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

Abstract

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.