Esophageal intramural pseudodiverticulosis (EIPD) is a disease of unknown pathogenesis characterized by usually systemic, cystic dilatation of the excretory ducts of esophageal submucosal glands. In this article, I review the epidemiology, clinical manifestations, endoscopic findings, esophagographic findings, and histopathology of EIPD. I also discuss the etiology and possible pathogenesis of EIPD based on my experiences with this disease and a review of the literature. EIPD usually presents with dysphagia in middle-aged individuals. It is often complicated with secondary infections, most commonly candidiasis. On esophagography, EIPD is delineated as small, multiple, flask-shaped outward projections within the esophageal wall. In recent years, EIPD has been mainly diagnosed by endoscopic findings of multiple, localized, small mucosal depressions. The orifices of the "pseudodiverticula" periodically open and close, and excrete mucus onto the mucosal surface. On histopathological examination, the luminal surface of dilated ducts in EIPD is covered by multilayered, hyperplastic epithelial cells, but myoepithelial cells in the glandular acini are well preserved. Treatment of EIPD is usually symptomatic therapy, and prevention of the infectious complications is important. The etiology and pathogenesis of EIPD are largely unknown, but functional abnormalities of autonomic nerve fibers innervating the esophageal glands likely play an important role, since the structures of the glands are basically preserved in this disease.

Oesophageal intraluminal pseudodiverticulosis is a rare benign condition of the oesophageal wall, with not many cases reported in the literature. Usually, patients present with dysphagia and food impaction in association with a proximal oesophageal stricture. Pathogenesis of the disease is not yet established; hence, it remains important to raise awareness about this distinctive pathology. Here, we present a case of a 62-year-old male admitted to Aberdeen Royal Infirmary, Scotland, UK, with a history of food bolus. Upper gastrointestinal endoscopy revealed food bolus impaction with underlying oesophageal pseudodiverticulosis in the distal two-thirds of the oesophagus.

We report a 39-year-old Native American female with an almost 20-year history of dysphagia that had increased in the 6 months prior to the initial evaluation. Investigation revealed a number of distinct esophageal disorders including Plummer-Vinson syndrome, gastroesophageal reflux disease with esophagitis, distal esophageal stricture, esophageal intramural pseudo-diverticulosis, and recurrent esophageal Candida infections. Although prolonged therapy with proton pump inhibitors, fluconazole, nystatin, and repeated esophageal balloon dilations relieved her symptoms, her prognosis remains uncertain.

Esophageal intramural pseudodiverticulosis is a rare disease that may lead to esophageal stenosis and dysphagia. The aim of the study was to evaluate the endoscopic diagnosis, treatment and clinical course of intramural pseudodiverticulosis. We retrospectively studied endoscopic criteria of intramural pseudodiverticulitis, associated diseases, and the clinical course, particularly in patients with dysphagia because of esophageal stenosis in a period from 2002 to 2012. In 23 patients, the diagnosis was made according to endoscopic criteria. As risk factors, alcohol and tobacco consumption were present in all patients. Concomitant candida infection was present in six (26%) patients. In 12 (52%) patients esophageal stenosis was present, which was localized in the upper half of the esophagus. In 11 patients bougienage has been performed with excellent improvement of the dysphagia score from 3.7 to 1.3 (P = 0.002). However, dysphagia was recurrent in four patients with need for repeated bougienage. About half of the patients with intramural pseudodiverticulosis present with stenosis of the esophagus at the time of diagnosis. In patients with proximal esophageal stenosis and a typical risk constellation, intramural pseudodiverticulosis should be suspected. Treatment of stenosis with bougienage is effective to resolve dysphagia, but repeated bougienage may be necessary.

Esophageal intramural pseudodiverticulosis (EIPD) is a rare disorder in adults, and even more in infants and children. It is characterized by the dilatation of the submucosal esophageal glands. The exact etiology and pathophysiology of EIPD is, however, unknown. Dysphagia is the predominant presenting symptom in both children and adults.

Here, we present a case of a boy with persistent dysphagia who had a thorough diagnostic workup and was eventually diagnosed with EIPD.

Esophageal diverticula are rare. The association of cancer and diverticula has been described. Some authors adopt a conservative non-surgical approach in selected patients with diverticula whereas others treat the symptoms by diverticulopexy or myotomy only, leaving the diverticulum in situ. However, the risk of malignant degeneration should be may be taken in account if the diverticulum is not resected. The correct evaluation of the possible risk factors for malignancy may help in the decision making process. We performed a literature review of esophageal diverticula and cancer. The incidence of cancer in a diverticulum is 0.3-7, 1.8, and 0.6% for pharyngoesophageal, midesophageal, and epiphrenic diverticula, respectively. Symptoms may mimic those of the diverticulum or underlying motor disorder. Progressive dysphagia, unintentional weight loss, the presence of blood in the regurgitated material, regurgitation of peaces of the tumor, odynophagia, melena, hemathemesis, and hemoptysis are key symptoms. Risk factors for malignancy are old age, male gender, long-standing history, and larger diverticula. A carcinoma may develop in treated diverticula, even after resection. Outcomes are usually quoted as dismal because of a delayed diagnosis but several cases of superficial carcinoma have been described. The treatment follows the same principals as the therapy for esophageal cancer; however, diverticulectomy is enough in cases of superficial carcinomas. Patients must be carefully evaluated before therapy and a long-term follow-up is advisable.

