講演情報
[PE4-5]術後抜管困難を契機に診断された筋緊強直性ジストロフィーの１例
○甲斐 健太郎1, 山田 知徳1, 深野 菜摘2, 軸丸 美香3, 衛藤 聡1, 栗林 由英2, 河野 康志1 (1.大分大学 医学部 産科婦人科学講座, 2.大分大学 医学部 麻酔科学講座, 3.大分大学 医学部 神経内科学講座)
Introduction: Myotonic dystrophy (DM), an autosomal dominant disorder characterized mainly by myotonia, is a clinically and genetically heterogenous disorder; it sometimes progresses without being noticed by the patient and his/her family.Case: A 42-year-old nulligravid Japanese woman was transferred to our department with acute abdomen and restrictive respiratory failure (30% %VC and 103% FEV1.0). She had a history of laparoscopic myomectomy for fibroids and infertility. Computed tomography showed a 15-cm right ovarian tumor and some atelectasis in both lungs. A blood gas analysis showed respiratory acidosis (pH 7.25, pCO2 68.2, HCO3 28.9 mmol/L). We performed an abdominal right salpingo-oophorectomy for the patient under general anesthesia; it lasted 130 min, with 50-mL blood loss. She was extubated after surgery but re-incubated due to poor oxygenation and moved to the intensive care unit (ICU) for a further work-up for weaning failure. During the ICU stay, weaning was attempted twice but failed both times. The patient underwent a tracheotomy 7 days after the surgery. Consultation with a neurologist suggested possible MD. After genetic counselling, type I MD with a finding of approx. 700-1,100 CTG repeats at DMPK gene was confirmed. She was transferred to a specialty hospital 2 months after the surgery.Conclusion: We treated a patient with undiagnosed MD who showed severe respiratory failure before emergency surgery. Clinicians should be familiar with this disease as a differential diagnosis of post-operative weaning failure.