PDF(Pubmed)
Abstract：
BACKGROUND: Myotonic dystrophy type 1 (DM-1) is a progressive multisystem genetic disorder that causes myotonia and both distal limb and facial/neck muscle weakness by expanding the CTG repeats of the DMPK gene in chromosome 19q13.3. General anesthesia is indicated in DM-1 patients owing to their sensitivity to anesthetic drugs such as opioids, hypnotics, and neuromuscular blocking agents.
METHODS: A 48-year-old male patient underwent a laparoscopic cholecystectomy for gallstones under general anesthesia. He experienced sudden cardiac arrest and respiratory failure the day after surgery. After a thorough review of past medical history, we recognized that 15 years prior, he had been diagnosed with classic type DM-1, but the diagnosis was not self-reported before general anesthesia. Symptoms of severe dysphagia developed subsequently. In a videofluoroscopic swallowing study (VFSS), we observed abrupt aggravation of myotonic dysphagia after general anesthesia. VFSS revealed cricopharyngeal opening dysfunction, with a remaining large residue in the pyriform sinus, resulting in a severe cricopharyngeal achalasia pattern.
METHODS: Acute cricopharyngeal achalasia after general anesthesia.
RESULTS: The patient underwent a dysphagia rehabilitation program that included cricopharyngeal opening exercises and functional electrical stimulation. However, no significant improvement was observed in the cricopharyngeal achalasia in a 3-month follow-up VFSS.
CONCLUSIONS: Low body temperature and anesthetic medications such as opioids and hypnotic agents can induce myotonia in the cricopharyngeal muscle.
METHODS: A 48-year-old male patient underwent a laparoscopic cholecystectomy for gallstones under general anesthesia. He experienced sudden cardiac arrest and respiratory failure the day after surgery. After a thorough review of past medical history, we recognized that 15 years prior, he had been diagnosed with classic type DM-1, but the diagnosis was not self-reported before general anesthesia. Symptoms of severe dysphagia developed subsequently. In a videofluoroscopic swallowing study (VFSS), we observed abrupt aggravation of myotonic dysphagia after general anesthesia. VFSS revealed cricopharyngeal opening dysfunction, with a remaining large residue in the pyriform sinus, resulting in a severe cricopharyngeal achalasia pattern.
METHODS: Acute cricopharyngeal achalasia after general anesthesia.
RESULTS: The patient underwent a dysphagia rehabilitation program that included cricopharyngeal opening exercises and functional electrical stimulation. However, no significant improvement was observed in the cricopharyngeal achalasia in a 3-month follow-up VFSS.
CONCLUSIONS: Low body temperature and anesthetic medications such as opioids and hypnotic agents can induce myotonia in the cricopharyngeal muscle.
摘要：
背景：强直性肌营养不良1型（DM-1）是一种进行性多系统遗传疾病，通过扩大染色体19q13.3中DMPK基因的CTG重复序列，导致肌强直和远端肢体和面部/颈部肌肉无力。由于DM-1患者对阿片类药物等麻醉药物的敏感性，因此需要进行全身麻醉。催眠药,和神经肌肉阻断剂.
方法：一名48岁男性患者在全身麻醉下接受腹腔镜胆囊切除术治疗胆结石。手术后的第二天，他经历了突然的心脏骤停和呼吸衰竭。在彻底回顾了过去的病史后，我们认识到15年前，他被诊断为经典型DM-1,但在全身麻醉前没有自我报告诊断.随后出现严重吞咽困难的症状。在视频透视吞咽研究(VFSS)中，我们观察到全身麻醉后肌强直性吞咽困难的突然加重。VFSS显示环咽开放功能障碍,在梨状窦中残留大量残留物，导致严重的环咽失弛缓症。
方法：全身麻醉后急性环咽失弛缓症。
结果：患者接受了吞咽困难康复计划，包括环咽开放训练和功能性电刺激。然而,在3个月的随访VFSS中未观察到环咽肌失弛缓症的显着改善。
结论：低体温和麻醉药物如阿片类药物和催眠药可诱导喉部肌强直。
方法：一名48岁男性患者在全身麻醉下接受腹腔镜胆囊切除术治疗胆结石。手术后的第二天，他经历了突然的心脏骤停和呼吸衰竭。在彻底回顾了过去的病史后，我们认识到15年前，他被诊断为经典型DM-1,但在全身麻醉前没有自我报告诊断.随后出现严重吞咽困难的症状。在视频透视吞咽研究(VFSS)中，我们观察到全身麻醉后肌强直性吞咽困难的突然加重。VFSS显示环咽开放功能障碍,在梨状窦中残留大量残留物，导致严重的环咽失弛缓症。
方法：全身麻醉后急性环咽失弛缓症。
结果：患者接受了吞咽困难康复计划，包括环咽开放训练和功能性电刺激。然而,在3个月的随访VFSS中未观察到环咽肌失弛缓症的显着改善。
结论：低体温和麻醉药物如阿片类药物和催眠药可诱导喉部肌强直。
