discharge care.