失弛缓症是一种罕见的吞咽障碍，是一种可能有严重症状的终身疾病. With proper treatment, symptoms can be managed so they do not disrupt everyday life.

什么是失弛缓症?

失弛缓症是一种罕见的 吞咽障碍 that affects the esophagus (the tube between the throat and the stomach). 在贲门失弛缓症患者中, 食道肌肉不能正常收缩，不能帮助将食物推进胃. 同时, the ring of muscle at the bottom end of the esophagus, called the lower esophageal sphincter (LES), is unable to relax to let the food into the stomach.

失弛缓性 typically affects adults between 30 and 60 years of age, with a peak in the 40s. The disorder is about twice as common in men than women.

失弛缓性原因

The causes of achalasia are unknown, but researchers are exploring several theories. 一种与位于食道肌层之间的神经细胞的退化有关. These nerve cells enable the esophagus to push food toward and into the stomach.

一些研究表明失弛缓症可能与寄生虫或病毒感染有关. People with achalasia may be more likely to show evidence of previous infections, such as antibodies to the herpes simplex virus, 人类乳头状瘤病毒, 麻疹病毒和其他病毒.

There is also evidence that achalasia may be an inflammatory autoimmune disorder, which means it could be caused by the body attacking itself. 研究人员已经在食道肌肉的神经细胞中发现了免疫系统活动的迹象. 此外，贲门失弛缓症患者为3.6 times more likely to have an autoimmune disorder such as uveitis, I型糖尿病, 类风湿性关节炎, systemic lupus erythematosus and Sjögren’s syndrome.

失弛缓性症状

一些贲门失弛缓症的症状是由于食物堆积在食道而无法进入胃. As the esophagus fills up, it widens and can become twisted. 其他贲门失弛缓症症状与食管肌肉的异常收缩有关.

Symptoms of achalasia occur during or after eating. 它们包括:

• 感觉食物或液体难以吞咽，卡在食道或“粘”在胃里(这是贲门失弛缓症最常见的症状)。
• Regurgitation (food and liquid backing up into the mouth after being swallowed)
• Chest pain, which can be severe and awaken the person from sleep
• 胃灼热
• 咳嗽，尤其是在晚上
• Choking,or inhalation of food or liquid into the airways (aspiration)

胸痛和反流, some of the first symptoms a person with achalasia might experience, 也与 胃食管反流障碍(GERD)). 然而, the two conditions are different. GERD is caused by a lower esophageal sphincter that is too loose, allowing stomach acid to enter the esophagus. With achalasia, the sphincter is too tight.

失弛缓症的症状会变得很严重. 严重的失弛缓症可能导致明显的胸痛、疲劳、营养不良和体重减轻. If food particles enter the airways due to coughing and choking while eating, it can lead to pneumonia — inflammation in the airways that can become life threatening.

失弛缓症的类型

The way muscles in the esophagus malfunction in people with achalasia varies. 在所有失弛缓症的病例中, 控制食道和胃之间通道的下食道括约肌没有在适当的时间放松. 根据同时发生的其他问题，医生确定了三种类型的失弛缓症:

• 1型失弛缓症 有时被称为经典贲门失弛缓症. 在这种情况下，食道肌肉几乎没有收缩，所以食物只因为重力就往下移动.
• In 2型失弛缓症, pressure builds up in the esophagus, causing it to become compressed. 这是最常见的失弛缓症类型，它通常会导致比I型更严重的症状.
• III型失弛缓症 有时被称为痉挛性失弛缓症因为食道底部与胃的交界处有不正常的收缩. This is the most severe type of achalasia. 这种收缩会引起胸痛，使人从睡眠中醒来，模仿心脏病发作的症状.

失弛缓性诊断

除了彻底的身体检查和回顾你的病史和症状, your doctor may recommend the following tests to help diagnose achalasia.

