A 29-year-old lady presented with dysphagia since two years. The dysphagia was more for solids than liquids and was progressively increasing. There was no history of vomiting, weight loss or fever.

RADIOLOGICAL FINDINGS:

A plain radiograph of the chest showed a soft tissue opacity in the right paravertebral region with an air-fluid level in the upper dorsal region This suggested a dilated esophagus(Fig 1).

Fig. 1

A CT scan of the chest confirmed this. There was narrowing at the lower end of the esophagus. No soft tissue mass was seen in the region of the narrowing (Figs 2,3,4).

Fig. 2	Fig. 3	Fig. 4 .

A barium esophagogram a showed smooth narrowing at the gastroesophageal junction extending for a distance of about 2 cms. with moderate dilatation of the proximal esophagus. A bird-beak appearance is seen at the distal portion of the esophagus with no evidence of shouldering. The esophageal mucosal pattern was normal. It showed no intrinsic or extrinsic filling defects or mass effect. Tertiary contractions were seen in the proximal esophagus. There was no evidence of hiatal hernia or gastro-esophageal reflux.

Fig. 5	Fig. 6
Fig. 7	Fig. 8

The patient was diagnosed to have achalasia cardia.

DISCUSSION:

Dysphagia is the most common presenting symptom in patients with achalasia. The ingestion of either solids or liquids can result in dysphagia, though dysphagia for solids is more common. The natural history varies. Some patients notice that the dysphagia reaches a certain point of severity and then stops progressing. In others, the dysphagia continues to worsen, resulting in decreased oral intake and malnutrition. Therefore, weight loss is included in the complex of signs and symptoms associated with achalasia, and it is usually a sign of advanced esophageal disease.

Some of patients with dysphagia complain of episodes of chest pain which are frequently induced by eating. Typically, chest pain is described as being retrosternal; this is a more common feature in patients with early or so-called vigorous achalasia. As the disease progresses and as the esophageal musculature fails, chest pain tends to abate or disappear. Some of patients with dysphagia complain of episodes of chest pain which are frequently induced by eating. Typically, chest pain is described as being retrosternal; this is a more common feature in patients with early or so-called vigorous achalasia. As the disease progresses and as the esophageal musculature fails, chest pain tends to abate or disappear.

Many of patients with achalasia experience spontaneous regurgitation of undigested food from the esophagus during the course of the disease. Some learn to induce regurgitation to relieve the retrosternal discomfort related to the distended esophagus.

As the disease progresses the likelihood that aspiration will occur increases. As a result, some patients may present with signs or symptoms of pneumonia or pneumonitis. Lung abscesses, bronchiectasis, and hemoptysis are some of the more severe pulmonary consequences of achalasia-associated aspiration.

Pathophysiology:

The exact etiology of achalasia is not known. The most widely accepted current theories implicate autoimmune disorders, infectious diseases, or both. The last decade has witnessed much progress in the understanding of the cellular and molecular derangements in achalasia. Degeneration of the esophageal myenteric plexus of Auerbach is the primary histologic finding.

Radiologcial Studies -
Plain radiograph -

Findings:

Plain chest radiographs occasionally offer clues in the diagnosis of achalasia. A double mediastinal stripe is occasionally depicted. An air-fluid level can be seen in the esophagus; this is frequently retrocardiac. Owing to the paucity of air progressing through the hypertensive LES, the gastric air bubble may be small or absent.

Barium Swallow-

Features of achalasia depicted at barium study under fluoroscopic guidance include the following:

CT scan -
CT scanning with oral contrast enhancement may demonstrate the gross structural esophageal abnormalities associated with achalasia, especially dilatation, which is seen in advanced stages. However, CT findings are nonspecific, and the diagnosis of achalasia cannot be made using CT alone. CT scan may be indicated in the workup of patients with suspected pseudoachalasia.