Spontaneous perforation in the ringed esophagus

A 54-year-old man presented to the ER with chest pain. He underwent an upper endoscopy revealing a large linear esophageal tear and a CT chest showed free air in the mediastinum. He was managed conservatively and was discharged 2 days later. An UGI series revealed a distal esophageal stricture. He was commenced on esomeprazole for gastroesophageal reflux symptoms and his dysphagia improved significantly. Upper endoscopy revealed multiple rings throughout the esophagus. Biopsies from the distal and mid-esophagus were normal. The underlying pathophysiology, in patients with dysphagia and a ringed esophagus has evoked debate in the literature. Opinions range from underlying gastroesophageal reflux disease (GERD) to eosinophilic esophagitis (EE). Our patient's symptoms of GERD and dysphagia resolved with proton pump inhibitor therapy. Normal histology excluded underlying EE. There have been a few case reports of esophageal perforation in patients with a ringed esophagus, and underlying EE, but none with spontaneous perforation occurring in a 'ringed esophagus'. Perforations in the upper and mid-esophagus can usually be managed conservatively, while those in the distal esophagus often need surgery due to the high risk of developing mediastinitis. However, our patient, despite sustaining a large tear in the distal esophagus, did well with conservative management. This case demonstrates that spontaneous perforation in the ringed esophagus, with normal underlying histology can occur in the distal esophagus and may not require surgery.