Esophageal Intramural Pseudodiverticulosis

Abstract

A 59-year-old-man without significant medical history presented with a 5-year history of progressive dysphagia for solid foods. An upper endoscopy, performed 5 years earlier in another hospital, showed a benign esophageal stricture with Candida albicans infection. He was treated with antifungal drugs, but he refused further investigations for several years. Because he was unable to swallow solids, he presented for further investigation at our institution. Apart from a significant weight loss of 15 kg over several months, the patient had no symptoms. Physical examination was unremarkable, except for a body weight of 45 kg (body mass index, 15.6 kg/m2). Upper endoscopy revealed a stricture between 23 and 29 cm of the incisors that barely could be passed with a pediatric endoscope. The mucosa of the upper esophagus was inflammatory with several small white spots, suggestive for fungal infection. No signs of peptic esophagitis were present at the gastroesophageal junction; stomach and duodenum were normal. Biopsy specimens taken from the stricture showed an ulcerated esophagitis and confirmed the presence of mycosis. Barium esophagogram was pathognomonic for esophageal intramural pseudodiverticulosis (Figure). Esophageal intramural pseudodiverticulosis is a benign condition of which only about 250 cases have been reported worldwide. The condition is characterized by multiple flask-shaped outpouchings in the esophageal wall. These pseudodiverticula, formed by dilatation of the esophageal submucosal glands, typically are diagnosed on barium esophagogram.1Teraishi F. Fujiwara T. Jikuhara A. et al.Esophageal intramural pseudodiverticulosis with esophageal strictures successfully treated with dilation therapy.Ann Thorac Surg. 2006; 82: 1119-1121Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar In many cases the multiple small orifices of the intramural pseudodiverticula can be appreciated on endoscopy.2Hahne M. Schilling D. Arnold J.C. et al.Esophageal intramural pseudodiverticulosis: review of symptoms including upper gastrointestinal bleeding.J Clin Gastroenterol. 2001; 33: 378-382Crossref PubMed Scopus (46) Google Scholar Computerized tomography sometimes shows esophageal wall thickening with diffuse irregularity of the lumen and intramural gas collection. Presenting symptoms are dysphagia and bleeding, but patients may remain asymptomatic.2Hahne M. Schilling D. Arnold J.C. et al.Esophageal intramural pseudodiverticulosis: review of symptoms including upper gastrointestinal bleeding.J Clin Gastroenterol. 2001; 33: 378-382Crossref PubMed Scopus (46) Google Scholar Even though esophageal intramural pseudodiverticulosis is a benign condition, severe complications including fistula formation, perforation, and mediastinitis have been reported. As was the case in our patient, the condition often is complicated by stricture formation. Treatment of the inflammation and endoscopic dilatation of the stenosis is an effective therapy for the dysphagia. Our patient was treated successfully with endoscopic dilatation. Six months after dilatation, he remains asymptomatic and has gained weight.