Achalasia: Case Report of an Unreported Post-treatment Result

Abstract

Background: Achalasia is a primary esophageal disorder affecting the esophageal body and lower esophageal sphincter (LES) whose pathophysiology involves the destruction of the myenteric plexus resulting in aperistalsis. Case: A 30-year-old Latin American woman with history of Hodgkin's lymphoma, status postchemotherapy and radiation, and GERD was referred to evaluate 4 years of progressive retrosternal pain, choking sensation, and regurgitation of food. EGD revealed normal-appearing mucosa without dilation or retained food/fluid; however, did have mild resistance with passage of the endoscope at the LES. Manometry showed esophageal aperistalsis with normal LES pressure, but incomplete relaxation. Physical exam was unremarkable. The patient's oncologist did not believe her symptoms were due to pseudo-achalasia from lymphoma given the lymphoma's aggressive nature and presence of symptoms for 3 years prior to lymphoma diagnosis. Barium swallow revealed a dilated fluid-filled esophagus with distal esophageal tapering and focal narrowing of the LES. Esophageal emptying study demonstrated abnormal emptying of the distal esophagus. Pneumatic dilation with a 30-mm achalasia balloon was performed with complete cessation of dysphagia and near resolution of her retrosternal pain. Unique to our patient is the previously unreported timed esophageal emptying study normalization with only 5% retention at 2.5 minutes and 2.6% retention at 10 minutes. The patient has become and remains symptom free at 9 months. Discussion: As in this patient, achalasia can go undiagnosed for years given its low prevalence, slow progression, and associated vague symptoms. Typical symptoms include dysphagia to solids and liquids, angina like chest pain, and regurgitation of undigested food. Diagnosis is based on symptoms and esophageal manometry demonstrating esophageal aperistalsis and poor LES relaxation. Barium esophagogram demonstrating esophageal dilation and distal tapering can assist diagnosis and may detect pre-clinical symptomatic recurrence. Upper endoscopy is typically normal, but can be utilized to rule out pseudo-achalasia. Treatment is palliative and is aimed at reducing LES pressure and facilitating esophageal emptying. Laparoscopic myotomy and pneumatic balloon dilatation offer definitive sustained benefits, as shown with our patient's esophageal emptying study. Intrasphincteric botulinum toxin injection and medications (calcium channel blockers and nitrates) can offer transient benefits in selected patients.