Vallejo Charles, Gheit Yousra, Nagi Talwinder K, Suarez Zoilo K, Haider Muhammad A
Internal Medicine, Florida Atlantic University, Boca Raton, USA.
Cureus. 2023 Sep 19;15(9):e45567. doi: 10.7759/cureus.45567. eCollection 2023 Sep.
Achalasia is a primary esophageal motility disorder that involves a failure of the lower esophageal sphincter to relax in response to swallowing. Specifically, the lower esophageal sphincter becomes hypertensive, and there is an absence of peristalsis in the esophagus. The pathophysiology is thought to be due to a loss of inhibitory nerve function from an autoimmune attack that targets the esophageal myenteric nerves. As a result, these abnormalities lead to a functional obstruction at the gastroesophageal junction. In severe cases, achalasia may present as a "sigmoid esophagus," a term used to describe the dilation and distortion of the cervical esophagus. In this case report, we discuss a patient with a known history of achalasia who presented with extra-esophageal symptoms including respiratory distress and tracheal compression from an esophagus dilated with a food impaction. She was found to have a sigmoid esophagus and required direct endoscopy and removal of the food bolus. We will review the pathogenesis of achalasia as well as medical and surgical approaches to treating severe achalasia as presented through other case reports.
贲门失弛缓症是一种原发性食管动力障碍性疾病，其特征为食管下括约肌在吞咽时不能正常松弛。具体表现为食管下括约肌压力升高，且食管缺乏蠕动。其病理生理机制被认为是由于自身免疫攻击导致食管肌间神经的抑制性功能丧失。因此，这些异常导致了胃食管交界处的功能性梗阻。在严重情况下，贲门失弛缓症可能表现为“乙状结肠型食管”，该术语用于描述颈段食管的扩张和扭曲。在本病例报告中，我们讨论了一位已知患有贲门失弛缓症的患者，其出现了食管外症状，包括呼吸窘迫以及因食管扩张伴食物嵌塞导致的气管压迫。她被发现患有乙状结肠型食管，需要直接进行内镜检查并取出食物团块。我们将通过其他病例报告回顾贲门失弛缓症的发病机制以及治疗严重贲门失弛缓症的内科和外科方法。
