ANSWER

Achalasia cardia: In 1672, Sir Thomas Willis first described this condition, which was further
characterized in 1881; the term achalasia cardia was coined in 1929 to signify a disorder of esophageal
motility characterized by decreased or absent peristalsis of the esophageal body, increased pressure in
the esophagus, and impaired relaxation of the lower esophageal sphincter (LES). The result is impaired
esophageal emptying with a resistance to antegrade flow that causes the esophagus to dilate and
elongate.

The condition can be divided into primary and secondary forms. Primary achalasia is caused by a loss of
ganglion cells in the myenteric plexus of Auerbach, and it is more common than secondary achalasia,
which may be caused by malignancy, diabetes, or Chagas disease (a Trypanosoma cruzi infection
acquired by the bite of an infected reduviid bug). The most common age range at presentation is 25-60
years, with no predilection for sex. Patients with this condition have an increased incidence of
malignancy; approximately 5% of patients develop squamous cell carcinoma, usually in the mid-
esophagus.

Dysphagia is present in more than 90% of patients with achalasia. Other symptoms include regurgitation,
weight loss, and chest pain or discomfort. Recurrent aspiration of secretions and food material leads to
pneumonia. The initial examination may be chest radiography, which often demonstrates homogeneous
paramediastinal soft-tissue opacity, usually on the right side. Other findings may include mediastinal
widening, air-fluid levels, absence of a gastric air bubble (due to a water-seal effect), and complications
such as aspiration pneumonia or lung abscess (patients with achalasia may have chronic aspiration
pneumonia involving Mycobacterium fortuitum-chelonae). A fluoroscopic barium swallow demonstrates
failure of the contrast agent to enter the stomach when the patient is recumbent; nonpropulsive tertiary
esophageal contractions; various degrees of dilatation; and the bird-beak sign (ie, abrupt, smooth
tapering of the distal esophagus). CT findings are nonspecific and insensitive, with esophageal dilatation
usually present. Symmetric wall thickening helps to distinguish achalasia from pseudoachalasia caused
by malignancy, in which mucosal irregularities or mass effect at the cardia are usually present.

Esophageal manometry is considered the diagnostic criterion standard. The findings include increased
LES pressure and incomplete relaxation, as well as abnormal esophageal peristalsis. Endoscopy can
yield biopsy samples to exclude malignancy and can permit direct visualization of esophagitis or ulcers.
In addition, therapeutic interventions, including pneumatic balloon dilation (which is 70% effective, with a
5% perforation rate) and botulinum toxin injection (although only 30% of patients have continued relief at
1-year follow-up), may be performed during the procedure. Laparoscopic esophagocardiomyotomy
usually results in progressive improvement, and it compares favorably with open surgery with regard to
relief of dysphagia and GERD. Various drugs, such as calcium channel blockers and nitrates, are
effective in a few patients, and they may be tried in patients with contraindications to pneumatic dilation
or surgery.

The patient in this case underwent a laparoscopic esophagocardiomyotomy, which had to be converted
to an open procedure. During the procedure, the patient also underwent a partial fundoplication to
prevent postprocedural reflux. A follow-up barium esophagogram demonstrated substantially improved
flow of the contrast material.

For more information on achalasia cardia, please visit the eMedicine article Achalasia in the Radiology
section.

References:

1. Dahnert W. Radiology Review Manual. 3rd ed. Philadelphia, Pa: Lippincott, Williams & Wilkins;
1996.
2. Eisenberg R. Gastrointestinal Radiology: A Pattern Approach. 4th ed. Philadelphia, Pa:
Lippincott, Williams & Wilkins; 2002.

BACKGROUND
A 37-year-old man presents with a 9-month history of food sticking in his throat. He states that he has
had pain behind his sternum when he swallows and has halitosis. The patient has more difficulty
swallowing solid food than liquids, and he has lost 30 lb in the past year. He has no history of fevers or
chills, and he denies any trauma, nausea, or vomiting. He is currently taking a proton-pump inhibitor for
severe gastroesophageal reflux disease (GERD) that another physician prescribed to him several months
ago, when his symptoms first began; this medication offers some symptomatic relief. He reports having at
least 1 episode of pneumonia in the past.

On physical examination, the patient appears thin but not emaciated. His vital signs, including
temperature, are normal. A conjunctival pallor is noted. Findings from the cardiac and respiratory portions
of the examination are unremarkable. He has a soft, nondistended abdomen with normal bowel sounds.
The remainder of the examination yields unremarkable results.

A single-contrast, barium upper-gastrointestinal radiograph series (see Images 1-2) and a contrast-
enhanced computed tomography (CT) scan of the chest (see Image 3) are performed.

What is the diagnosis?

CASE DIAGNOSIS
What is the diagnosis?
Click here for the answer
HINT
This is an uncommon cause of dysphagia.
Author:
Gautam Dehadrai, MD,
Staff Physician,
Department of Radiology,
Veterans Administration Medical Center,
Albuquerque, NM

eMedicine Editor:
Rick G. Kulkarni, MD,
Assistant Professor,,
Yale School of Medicine,
Section of Emergency Medicine,
Department of Surgery,
Attending Physician,
Medical Director,
Department of Emergency Services,
Yale-New Haven Hospital, Conn