Department of Neurology, Shodo-kai Southern Tohoku General Hospital, 1-2-5, Satonomori, Iwanuma, Miyagi, Japan.
Department of Neurology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
BMC Neurol. 2022 Mar 16;22(1):94. doi: 10.1186/s12883-022-02619-z.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects motor neurons selectively. In particular, weakness in respiratory and swallowing muscles occasionally causes aspiration pneumonia and choking, which can be lethal. Surgery to prevent aspiration, which separates the trachea and esophagus, can reduce the associated risks. Central-part laryngectomy (CPL) is a relatively minimally invasive surgery to prevent aspiration. No studies have been conducted on the long-term outcomes of surgery to prevent aspiration in patients with ALS. This case series aimed to determine the long-term outcomes of surgery to prevent aspiration and the use of a continuous low-pressure aspirator in patients with ALS by evaluating the frequency of intratracheal sputum suctions performed per day, intra- and postoperative complications, oral intake data, and satisfaction of patients and their primary caregiver to predict improvement in patients' quality of life (QOL).
We report a case series of six patients with ALS who underwent CPL along with tracheostomy to prevent aspiration between January 2015 and November 2018. We evaluated their pre- and postoperative status and administered questionnaires at the time of last admission to the patients and their primary caregivers.
The mean follow-up period after CPL was 33.5 months. Aerophagia was a common postoperative complication. The use of a continuous low-pressure aspirator resulted in reduced frequency of intratracheal sputum suctions. All cases avoided aspiration pneumonia. Oral intake was continued for 2-4 years after the tracheostomy and CPL. The satisfaction levels of the patient and primary caregiver were high.
Our case series suggests that the use of a continuous low-pressure aspirator in patients undergoing CPL improves oral intake and reduces the frequency of intratracheal sputum suctions, which improves the QOL of patients with ALS and their families and caregivers. CPL and continuous low-pressure aspiration should be considered as a management option for ALS with significant bulbar and respiratory muscle weakness/dysfunction.
肌萎缩侧索硬化症（ALS）是一种选择性影响运动神经元的进行性神经退行性疾病。特别是呼吸和吞咽肌肉的无力偶尔会导致吸入性肺炎和窒息，这可能是致命的。为了预防因气管和食管分离而导致的吸入，可进行手术。中央型喉切除术（CPL）是一种相对微创的预防吸入术。目前尚无研究报道 ALS 患者预防吸入手术的长期结果。本病例系列研究旨在通过评估每天进行的气管内痰液抽吸次数、手术相关并发症、口腔摄入数据以及患者及其主要照顾者对手术的满意度，来确定预防吸入手术和使用持续低压抽吸器治疗 ALS 患者的长期效果，以预测患者生活质量（QOL）的改善。
我们报告了 2015 年 1 月至 2018 年 11 月期间进行 CPL 联合气管切开术预防吸入的 6 例 ALS 患者的病例系列研究。我们评估了他们的术前和术后状态，并在患者及其主要照顾者最后一次入院时进行了问卷调查。
CPL 后平均随访时间为 33.5 个月。术后气腹是一种常见的并发症。使用持续低压抽吸器可减少气管内痰液抽吸的频率。所有病例均避免了吸入性肺炎。气管切开术和 CPL 后，患者继续口服摄入 2-4 年。患者和主要照顾者的满意度水平均较高。
我们的病例系列研究表明，CPL 术后使用持续低压抽吸器可改善口腔摄入，减少气管内痰液抽吸次数，从而提高 ALS 患者及其家庭和照顾者的生活质量。对于存在明显延髓和呼吸肌无力/功能障碍的 ALS 患者，CPL 和持续低压抽吸术应作为一种治疗选择。