Esophageal diverticula are rare. They may occur in the pharyngoesophageal area (Zenker's), midesophagus, or distally (epiphrenic). A motility disorder (either at the level of the esophageal sphincters or body) is frequently associated with esophageal diverticula. The risk of malignant transformation is low.

A literature search was performed using Medline/PubMed database.

The treatment of esophageal diverticula must be based on the pathophysiology and natural history of the disease: (a) asymptomatic diverticula do not need a specific treatment, (b) small diverticula may be left in place and not resected, (c) medium-size diverticula may be either treated by diverticulectomy, diverticulopexy, or esophagodiverticulostomy in case of pharyngoesophageal diverticula, (d) resection is probably the ideal therapy for larger diverticula, and (e) a myotomy should always be included to the procedure.

Due to its rarity, esophageal diverticula must be treated by esophageal surgeons since even in experienced hands the complication rate can be significant.

Treatment of symptomatic pharyngeal and esophageal strictures requires endoscopic dilatation. The Savary-Gilliard bougienage was developed by our department and has been used since 1980 for this purpose. We report our experience using this technique. The records of patients seen from January 1, 1963 to December 31, 2005, who had pharyngeal and esophageal strictures needing dilatation, were reviewed. The prevalence of different etiologies, and the incidence of complications using the Savary-Gilliard dilators were assessed. Efficiency of dilatation was assessed over a 17-year segment of this period, using number of dilatations and time intervals between dilatations until resolution of symptoms as outcome measures. Of the 2,652 pharyngeal and esophageal strictures reviewed, 90% were of organic origin (45% benign and 55% malignant stenoses), and 10% were of functional etiology. The most common etiologies were peptic strictures before the era of proton pump inhibitors, and postoperative anastomotic strictures thereafter. A total of 1,862 dilatations using the Savary-Gilliard technique were analyzed. Complication and mortality rates were 0.18 and 0.09% for benign and 4.58 and 0.81% for malignant etiologies, respectively. The number of dilatations per stricture and the time interval between different sessions were dependent on the type of strictures, varying from 1 to 23 dilatations and 7 days to 16 years, respectively. Pharyngeal and esophageal dilatations using the Savary-Gilliard technique were safe when used together with fluoroscopy. Overall, the efficiency of the dilatation procedure was good, but some types of strictures (e.g., caustic, post-surgical and/or post radiotherapy) were refractory to treatment and required repeated dilatations.

This report describes the clinical course of a patient with complications of esophageal intramural pseudodiverticulosis. The condition led to fistulization and abscess formation in the mediastinum. The initial presentation was for the septic process and appropriate antibiotic therapy led to infection control while the abscess drained spontaneously back into the esophageal lumen. A long stricture affecting the distal half of the esophagus became evident after a few months and could not be managed by repeat dilatations. After appropriate preparation, subtotal esophagectomy was offered to the patient with an initial right thoracic approach followed by laparotomy and left cervical reconstruction. A total gastric tube was used for reconstruction and placed in a substernal position. An uneventful postoperative evolution led to normal swallowing comfort.

We report a rare case of esophageal intramural pseudodiverticulosis with esophageal strictures. Barium esophagogram demonstrated multiple flask-shaped diverticula out of the esophageal wall with comprehensive luminal stenosis involving the proximal 8 cm and distal 4 cm of the esophagus. Chest computed tomographic scan demonstrated round wall thickening and several intramural gas collections of the proximal esophagus. Endoscopy revealed a fibrotic stricture and multiple small orifices of pseudodiverticula with mild inflammatory changes. Biopsy specimens showed active chronic inflammatory changes of the mucosa with candidiasis. Dysphagia improved dramatically with esophageal dilation. However, the tiny diverticula did not resolve after treatment.

Our purpose was to determine the frequency of intramural tracking in patients with esophageal intramural pseudodiverticulosis and to characterize the morphologic features of this finding on barium studies.

A review of radiology files at two institutions revealed 30 cases of esophageal intramural pseudodiverticulosis diagnosed at esophagography. In all cases, the radiographs were reviewed retrospectively to determine the frequency and morphologic features of intramural tracking in these patients. The number and distribution of pseudodiverticula and the presence or absence of strictures or esophagitis were also noted.

Fifteen (50%) of 30 patients with esophageal intramural pseudodiverticulosis had intramural tracking on esophagography. The tracks had an average length of 1.2 cm (length range, 0.3-7 cm) and an average width of 1.6 mm (width range, 1-4 mm). The pseudodiverticula were more numerous and had a more diffuse distribution in patients with tracking than in patients without tracking. Although patients with and without tracking had a similar frequency of strictures and esophagitis, patients with tracking were more likely to have strictures involving the upper or mid esophagus, whereas patients without tracking were more likely to have strictures in the distal esophagus. These findings indicate that intramural tracking is more likely to occur in patients with the diffuse form of esophageal intramural pseudodiverticulosis.

Intramural tracking was detected on esophagography in 50% of patients with esophageal intramural pseudodiverticulosis, so this type of tracking is a more common radiographic finding than has previously been recognized. Although intramural tracking has little or no known clinical significance, it is important to be aware of this finding so that it is not mistaken for a large flat ulcer in the esophagus or for an extramural collection associated with esophageal peridiverticulitis.

Esophageal intramural pseudodiverticulosis (EIPD) is a rare benign disorder characterized by dilation of the submucosal glands. Its etiology and pathogenesis are largely unknown. So far, less than 10 pediatric cases of EIPD have been reported. The authors present the case of a 12-year-old boy with EIPD that was associated with a cervical esophageal web. He was treated successfully with dilation therapy.