Abstract

Abstract Achalasia is a primary motor disorder of the oesophagus. End-stage achalasia is characterised by a grossly dilated sigmoid-shaped oesophagus with food stasis leading to chronic inflammation. Despite treatment, approximately 5% of patients progress to this late stage, needing oesophagectomy. Current guidelines recommend oesophagectomy as the last resort after failure of first-line endoscopic treatment or cardiomyotomy. We review a case of a 70 years old female with end-stage achalasia who successfully underwent cardiomyotomy but required a looser wrap for oesophageal emptying. Patient first presented in her 50s with dysphagia and Oesophago-Gastro-Duodenoscopy (OGD) showed a dilated oesophagus with food stasis and no contractions. Barium swallow confirmed the diagnosis of achalasia. She underwent pneumatic dilatations with minimal symptom improvement and eventually defaulted follow-up. Several years later she presented with haematemesis due to severe oesophagitis and Eckardt score of 7. Both OGD and CT scans showed a grossly dilated megaoesophagus. High-resolution manometry confirmed Type I achalasia with IRP (integrated relaxation pressure) 25.8 and complete absence of peristalsis. She was initiated on nasogastric feeding and was also diagnosed with pulmonary embolism (PE). In view of PE and patient’s comorbidities, she was not fit for oesophagectomy. Peroral endoscopic myotomy (POEM) was also deemed challenging in the presence of severe, chronic oesophagitis and patient’s need for anticoagulation with potential difficulty in endoscopic haemostasis. She underwent laparoscopic cardiomyotomy with anterior 180-degree fundoplication. However, post-operation contrast swallow showed hold up of contrast despite intraoperative OGD showing smooth passage of scope through LES. Decision was made for revision of fundoplication to anterior 90-degree to further promote oesophageal emptying. Post-operation contrast swallow showed satisfactory drainage into stomach and patient tolerated post-fundoplication diet regime. This case illustrates the role of cardiomyotomy as first-line in patients with end-stage achalasia who are not fit for oesophagectomy. However, an anterior 180-degree fundoplication may not allow adequate drainage from a megaoesophagus and a less tight wrap should be considered in such cases. Post-operation, reported Eckardt score of our patient was 1 with no reflux symptoms, indicating marked alleviation of symptoms. Thus, cardiomyotomy with a lesser 90-degree fundoplication is feasible as first-line treatment in end-stage achalasia.

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