Cite this page: Achalasia of cardia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/stomachachalasia.html. Accessed January 22nd, 2017.
Definition / General
- Controversial diagnosis: some authors do not consider this diagnosis distinct from achalasia of esophagus
- Rare disorder of children
- Due to defective relaxation of the cardia and absence of peristalsis in esophageal body
- Associated with lack of nitrous oxide nerve fibers and with lymphocytes infiltrating myenteric plexus
- May occur as part of Allgroves syndrome (triple A syndrome, OMIM 231550, Am J Surg Pathol 2003;27:667), an autosomal recessive disorder which features achalasia, Addisonianism (ACTH insensitivity), and alacrima (lack of tears), and may have late-onset progressive neurologic symptoms; may be due to mutations of Aladin (Adracalin) gene at 12q13
- Fibrosis between circular and longitudinal muscles, reduction in myenteric ganglia and myenteric neurons