GI Teaching File

Achalasia

PA and lateral views of the chest demonstrate undulating soft tissue mass along the right side of the mediastinum from above the aortic arch level to the diaphragm. The lateral view shows the trachea bowed and deviated anteriorly.

Etiology--idiopathic, Chaga's disease
Clinical
most cases occur between ages 20 and 40
complaints of dysphagia, which becomes worse during periods of emotional stress or rapid eating
Pathology
obstruction of the distal esophagus with proximal dilitation
incomplete relaxation of the lower esophageal sphincter
failure of normal peristalsis in the smooth muscle portion of esophagus
lack or paucity of ganglion cells in Auerbach's plaxus in distal esophagus
Megaesophagus--dilitation of esophagus beginning at upper 1/3, eventually involving entire length
Carcinoma develops in 7%
Plain film
dilated and tortuous esophagus, with amounts of retained food and fluid
aspiration of material leads to chronic interstitial pulmonary disease
stomach bubble is small or absent
Esophagram
weak nonpropulsive peristaltic waves below level of cricopharyngeus
will persist even after LES has been fixed
incomplete emptying of esophagus even in upright position
"rat tail" or "beak" appearance--gradual smooth tapering of distal esophagus
extends for 1 to 3 cm
Hurst Phenomenon--temporary transit through cardia provoked by hydrostatic pressure of barium column reaching above a critical level

Chaga's Disease
damage of ganglion cells by neurotoxin released from Trypanosoma cruzi (protazoan)
endemic to Central and South America, especially east Brazil
Clinical
intermittent/persistent dysphagia
odynophagia
Peak age 30-50
Organs involved
Heart--dilative cardiomyopathy
Colon--megacolon
Esophagus

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