A 57 year old man with a 30 year history of smoking presents with a difficulty of swallowing solid foods such as steak. This has been going on for 5 months now and it has progressed to where he is having difficulty swallowing liquids. The patient states that he has decreased appetite and a 9 lb weight loss in the past 3 months. The patient denies reflux symptoms, nausea, vomiting, or abdominal pain. Which is the likely cause of his symptoms?
- A. Peptic strictures
- Shatzki ring
- Zenkers diverticulum
- Squamous cell carcinoma
- Diffuse esophageal sphincter
- Hiatal Hernia
- Candida esophagitis
Achalasia: is a rare disease caused by the loss of ganglion cells within the esophageal myenteric plexus. The usual presenting age of the patient is between 25 and 60 years old.
- We know that excitatory (cholinergic) ganglionic neurons are variably affected and inhibitory (nitric oxide) ganglionic neurons are necessarily involved.
- What this means is that these affected ganglionic neurons mediate deglutitive lower esophageal sphincter (LES) relaxation and peristalsis. The absence leads to impaired deglutitive LES relaxation and absent peristalsis.
- There is increasing evidence suggesting that the ultimate cause of ganglion cell degeneration in achalasia is an autoimmune process attributable to latent infection with human herpes simplex virus combined with genetic susceptibility.
Clinical manifestations include: dysphagia, regurgitation, chest, pain and weight loss. Most patients report solid and liquid food dysphagia. Regurgitation occurs when food, fluid, and secretions are retained in the dilated esophagus. Patients with advanced achalasia are at risk for bronchitis, pneumonia, or lung abscess from chronic regurgitation and aspiration (especially those with a history of alcohol abuse (EtOH), or any state of altered mental status. Chest pain is frequent early in the course of achalasia, and is thought to be from esophageal spasm not (DES).
Achalasia is diagnosed with barium swallow x-ray and/or esophageal manometry, endoscopy has a relatively minor role other than ruling out pseudoachalsia. The barium swallow will give the classic “bird’s beak” like appearance due to the tapering of the LES.
Therapy: the only durable therapies for achalasia are pneumatic dilation and Heller myotomy. Pneumatic dilation, with a reported efficacy of 37-98% is an endoscopic technique using non-compliant, cylindrical balloon dilator positioned across the LES and inflated to a diameter of 3-4 cm. The major complication, however, is perforation (with a reported incidence of 1-5%). The most common surgical procedure for achalasia is laparoscopic Heller myotomy.
If left untreated achalasia, esophageal dilation predisposes to stasis esophagitis. Prolonged stasis is likely the explanation for the association between achalasia and esophageal squamous cell cancer.