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Stomach

Non-neoplastic anomalies

Achalasia of cardia


Reviewers: Elliot Weisenberg, M.D. (see Reviewers page)
Revised: 28 July 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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Note: some authors define achalasia as restricted to the esophagus only
● 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 Allgrove’s 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

Micro description
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● 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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