• 咽, 食管测压法 测量并记录吞咽时喉咙和食道的压力变化. Some consider manometry to be the most reliable test for achalasia, 因为它可以检测影响吞咽能力的肌肉收缩的位置和严重程度(或缺乏).
• 胃镜检查 用内窥镜检查食道和胃，内窥镜是一种带摄像头的细管，从口腔插入. 该检查有助于发现食道的异常情况，如狭窄和肿瘤. 在内窥镜检查中，你的医生可以进行活检或去除异常组织作进一步分析.
• 无线pH值测试 or 24小时pH阻抗测试 评估食管在一段较长时间内的酸度，并排除其他情况，如胃反流.
• 吞钡, 一种使用造影剂钡和x射线成像上胃肠道的成像检查. This test may reveal a dramatic narrowing of the esophagus, which is sometimes called an achalasia bird beak.

失弛缓性治疗

目前, 没有任何治疗可以完全解决失弛缓症, but there are several approaches to relieve symptoms, 比如膨胀, 手术, botulinum toxin injections and medications. 治疗的目的是使食道的收缩正常化，帮助括约肌放松，使食物进入胃.

食管括约肌扩张

Dilation of a passageway means widening it. 在贲门失弛缓症的扩张过程中, 当胃肠病学家将内窥镜插入口腔和食道时，患者处于睡眠状态, and slowly inflates a balloon near the lower esophageal sphincter. 这可以减少食道的压力，拉伸括约肌，直到它们放松到足以让食物和液体轻松通过.

Balloon dilation is probably the most common approach to treating achalasia. 大多数患者(65% - 80%)报告术后吞咽有明显改善. 然而, the improvement may be temporary — achalasia symptoms can return over time, 呼吁进一步治疗.

贲门失弛缓症手术-食道肌切开术

在肌切开术, a gastroenterologist cuts the muscles in the esophagus, esophageal sphincter and lower stomach to prevent them from tightening.

An achalasia myotomy can be performed through the mouth with an endoscope (经口内窥镜肌切开术) or through several small incisions in the abdomen (laparoscopic Heller myotomy). 下食道括约肌切开术破坏的肌肉足以缓解贲门失弛缓症的症状，但不足以引起胃酸反流. 这是贲门失弛缓症最永久的解决方案，但并非适用于所有患者.

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肉毒杆菌毒素注射

向食管下括约肌注射肉毒杆菌毒素已被证明可以帮助一些贲门失弛缓症患者. 这些治疗对老年患者和那些不适合扩张或肌切开术的患者特别有用.

肉毒杆菌毒素注射治疗贲门失弛缓症通过麻痹括约肌和周围神经来起作用, 让它放松. The injections are done with a special needle inserted through an endoscope. They have a limited effect, which lasts about one year. 可能需要重复注射. Some patients may experience mild chest pain, and there have been reports of skin rashes after this treatment.

治疗失弛缓症的药物

Medications are generally not effective in treating achalasia. 一些含有硝酸盐或钙通道阻滞剂的药物可以帮助暂时放松下食管括约肌. 然而，它们必须在每餐前服用，而且它们的副作用通常大于益处.

与失弛缓症一起生活

If you continue to have symptoms after achalasia treatment, you should work with your doctor to explore other solutions. 调整饮食, eating habits or other lifestyle factors could play a role in your quality of life.

有无失弛缓症饮食法?

No diet is proved to help with achalasia symptoms, 但是你应该和你的医生或语言病理学家讨论一下你是否应该改变你的饮食. 某些食物可能更难或更容易吞咽，这取决于你所患的失弛缓症的类型. 也, if you have lost much weight because of achalasia, 你的医生可能会推荐高热量食物，以确保你有一个健康的身体质量指数.

Some people with achalasia feel better when avoiding certain foods, such as:

• 生水果和蔬菜
• 切得硬的肉
• 高酸性食物，如柑橘类水果
• 酒精
• 咖啡因

How and when you eat can make a difference too. Small bites of food, chewed carefully, can be easier to swallow. Avoiding solid foods before bedtime can minimize nighttime chest pain and regurgitation.

失弛缓症致命吗?

People don’t typically die because of achalasia. Although it tends to be a lifelong condition, 通过症状管理, you can avoid the most serious complications of achalasia, 比如渴望, 窒息和营养不良.

People with achalasia tend to have a higher risk of developing esophageal cancer. 与你的医生讨论，如果你有这种癌症的家族史或其他可能进一步增加你的风险的因素.