Epiphrenic esophageal diverticulum within a leiomyoma: A rare cause of dysphagia

Introduction: The diagnosis of symptomatic epiphrenic esophageal diverticulum is rare (15-20%). Esophageal leiomyoma and epiphrenic diverticula are both uncommon, but may rarely occur together. In this patient a leiomyoma was found within an epiphrenic diverticulum. Methods: We report the case of a 58-year-old man referred to our unit with a 4-year history of progressive and intermittent dysphagia, mainly to solids, and weight loss. Barium swallow revealed a distal esophageal stenosis secondary to a diverticulum; upper endoscopy showed an extrinsic submucosal compression of the cardia; and CT scan showed an infradiaphragmatic saccular dilation of the esophagus with a thickened wall. The patient was diagnosed with distal esophageal diverticulum with a submucosal tumor inside. Laparoscopic hiatal diverticulectomy including tumor, cardiomyotomy and a partial fundoplication was performed. Our objective is to discuss the diagnosis and treatment of this disease. Results: The pathological study confirmed a leiomyoma of 5x4 cm in an esophageal diverticulum. On the 3rd postoperative day, barium swallow confirmed good esophageal passage and gastric emptying. The patient was discharged on day 4. In the follow-up monitoring after one month and one year the patient was asymptomatic with good quality of life. Discussion: Traditionally, treatment for an epiphrenic diverticulum consists of a diverticulectomy with or without myotomy, performed through a thoracotomy. Hiatal laparoscopy obviates the need for thoracotomy and placement of a chest tube. It would also allow an easier fundoplication after myotomy and diverticulectomy. This case has several unique aspects. Firstly, an esophageal diverticulum with a leiomyoma inside, which is in a subdiaphragmatic location; and secondly, the fact that it is not associated with primary motor disorder. Here, the leiomyoma causes the epiphrenic diverticulum. Conclusion: Whenever a submucosal tumor within the diverticulum is present careful consideration should be given to the possibility of an associated neoplasm. We suggest that the majority of epiphrenic diverticula of the distal esophagus can be treated successfully by laparoscopic approach, which makes it possible to remove the associated tumor; and that it is at least as safe and effective as conventional transthoracic access but with the advantages of an early postoperative recovery. Introduction Esophageal leiomyoma and epiphrenic diverticula are both uncommon, but may rarely occur together. The epiphrenic diverticula are found in the last 10 cm of the esophagus; they represent 10% of all esophageal diverticula, with a radiological prevalence of 0.015% in the US and up to 2% in Europe [1], although the actual prevalence is unknown. They are usually the result of a pulsion mechanism, originated by an alteration of the esophageal motility [1-3]. The most frequently associated cause is achalasia, although it also has been identified in patients with diffuse esophageal spasm, symptomatic esophageal peristalsis and less often with peptic strictures and tumors [2]. Leiomyoma is the most common benign esophageal tumor, and yet it is 50 times less frequent than esophageal carcinoma. Their incidence ranges from 0.005% to 5% [3], and they only represent 0.4% of all esophageal tumors. 50% of them are located in the distal esophagus and usually intramurally [4]. Intradiverticular neoplasms are rare, and most are squamous cell carcinomas. A few cases have been published of leiomyoma [5,6], malignant fibrous histiocytoma and metastatic neuroma [7]. There is little scientific literature on this topic and multiple controversies exist regarding the management of this disease. Clinical case We present the case of a 58-year-old man, without toxic habits and a medical history of malaria, trigeminal neuralgia, total thyroidectomy, and parathyroid resection for papillary cancer, hypothyroidism, and postoperative hypoparathyroidism. He reports a 4-year history of dysphagia, first intermittent and then progressive, both to solid and liquids, exacerbated in the last 6 months, and associated with regurgitation and with loss of 10 kg of weight, without associated vomiting. He has not had previous episodes of aspiration or respiratory infections. The findings on the physical examination were within normal Correspondence to: Dr. Omar Abdel-lah Fernández, MD, Gastro-esophageal Pathology Unit of the Department of Surgery and Digestive System, University Hospital of Salamanca, C/Muñoz Torrero 1 5B, 37007 Salamanca. Spain, Tel: +34 923261198; E-mail: [email protected]