Achalasia is a rare disease of the esophagus with incidence and prevalence estimated to be 1.63/100,000 &10.82/100,000 respectively. The most common initial presentation is dysphagia, regurgitation and chest discomfort, which can mimic benign conditions leading to delays in diagnosis. An 89 years old female presented with progressive dyspnea for 2 months, which was partially relieved by rest and inhaler use. Associated symptoms were a dry cough, wheezing and chest tightness exacerbated at meal times. She denied odynophagia or dysphagia. The patient is active at baseline. On initial examination, she had tachypnea with bilateral expiratory wheezes. ABG showed hypoxic, hypercapnic respiratory failure and was treated accordingly. Chest x-ray showed a prominent cardiomediastinal silhouette. On chest computed tomography (CT) a torturous, massively dilated esophagus (10.6 cm) was visualized. A barium swallow study showed the classic bird beak appearance. Endoscopy was notable for initial resistance in passing the scope at gastroesophageal junction and 2L of residual food that was evacuated. No masses were found. Achalasia is a neurodegenerative disease that involves the nitric oxide-releasing inhibitory neurons that affect the myenteric plexus and prevents relaxation of the lower esophageal sphincter. It can be divided into primary (idiopathic) and secondary (autoimmune, inflammatory or infectious). Dysphagia for both solids and liquids is the hallmark of achalasia; patients also report regurgitation of undigested food material, difficulty belching, chest pain and odynophagia. Respiratory symptoms have been reported in a single center study, and ranged from a cough, aspiration, hoarseness, wheezing, shortness of breath, and sore throat. In this case, the presentation was atypical as her symptoms were primarily respiratory. The likely mechanism behind respiratory symptoms in achalasia is the mechanical compression of the upper airway by the massively dilated esophagus. Other hypothesis has been suggested. Treatment of achalasia can be divided into surgical and nonsurgical approaches. In this case, options were limited given comorbidities. She underwent botulinum toxin injections as the initial management strategy. Achalasia is a rare disease that presents with non-specific symptoms and can mimic more common conditions. It is a rare but important cause of respiratory presentation. A high suspicion for this condition is important in the differential of chest pain.1708_A Figure 1. Barium swallow at the level of GEJ showing bird beak appearance and absence of peristalsis, consistent with type 1 achalasia.1708_B Figure 2. Chest CT scan without intravenous contrast, axial view, demonstrating a torturous, and massively dilated esophagus (10.6 cm) pressing on the trachea.1708_C Figure 3. Esophagogastroduodenoscopy at the lower third of esophagus showing esophageal dilation and monilial esophagitis.
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Open Access
Oct 1, 2018
A Rimner 
