Myotonic Dystrophy Confirmed after Cesarean Section

We report a case of Myotonic dystrophy (MD) confirmed after cesarean section under general anesthesia. MD is a common neuromuscular disease in adults, and is characterized by myotonia, and myopathy of the voluntary and involuntary muscles. Patients with MD are at high risk during general anesthesia. A 33-year-old pregnant woman with hydramnios was admitted to the delivery room, and emergency cesarean section was performed under general anesthesia using 40 mg of rocuronium. After the surgery, the anesthesiologist confirmed partial neuromuscular blockade by train-of-four electrical stimulation and neostigmine 1mg was used for reversal of neuromuscular blocking agents. However, as the patient’s self-respiration was continuously weak, sugammadex 200 mg was additionally administered and then the patient was extubated. In the postanesthesia care unit, she was alert and cooperated well, but presented with shallow breathing continuously; and arterial blood sampling revealed respiratory acidosis, hypoventilation, and hypercapnia. Due to diminished consciousness, reintubation was performed, and the patient was admitted to the intensive care unit (ICU). On both the second and third day after ICU admission, she met the spontaneous breathing trial (SBT) safety criteria respectively which consists of inspiratory pressure < –25 cmH2O, tidal volume > 5 mL/kg, vital capacity > 10 mL/kg, minute ventilation < 10 L/min, and rapid shallow breathing index < 100. Extubations was performed on the second and third day post admission, but each time we had to reintubate the patient because of consistent hypercapnia, shallow breathing, and sequential desaturation within several hours. For diagnosis, we obtained medical history about the patient’s shallow breathing pattern. Her husband reported that she had “a feeling of weakness” and exhibited upper eyelid drooping in the evening during the third trimester of pregnancy. Gene analysis performed on the seventh day after ICU admission revealed expansion of