Pseudoachalasia: A peculiar case report and review of the literature

Abstract

Pseudoachalasia is a rare secondary achalasia, which accounts for only a small subgroup of patients. We describe a 77-year-old woman with recent onset of dysphagia and typical esophageal manometric findings of achalasia. Moreover, esophageal manometric findings of vascular compression at 36 cm from the nose were associated with dysphagia. An upper endoscopy showed the absence of lesions both in the esophagus and gastro-esophageal junction, whilst a 15-mm ulcer on the gastric angulus was detected. The gastric ulcer resulted in being a diffuse signet ring cell carcinoma at histology, suggesting pseudoachalasia. An abdominal computed tomography scan showed an irregular concentric thickening of the gastro-esophageal junction wall extending for 7 cm and a dilated ascending thoracic aorta with no presence of the inferior vena cava, with an enlarged azygos as the source of vascular compression of esophagus. Moreover, cardia involvement from diffuse signet ring cell carcinoma of the gastric angulus was also recognized as the cause of dysphagia. The cancer was not suitable for a surgical approach in an old patient with cardiovascular comorbidities and support therapy was started. In our ambulatory series, pseudoachalasia was eventually diagnosed in 4.7% of 234 consecutive patients with esophageal manometric finding suggestive of achalasia. We also reviewed cases in the literature and aimed to evaluate the reported causes of pseudoachalasia.

Keywords: Pseudoachalasia; Achalasia; Esophageal vascular compression; Thoracic aorta; Azygos vein

Core tip: Typical esophageal dysmotility can be observed in pseudoachalasia, a secondary form of achalasia mostly due to cancer or even benign tumors, postoperative complications or paraneoplastic syndromes. Dysphagia is frequently observed in subjects with pseudoachalasia. We describe a peculiar case where dysphagia could be due to a vascular compression of the esophagus rather than involvement of the esophagus at the gastro-esophageal junction from gastric neoplasia. The less invasive therapeutic option should be proposed in an old patient. The reviews of our cases of pseudoachalasia and the literature are included.