ANAESTHETIC MANAGEMENT IN A PATIENT WITH CHARCOT-MARIE-TOOTH DISEASE UNDERGOING TRIPLE ARTHRODESIS OF THE FOOT

Abstract

Background: Charcot-Marie-Tooth (CMT) disease is an inherited peripheral neuropathy that causes progressive muscle weakness and atrophy, particularly in the distal limbs. Anesthetic management for patients with CMT is challenging due to risks of neurotoxicity, altered responses to anesthetic agents, and potential for exacerbating muscle weakness. This report details the anesthetic care for a pediatric patient with CMT undergoing corrective foot surgery.

Case Presentation: A 19-year-old male with a confirmed diagnosis of CMT was scheduled for a triple arthrodesis of the foot to address progressive deformities and instability. Preoperative assessment revealed significant distal muscle wasting and sensory loss in the lower limbs, but no significant respiratory or cardiac compromise. The anesthetic plan prioritized regional anesthesia to minimize systemic drug exposure. The patient received a low-dose subarachnoid block with hyperbaric bupivacaine and a popliteal nerve block with ropivacaine for postoperative analgesia. The intraoperative course was stable, and neuromuscular function was closely monitored. The postoperative recovery was uneventful, with effective pain control achieved through multimodal analgesia, primarily using NSAIDs and paracetamol.

Discussion: The anesthetic management of patients with CMT requires careful consideration of their heightened sensitivity to both general and local anesthetics, as well as neuromuscular blocking agents. While regional anesthesia is beneficial for postoperative pain, it must be administered cautiously to avoid worsening the existing neuropathy. In this case, a balanced approach combining a carefully titrated subarachnoid block with diligent neuromuscular monitoring proved to be a safe and effective strategy, resulting in an uncomplicated postoperative course.

Conclusion: This case highlights the critical importance of a tailored anesthetic plan for patients with Charcot-Marie-Tooth disease. An approach that utilizes titrated regional anesthesia combined with close intraoperative monitoring can provide safe and effective management for patients undergoing orthopedic procedures such as triple arthrodesis.