1706 Achalasia With Megaesophagus Presenting as Septic Shock

Abstract

INTRODUCTION: Achalasia has wide range presentations including gastrointestinal and respiratory symptoms. Septic shock is less common. CASE DESCRIPTION/METHODS: We report a case of a 59-year-old Caucasian woman who presented with 1-month history of dyspnea and cough worsening for one week. She reported intermittent vomiting, dysphagia and unintentional weight loss for the past 2 months. Upon admission, she was hypoxemic and developed septic shock. Computer tomography showed multifocal pneumonia and severe distension of the esophagus filled with debris (Figure 1) measuring 9.5 × 6 cm at the largest diameter. She was intubated and given board spectrum antibiotics and then transferred to the medical intensive care unit. Upper endoscopy demonstrated dilation in the entire esophagus filled with food and scattered whitish esophageal plaques, consistent with esophageal candidiasis. Four weeks of oral fluconazole was initiated to treat esophageal candidiasis. Propionibacterium species grew in one out of two blood cultures but the respiratory culture was negative. She required vasopressors for two days and then successfully extubated two days later. She underwent a barium esophagography showing dilated esophagus with distal narrowing “bird beak’s” appearance (Figure 2). Esophageal manometry confirmed type I achalasia demonstrating apersistalsis of the entire esophagus and hypertensive lower esophageal sphincter (Figure 3). She recovered from aspiration pneumonia and weaned off oxygen. She was scheduled to come back in 2 weeks for perioral endoscopic myotomy evaluation and was discharged home with oral fluconazole. DISCUSSION: Achalasia is an esophageal disease caused by loss of relaxation in the distal esophagus and lower esophageal sphincter. Most common presentations are dysphagia (90%), heartburn (75%) and regurgitation or vomiting (45%). Respiratory symptoms including cough or chronic aspiration account for 20-40%. However, septic shock as initial presentation is not common. Our patient had chronic aspiration to the lungs and developed aspiration pneumonia progressing to septic shock. Patients presenting with respiratory symptoms are reported to have a more dilated esophagus with an average mean diameter of 4.3 ± 2 cm. Our case has an esophagus double the average size. Perioral endoscopic myotomy has gained universal acceptance to treat achalasia and shows safety and efficacy.