Achalasia

Patients with achalasia have an increased risk of esophageal cancer, but the absolute risk is low. In a study in which 448 cases were followed for a median of 9.6 years, esophageal cancer developed in only 15 of them (3.3%) after a mean symptom duration of 13 years. Esophageal cancer secondary to achalasia is typically squamous cell carcinoma (SCC), although some studies have also demonstrated an increased risk of adenocarcinoma. Lower esophageal sphincter (LES) pressure is coordinated with both inhibitory (e.g., vasoactive intestinal peptide, nitric oxide) and excitatory (e.g., substance P, acetylcholine) neurotransmitters. There is progressive degeneration of inhibitory ganglion cells in the myenteric plexus of patients with achalasia. Histopathological examination typically reveals reduced numbers of ganglion cells in the myenteric plexuses, and the remaining ganglion cells are often surrounded by lymphocytes and, less prominently, by eosinophils. This situation leads to an imbalance between inhibitory and excitatory neurotransmitters. The result of this imbalance is a hypertensive LES that cannot relax. The classic symptom triad of achalasia is dysphagia, regurgitation, and weight loss. Physical examination is usually noncontributory in achalasia. Although it can be diagnosed with a conventional standard manometer, a high-resolution manometer should be preferred. Recent studies have confirmed that high-resolution manometry (HRM) is superior to standard manometry and that it has a higher sensitivity to diagnose achalasia. Today, the gold-standard method for esophageal motility dysfunction diagnosis is HRM. In achalasia, the primary goal of the current therapies is to eliminate the outflow resistance of the LES. Once the obstruction is relieved, the food bolus can pass through the LES with the contribution of gravity. When deciding on the initial treatment for achalasia, patient’s age, comorbidities, expectations, preferences, and local institutional expertise should be considered. Graded pneumatic dilation (PD) or laparoscopic myotomy is recommended as the initial therapy, if the patient is fit for surgery.