Diffuse esophageal leiomyomatosis: another cause of pseudoachalasia

The patient was a 22-year-old woman who had presented in early childhood wi th gastroesophageal reflux and who subsequently underwent surgery. It was c ommented upon by the surgeons at that time that the esophagus was abnormall y thickened. The patient subsequently presented during her first pregnancy with a vulval tumor, which proved histologically to be a leiomyoma. She was also found to have a grossly dilated esophagus and was thought to have ach alasia. However, endoscopic ultrasound imaging showed gross hypertrophy of the mid- and distal esophageal wall, with only mild symptoms of dysphagia, which had been long-standing. The appearance of the esophagus was consisten t with diffuse esophageal leiomyomatosis. In view of the associated vulval leiomyoma, the patient demonstrated esophagovulvar syndrome.