Recommended Citation
Bawdane K, Ebrahimi F, Dhargalkar J, Yu T. Labor analgesia management of a patient with charcote marie tooth disease. Presented at: Midwest Anesthesia Residents Conference (MARC); April 26, 2025; Indianapolis, IN.
Presentation Notes
Presented at: Midwest Anesthesia Residents Conference (MARC); April 26, 2025; Indianapolis, IN.
Abstract
Introduction: Charcot-Marie-Tooth disease (CMT) is one of the most common inherited sensory & motor polyneuropathies. Most patients present with distal muscle wasting and weakness. One-third of pregnant patients with CMT exhibit exacerbation of symptoms during pregnancy [1]. Anesthesia considerations for CMT are multiple, including variable effects of neuromuscular blocking drugs, reduced pulmonary reserve, difficult positioning etc. Limited data exists regarding use of neuraxial blockade in patients with CMT. We hereby present a case of 31-year-old female with CMT, who successfully received labor epidural analgesia for the vaginal delivery of her baby. Case Presentation: A 31-year-old female, Gravida1, Para 0 (G1P0) with past medical history of CMT, presented at 36 weeks of gestation requesting an epidural for labor analgesia. Her obstetric history was otherwise uncomplicated. She was diagnosed with CMT at 5 years of age & was using braces bilaterally for ambulation. On physical examination, she had bilateral (b/l) lower extremity (LE) weakness and neuropathy. She had 0/5 power for LE dorsiflexion, plantarflexion, inversion, eversion at b/l ankles, 2/5 power for flexion and extension at b/l knees and 4/5 power for b/l hip flexion & extension. She had intact sensation to her bilateral LE. She walked using a walker or cane. She reported no recent changes to her functional status, nor any back surgeries or history of instrumentation to her spine. An epidural was performed at the L4-L5 level in a first attempt for labor analgesia, wherein she received, a test dose of 3 ml of lidocaine-epinephrine (PF) 1.5 %-1:200000, followed by Patient Controlled Epidural Analgesia (PCEA) with epidural infusion containing fentanyl 2 mcg/mL - ropivacaine 0.1% premix. She remained vitally stable and had good pain relief during her entire course of labor (7 hours), which resulted in Normal Spontaneous Vaginal Delivery (NSVD) of a male child. Conclusion: Anesthesia management of patients with CMT is not straightforward and pregnancy makes it even more challenging. Patients with CMT are more sensitive to the effect of neuromuscular blocking agents, moreover some can also present with diaphragmatic involvement which further increases the risk associated with general anesthesia (GA).Though there is paucity of evidence regarding the use of neuraxial anesthesia in CMT, multiple case reports have demonstrated safety of neuraxial block in CMT [2].Our report further strengthens the consensus that neuraxial anesthesia can be a safe alternative to general anesthesia in obstetric patient population. 1. Rudnik-Schöneborn, S. et al. (2020) Eur J Neurol.27(8):1390-1396. 2. Roriz, D. et al. (2019) Regional Anesthesia & Pain Medicine 44: A143 3. Peruzzi, E. et al (2023) Saudi J Anaesth. 17(3):456-457
Type
Oral/Podium Presentation
Affiliations
Advocate Illinois Masonic Medical Center