1816 Achalasia in the Setting of Esophageal Varices Achalasia in the Setting of Achalasia in the Setting of Esophageal Varices

Abstract

INTRODUCTION: Primary achalasia is an esophageal motility disorder characterized by inadequate or absent lower esophageal sphincter (LES) relaxation and loss of normal esophageal peristalsis of the esophageal body. We present an unusual case of achalasia in the context of esophageal varices, creating a challenging therapeutic dilemma. CASE DESCRIPTION/METHODS: A 63-year-old male with a history of hypertension, type II diabetes, decompensated HCV cirrhosis (status post sofosbuvir/velpatasvir and ribavirin without sustained virologic response) with hepatic encephalopathy, initially presented with a five month history of progressively worsening dysphagia, unintentional 40 lb. weight loss, and epigastric abdominal pain. He underwent an EGD with findings highly suspicious of achalasia with dilated esophagus, a large amount of retained food with associated stasis esophagitis, hypertonic LES, and no obvious esophageal varices. Esophageal manometry confirmed absence of normal contractility and elevated integrated relaxation pressure (29 mm Hg) consistent with Type I achalasia. On subsequent EGD, he was noted to have grade II esophageal varices. Treatment of achalasia was performed with botulinum toxin injections of the LES, carefully avoiding the esophageal varices. In the next 48 hours, he had significant improvement in symptoms of dysphagia, tolerated a full liquid diet, and then underwent a repeat EGD with successful decompression of his varices with placement of two bands. He was discharged from the hospital tolerating a regular diet. DISCUSSION: Achalasia is uncommon, with an annual incidence of approximately 1 in 100,000 individuals. Achalasia in combination with esophageal varices is a very rare occurrence with only 12 case reports and one retrospective study of 14 patients in the current published literature. This particular disease combination presents a therapeutic challenge as our patient was a poor candidate for surgical myotomy given his cirrhosis and bleeding risk. Our case is consistent with previously documented reports showing short-term clinical improvement with botulinum toxin injections as a well-tolerated first line therapy.