BACKGROUND: Charcot-Marie-Tooth disease (CMTd) is a hereditary demyelinating peripheral neuropathy that is characterized by progressive muscular atrophy and sensory-motor abnormalities.   The major anesthetic considerations in CMTd are increased sensitivity to non-depolarizing muscle relaxants and the risk of malignant hyperthermia.

CASE REPORT: A 17-year old male patient, 50 kg, ASA III, diagnosed with CMTd was admitted for laparoscopic appendectomy. In the pre-anesthetic evaluation the sensory-motor deficits were more evident in the lower limbs. We performed a Balanced General Anesthesia (BGA) using ASA standard monitoring and bispectral index (BIS). Neuromuscular relaxation was assessed through nerve stimulation of the ulnar nerve with a train-of-four (TOF) stimulus sequence and subsequent quantification of the thumb adductor response. The supramaximal neuromuscular response was measured and recorded using the Organon TOF-Watch SX monitor and rocuronium (0.6mg / kg) was administered. The TOF stimulation was repeated every 5 minutes with a frequency of 2 Hz and an intensity of 70 mA. Orotracheal intubation (OTI) was performed 3 minutes and 30 seconds after non-depolarizing muscle relaxant administration, when the 4 responses to TOF disappeared. During the procedure, neuromuscular blockade monitoring revealed 2 to 3 responses on the TOF-Watch SX monitor. The surgery lasted 90 minutes without complications. For reversion of the residual neuromuscular block, sugammadex (2mg / kg) was used and after 2 minutes a TOF-ratio of 100% was obtained (TOF-ratio of 70% at 1 minute and 20 seconds, and 90% after 1 minute and 45 seconds). The postoperative period was uneventful and the neurological and musculoskeletal examination did not present any new deficits.

DISCUSSION: The main features of CMTd are muscle atrophy and motor-sensory disorders. The response to non-depolarizing neuromuscular relaxants is variable in CMTd and its effects may be prolonged (1). On the other hand, due to the chronic demyelination present, the use of succinylcholine can trigger a hyperkalaemic response (2). There are no references to the use of sugammadex in CMTd.

LEARNING POINTS: In this clinical case we report the absence of adverse events in the intraoperative and postoperative periods of a laparoscopic appendectomy under BGA using a non-depolarizing neuromuscular relaxant in a patient with CMTd. We describe, for the first time, the use of Sugammadex for the reversal of neuromuscular blockade in a patient with CMTd which occurred successfully and without adverse events.

REFERENCES
1. Ohshita N. Anesthetic Management of a Patient With Charcot-Marie-Tooth Disease. 2016;63(2):80-3.

2. Alzaben KR, Samarah OQ, Obeidat SS, Halhouli O, Al Kharabsheh M. Anesthesia For Charcot-Marie-Tooth Disease: Case Report. Middle East journal of anaesthesiology. 2016;23(5):587-90.


06 / Reversion of neuromuscular block with sugammadex in Charcot-Marie-Tooth disease: clinical case.