Rawan Dayah, MD1, August Clark, MD2, Gabriel Reep, MD2, Ikenna Okereke, MD2, Suimin Qiu, MD, PhD2, Karen Nahmood, MD, PhD2
1The University of Texas Medical Branch, Galveston, TX; 2University of Texas Medical Branch, Galveston, TX
Introduction: Achalasia is an esophageal motility disorder in which the lower esophageal sphincter fails to relax and there is a loss of esophageal peristalsis. Pseudoachalasia closely mimics achalasia. We present a rare case of a patient who was initially diagnosed with pseudoachalasia but was later found to have achalasia after symptoms persisted following the resection of an esophageal leiomyoma.
Case Description/Methods: A 39 year-old male was evaluated for weight loss and progressively worsening dysphagia to solids and eventually liquids with a 100 pound weight loss over 3 years. Esophageal manometry was consistent with achalasia with esophageal compression, subtype II (Figure 1). He underwent laparoscopic exploration and planned Heller myotomy and Dor fundoplication. During surgery, a very large submucosal mass was found in the distal esophagus extending to the gastroesophageal junction. The mass was enucleated and a Dor fundoplication was performed. Pathologic analysis showed the mass to be a leiomyoma measuring 6.7 centimeters in maximum diameter (Figure 2). Postoperatively, his symptoms of dysphagia persisted andrepeat esophageal manometry remained consistent with achalasia. He underwent Heller myotomy and redo Dor fundoplication 5 months after the initial surgery. His symptoms completely resolved after the second surgery and he has remained asymptomatic on follow-up.
Discussion: Patients with achalasia typically present with progressive dysphagia, initially to solids and then to liquids, due to the degeneration of ganglion cells within the myenteric plexus in the distal esophagus resulting in the loss of peristalsis. Pseudoachalasia closely mimics the presentation of idiopathic achalasia and is the diagnosis in approximately 4% of patients with achalasia-like symptoms. Intraluminal evaluation with endoscopic ultrasound may be a valuable tool to evaluate muscular wall thickness in motility disorders and could result in earlier diagnosis of pseudoachalasia. Intraoperative esophageal pressure monitoring is not used frequently but may have also been beneficial in this case. This case is unique since the patient was discovered to have an esophageal leiomyoma after he was diagnosed with primary achalasia. There are no documented cases of a patient with both primary achalasia and pseudoachalasia based on a literature review.
Citation: Rawan Dayah, MD; August Clark, MD; Gabriel Reep, MD; Ikenna Okereke, MD; Suimin Qiu, MD, PhD; Karen Nahmood, MD, PhD. P0325 - A RARE CASE OF COEXISTING ACHALASIA AND PSEUDOACHALASIA. Program No. P0325. ACG 2019 Annual Scientific Meeting Abstracts. San Antonio, Texas: American College of Gastroenterology.