Esophageal achalasia

Girolamo Mattioli, Alessio Pini Prato, Marco Castagnetti, Vincenzo Jasonni

Research output: Chapter in Book/Report/Conference proceedingChapter


Esophageal achalasia is a functional disorder of the esophagus characterized by abnormal motility of the esophageal body (non-peristaltic waves) associated with incomplete, delayed, or absent mechanical and not neurogenic relaxation of the lower esophageal sphincter (LES). The incidence of esophageal achalasia is about 0.311 per million per year (Podas et al. 1998). Only 5% of the patients suffering from this disease are younger than 15 years of age (Mattioli et al. 1997). The association of childhood achalasia with alacrima and adrenal insufficiency in the triple A or Allgrove syndrome is well known (Ambrosino et al. 1986; Huebner et al. 2000; Verma et al. 1999). Associations with other diseases, such as Alports syndrome, mongolism, Hirschsprungs disease, and megacystis-microcolonhypoperistalsis syndrome have also been described (Al Harbi et al. 1999; Kelly et al. 1997; Leichter et al. 1988; Zarate et al. 1999). Familial cases have been reported (Kasgira et al. 1996; Nihoul-Fket et al. 1991). Esophageal achalasia is usually considered an acquired motility disorder. The onset of symptoms is progressive. Almost all the patients present with dysphagia for fluids, called "paroxysmal dysphagia", retention, and regurgitation of undigested food. Chest pain is described in up to 40% of patients. Pulmonary aspiration, failure to thrive, and halitosis are associated symptoms. Many different forms of treatment have been proposed over the past decades such as calcium entry blockers, pneumatic dilatation, and botulin toxin injection. None of these are satisfactory and surgical treatment seems preferable. In 1914, Heller described a double anterior and posterior myotomy for the treatment of "cardiospasm." Later on, Zaaijer et al. proposed an anterior myotomy alone for the same purpose (Heller 1914). In children these techniques should also be associated with a fundoplication aimed at avoiding postoperative reflux and/or at protecting esophageal mucosa (Mattioli et al. 1997; Patti et al. 2001). The technique described here is a laparoscopic anterior esophageal myotomy with partial anterior fundoplication.

Original languageEnglish
Title of host publicationEndoscopic Surgery in Infants and Children
PublisherSpringer Berlin Heidelberg
Number of pages6
ISBN (Print)9783540001157
Publication statusPublished - 2008

ASJC Scopus subject areas

  • Medicine(all)


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