S1973 Achalasia With Diffuse Esophageal Dilation Causing Tracheal Compression and Severe Asthma

Abstract

INTRODUCTION: Achalasia is a rare esophageal disorder that is caused by the lack of relaxation of the lower esophageal sphincter, causing loss of peristalsis and a dilated esophagus. Patients present with various upper gastrointestinal complaints. Here, we present the case of a 16-year-old female with history of uncontrolled asthma, including intubation at age 14 for status asthmaticus, who was found to have a massively dilated esophagus causing compression of the trachea. Peroral endoscopic myotomy (POEM) was performed and she experienced complete regression of her asthma symptoms. CASE DESCRIPTION/METHODS: 16-year-old female with history of uncontrolled asthma presents to pulmonology clinic. Pulmonary function testing (PFT) demonstrates severe obstruction (FEV1 33%, FEV1/FVC 45%) with little improvement following a 5-day course of oral steroids. She endorses mild, nonproductive cough. She appears well, speaking full sentences and ambulating without difficulty. She has diminished breath sounds bilaterally. Labs reveal only mild normocytic anemia. She is started on intravenous steroids and inhaler therapy. CT chest without contrast shows diffusely distended esophagus filled with liquid and food debris, exerting mass effect on the trachea and proximal mainstem bronchi, as well as diffuse groundglass opacities and scattered pulmonary nodules. Upon further assessment, the patient endorses occasional dysphagia with solid foods. She has infrequent vomiting episodes but denies odynophagia or weight loss. Eckardt score is 5. EGD reveals dilation in the entire esophagus; EndoFLIP reveals decreased distensibility and absent contractility consistent with type I achalasia. The following day, POEM procedure is performed. Post-procedure Eckardt score is 0. She is discharged home on lansoprazole 30 mg twice daily for 2 months. Over the next 6 months, she experiences complete remission of her asthma symptoms and inhaler therapy is discontinued. She also has complete resolution of dysphagia and gains 50 pounds. DISCUSSION: In our case, the patient had worsening PFT despite adequate treatment for asthma. Chest CT found a dilated esophagus, which may have been present 2 years prior when she was intubated for status asthmaticus. Her respiratory symptoms regressed completely after POEM for achalasia. This case demonstrates the importance of diagnostic review, especially in cases of primary treatment failure. Maintaining a broad differential can help clinicians think critically and correct potential reversible causes.Figure 1.: Chest CT shows diffusely dilated esophagus, likely secondary to achalasia.Figure 2.: Dilated esophagus causing compression of proximal mainstem bronchus.Figure 3.: Liquid and food debris in dilated esophagus near the LES.