28 year old presents complaining of dysphagia to both solids and
liquids that has been worsening over the period of several months.
Oblique image from a barium swallow examination, as well as lateral
image of the chest obtained following the procedure. Images
demonstrated dilatation of virtually the entirety of the esophagus.
In the area of the GE junction, there is a rather smoothly tapering
stricturing, resembling a bird's beak. The mucosa appears to be
intact on these images.
Primary achalasia is a motility disorder thought to be related to a
decrease in ganglion cells within the distal 2/3 of the esophagus.
This, combined with failure of relaxation of the lower esophageal
sphincter, results in the appearance of achalasia. Symptoms in
patients with primary achalasia are generally due to dysphagia with
both solids and liquids, which tend to be slowly progressive over a
long period of time. Differentiation must be made with secondary
achalasia, due to an obstructing mass. In these patients, a history
can often be obtained of initial dysphagia to solid foods, followed
by difficulty swallowing liquids. Fluoroscopically, barium swallow
is utilized to demonstrate achalasia. This demonstrates a dilated,
at times tortuous appearance to the esophagus with absence of
peristalsis in the lower esophagus. Disordered, tertiary
contractions can be visualized within the mid esophagus. The
smoothly tapered narrowing at the distal esophagus is
characteristic of primary achalasia. This can often be
differentiated from secondary achalasia, which demonstrates mucosal
irregularity. Differentiation may also be possible in terms of age
(carcinoma most commonly age >50) and in terms of the duration
of patient's symptoms (tends to be less than 6 months in secondary
achalasia). Other entities in the differential would be peptic
stricture, scleroderma with a reflux stricture and Chagas disease.
Provenzale, Nelson, Duke Radiology Case Review. Lippincott-Raven,
Philadelphia, 1998. Eisenberg, Gastrointestinal Radiology
Companion. Lippincott Williams & Wilkins, Baltimore, 1999