一例9岁的先天性强直性肌营养不良儿童因绞窄性肠梗阻而手术，术中发生严重的低氧血症，急查支气管镜发现左侧主支气管坍陷，术后给予机械通气。因此对于严重的强直性肌营养不良的患者，若需要行上腹部手术，机械通气应该给予准备，直到麻醉肌松作用消失。 另外，强直性肌营养不良与恶性高热相关，全部静脉麻醉，配合以局部阻滞，对于这些患者可能是更可取的麻醉方式。武警总医院神经内科吴士文

 

1: Masui. 2009 Feb;58(2):183-6.

     
    [Anesthetic management of a child with congenital myotonic dystrophy and perioperative hypoxia]
    [Article in Japanese]
     
    Furutani K, Ohkuro M, Komura R, Honma T, Saito N, Baba H.

    Division of Anesthesiology, Niigata University Graduate School of Medical and Dental Sciences, Niigata 951-8510.

    A 7-year-old boy with congenital myotonic dystrophy (MD) and developmental retardation underwent an emergency surgery for strangulation ileus. General anesthesia was maintained using sevoflurane and fentanyl. While intraoperative arterial blood pressure, pulse and rectal temperature remained stable, the arterial blood oxygenation gradually deteriorated during the procedure. We suspected the existence of atelectasis or some other obstructive lung lesion to be the underlying cause, and performed bronchoscopic examination which revealed a collapse of the left main bronchus. Therefore, postoperative mechanical ventilation was continued for several hours in the ICU. According to the postoperative computed tomography, the left main bronchus was sandwiched between the aortic arch and thoracic vertebra. It has been reported that MD patients have a risk of perioperative pulmonary complications, particularly in those who have severe muscular disability undergoing upper abdominal surgery. These risk factors combined with bronchial stenosis could have caused intraoperative hypoxia in our patient. We conclude that when a severe MD patient is scheduled for an upper abdominal surgery, mechanical ventilation should be considered until spontaneous recovery from muscle relaxants occurs. Also, since MD has been related to malignant hyperthermia, total intravenous anesthesia, possibly combined with regional blockade, is a preferable method of anesthesia for such patients.