66 year old male patient presents with history of dysphagia for solids and liquids since 3 months and chest radiograph was normal. Barium esophagogram showing long segment smooth narrowing in the distal esophagus with significant dilatation and hold up of contrast in the proximal esophagus. On noticing carefully there is mild irregularities in the GE junction and cardiac end of stomach. We kept the possibility of achalasia and pseudoachalasia. Advised further workup of CT scan and endoscopy.
Contrast enhanced CT scan with on table oral contrast showing concenteric wall thicekning (arrow) obliterating the lumen in the distal esophagus involving GE junction and having less than 90 degrees of contact with the descending thoracic aorta and abutting left atrium. Endoscopic biopsy revealed adenocarcinoma. These findings are consistent with pseudoachalasia.
Achalasia cardia is due to loss of myenteric ganglion cells in the gastroesophageal junction and the etiology is idiopathic. But a similar clinical picture can be produced by other diseases, a condition termed as secondary or pseudoachalasia. A very high index of suspicion is required for the diagnosis of this condition because this is most commonly produced by a malignancy involving the gastroesophageal junction which is likely to be missed. In true or primary achalasia, there is loss of myenteric ganglion cells with loss of peristalsis in esophageal body and failure of lower esophageal sphincter to relax when swallowing. Barium swallow shows a typical bird's beak appearance.
The causes for pseudoachalasia include primary malignancy of esophagus or gastroesophageal junction, 53.9%, secondary malignancies such as metastasis from lung or breast, 14.9%, benign disorders like mesenchymal tumors, secondary amyloidosis and peripheral neuropathy, 12.6%, and as a postoperative complication following antireflux surgery, 11.9%. Other rare causes are neurological disorders like meningomyelocele, brain metastasis, infiltration by lymphoma and paraneoplastic syndromes associated with small cell carcinoma lung, bronchial carcinoids and pleural mesothelioma.
It is very important to differentiate pseudoachalasia from true achalasia. The patients with pseudoachalasia tend to be older in age ( more than 60 years), have shorter duration of dysphagia (less than 6 months) and have more substantial weight loss. Barium swallow may reveal a nodular of shouldered segment of distal esophageal narrowing. The length of the narrowed esophageal segment was found to be longer than 3.5 cm in 80% of the patients with pseudoachalsia which is the most important feature in radiological differentiation. A CT scan may show asymmetric thickening of the esophageal wall or cardia, mediastinal lymphadenopathy or may identify primary malignancy in secondary achalasia.