What is the role of surgery in the treatment of Charcot-Marie-Tooth (CMT) disease?

Updated: Feb 19, 2019
  • Author: Francisco de Assis Aquino Gondim, MD, MSc, PhD, FAAN; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

Answer

Depending on the degree of foot deformities, patients may benefit from Achilles tendon lengthening, tendon transfers, hammertoe correction, and release of the plantar fascia. However, such surgeries can often be prevented by conservative measures and lifelong follow-up with physical therapists. Patients should only be referred to orthopedic surgeons or podiatrists with specific training with foot surgery and experience with CMT. Similarly, because of concerns that the median and ulnar nerves may be more sensitive to manipulation in CMT patients, special caution must be exercised during entrapment surgery. The authors routinely refer patients for separate opinions from more than one surgeon. Orthopedic surgeons also play a role in the treatment of secondary joint problems at more proximal sites and in the evaluation and treatment of scoliosis.

In a series of 161 surgical procedures performed on 86 patients with CMT, the patients had no difficulties tolerating anesthetics, even with succinylcholine. However, in patients who are rapidly becoming weak from CMT, using succinylcholine may be inadvisable. Nitrous oxide, by inactivating the cobalamin-dependent enzyme methionine synthase, may be neurotoxic. Other risks, including sensitivity to neuromuscular blocking agents and malignant hyperthermia, are reported to be minimal.

Prolonged body and limb positions can result in nerve compression. Regional anesthesia is relatively contraindicated in CMT.


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