Reichert T J, Bluestone C D, Stool S E, Sieber W K, Sieber A M
Ann Otol Rhinol Laryngol. 1977 Sep-Oct;86(5 Pt 1):603-10. doi: 10.1177/000348947708600507.
Fifteen infants with congenital cricopharyngeal achalasia are reviewed. Although most of the patients had symptoms at birth, the diagnosis was frequently not confirmed until later in the first year of life. While four of the infants had only cricopharyngeal achalasia, 11 had associated diseases related to the central nervous system. Those patients without associated diseases improved spontaneously with conservative management; most of the infants with other abnormalities also improved, although their clinical progress was slower and more complicated. In three of the patients, the symptoms were persistent and there were two deaths related to associated diseases. Cricopharyngeal myotomy was performed on two children with only moderate improvement in symptoms. Congenital cricopharyngeal achalasia is more common than formerly recognized. When suspected, an esophagram with tele- or cineradiography is essential to confirm the diagnosis. Esophageal motility studies will quantify changes and also evaluate lower esophageal dysfunction not easily identified on esophagrams.
对15例先天性环咽肌失弛缓症婴儿进行了回顾性研究。尽管大多数患者在出生时就有症状，但诊断往往直到出生后第一年晚些时候才得到证实。15例婴儿中4例仅有环咽肌失弛缓症，11例伴有中枢神经系统相关疾病。那些无相关疾病的患者通过保守治疗自发改善；大多数有其他异常的婴儿也有改善，尽管其临床进展较慢且更复杂。3例患者症状持续存在，有2例死于相关疾病。对2例儿童进行了环咽肌切开术，症状仅稍有改善。先天性环咽肌失弛缓症比以前认为的更常见。当怀疑该病时，食管造影（透视或电影造影）对于确诊至关重要。食管动力研究将量化变化，还可评估食管造影不易发现的食管下段功能障碍。
