Achalasia of cardia
Reviewers: Elliot Weisenberg, M.D. (see Reviewers page)
Revised: 2 April 2016, last major update July 2016
Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.
● 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
End of Stomach > Non-neoplastic anomalies > Achalasia of cardia
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