Delayed discovery and diagnosis of achalasia resulting in megaesophagus in an elderly nursing home resident.

Abstract

To the Editor: An 87-year-old woman with a past history of osteoarthritis and cardiomegaly who denied any systemic chronic disease was referred to the emergency department from a nursing home where she had been living for 7 years. She had chief complaints of chest tightness, abdominal pain, nausea, and vomiting (with watery vomitus) for 1 day; increased drooling for 2 weeks; and poor appetite and body weight loss of approximately 5 kg for 1 month. Physical examination demonstrated epigastric tenderness. Her supine chest radiograph showed a suspected dilated esophagus at the neck and superior mediastinum and opacities at the bilateral lower lobes of the lungs (Figure 1A). Computed tomography (CT) showed marked distention of the esophagus, with air and fluid retention due to chronic lower esophageal obstruction and highly suspected achalasia or esophagocardiac junctional cancer (Figure 1B,C). Ground-glass infiltrates in the bilateral lower lobes were consistent with aspiration pneumonia. After admission, a gastroenterologist confirmed cardiac achalasia and dilated esophagus without peristalsis (megaesophagus) using endoscopy. Because of her age, he performed endoscopic balloon dilation of the esophageal cardiac portion instead of surgical intervention, which led to significant improvement in symptoms. Achalasia, a primary esophageal motor disorder secondary to the degeneration of ganglion cells of the inhibitory intramural myenteric plexus of unknown etiology, involves hypertension and poor relaxation of the body of the esophagus and the lower esophageal sphincter, affects both sexes and all ages, and has two peaks of incidence, one in the third to fourth decades of life and the other after 60 years of age.1-6 Because of the slow progression of its symptoms and low incidence (~1–2 per 100,000 per year), achalasia may remain undiagnosed for years.1, 7, 8 The effect of age on esophageal motility of individuals with achalasia is not well known. Pathophysiology of achalasia is considered to be an impairment of inhibitory neurons mainly releasing nitric oxide and vasoactive intestinal peptide (VIP) neurotransmitters and regulating the inhibitory arch of the peristaltic reflex and lower esophageal sphincter relaxation.8 VIPs present in myenteric plexus act as inflammatory cytokines and affect smooth muscle relaxation. In achalasia, the earliest pathological change is a myenteric inflammation, causing limited damage of the myenteric plexus and then ganglion loss and neural fibrosis.8 A study showed that individuals with late onset of idiopathic achalasia presented with a significant difference in allele, genotype, and phenotype distribution of SNP rs434876 mapping in atron 4 in the human VIP receptor 1 gene (VIPR1) from that of randomized controls from the same geographic area.8 Achalasia is suggested based on clinical manifestations and findings in radiological studies (barium esophagography and CT), which should be confirmed using manometry and endoscopy.1, 2, 6 Typical clinical presentations of untreated achalasia include dysphagia, regurgitation, heartburn, chest pain, and weight loss, in descending order.2, 4 Older adults with idiopathic esophageal achalasia have similar clinical and manometric presentations but less-severe chest pain than younger individuals.1 Achalasia of older adults is significantly associated with greater risks of pulmonary complications, malnutrition, and gastroesophageal cancer and higher prevalence of Chagas disease in South American countries.1, 3 Owing to chronic microaspiration, individuals with achalasia may have respiratory symptoms of nocturnal cough, poor pulmonary function in the form of restrictive or obstructive airway disease, and abnormal findings on high-resolution chest CT, including tracheobronchial compression by the dilated esophagus, pulmonary nodules, ground-glass opacities, fibrosis, consolidations, air-trapping, and bronchiectasis.4, 5 Tumors associated with secondary achalasia (pseudoachalasia) include gastroesophageal cancer, pancreatic cancer, lung cancer, breast cancer, mesothelioma, hepatocellular carcinoma, sarcoma, and lymphoma, which should be excluded before a diagnosis of idiopathic achalasia is made.1, 5, 7 Megaesophagus, also termed sigmoid esophagus, results from peristaltic disorders and slow decompensation of the esophageal muscular layer at the end stage of untreated cardiac achalasia.9, 10 The characteristics of megaesophagus are lack of peristalsis of the esophagus, low intraluminal pressure and lack of response to stimulation, breakdown of the axis of the esophagus, and classification of Los Angeles C/D esophagitis.10 In addition, dysphagia decreases because the esophagus stretches, but chest pain or discomfort occurs because of greater acid regurgitation, and most individuals lose weight. We remind healthcare providers that megaesophagus can be avoided in elderly adults if achalasia is disclosed, diagnosed, and treated appropriately at an early stage.9, 10 Treatment options include medical methods (e.g., botulinum toxin injection at the lower esophageal sphincter), pneumatic dilatation and surgery (e.g., myotomy with fundoplication, jejunum esophagogastric bypass, laparoscopic Heller myotomy).10, 11 Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors and has determined that the authors have no financial or any other kind of personal conflicts with this paper. Author Contributions: Cheung: concept and design, acquisition of subjects and data, preparation of manuscript. Cheung, Ho, Chou: analysis and interpretation of data, critical review and approval. Sponsor's Role: None.