Gastroenterology and Hepatology Board Review: Pearls of Wisdom, Third Edition

Section II ESOPHAGUS

CHAPTER 8. Esophageal Congenital and Structural Abnormalities

Ronnie Fass, MD

Image What type of fistula is the most common embryologic developmental anomaly?

Tracheoesophageal fistula (85%–90%). In the most common subtype, the upper part of the esophagus ends as a blind sac, while the lower part is connected posteriorly to the trachea.

Image What is the H-type tracheoesophageal fistula?

This occurs when the esophagus and the trachea are attached by a short connection, creating an H-type fistula.

Image What is the most common congenital abnormality associated with esophageal atresia?

Cardiac abnormality, most commonly patent ductus arteriosus and septal defects.

Image When considering an operation for congenital tracheoesophageal fistula, what is the most important anatomic information the surgeons need?

The type of fistula and whether the distance between the upper and lower ends of the esophagus is long (long gap) or closely approximated (short gap).

Image What is the most common anatomic presentation of esophageal duplication?

In up to 80% of the cases, it presents as a cyst without luminal connection.

Image Where in the esophagus are duplication cysts most commonly encountered?

The most common location is the distal third (60%) followed by the proximal third (23%).

Image At what age do vascular rings usually become symptomatic?

Most commonly during infancy and early childhood, although they may present at any age.

Image What are the most common vascular rings encountered in the pediatric population?

Double aortic arches and right-sided aortic arch with either patent ductus arteriosus or ligamentum arteriosum.

Image True/False: Dysphagia lusoria is most commonly associated with an aberrant aortic arch.

False. Dysphagia lusoria is most commonly associated with an aberrant right subclavian artery. The aberrant artery arises from the left side of the aortic arch and on its course to the right arm compresses the esophagus posteriorly.

Image How common is an aberrant right subclavian artery in the general population?

It has been estimated to occur in up to 1% of the population.

Image True/False: Up to 50% of aberrant right subclavian arteries cause dysphagia.

False. The vast majority (90%) are asymptomatic.

Image What are the esophageal A-ring, B-ring, and C-ring?

These are radiographic terms. The A-ring is usually asymptomatic and involves hypertrophied or hypertonic muscle typically 1.5–2 cm above the squamocolumnar junction. The B-ring is synonymous with Schatzki’s ring and involves only mucosa. A C-ring refers to the indentation on the esophagus created by the diaphragmatic crura.

Image How common is a Schatzki’s ring?

Unknown, because most of Schatzki’s rings are asymptomatic. They are found in up to 14% of routine barium esophagrams.

Image What is the relationship between luminal diameter of Schatzki’s ring and dysphagia symptoms?

Patients with Schatzki’s ring and esophageal lumen less than 13 mm will almost always experience dysphagia, between 13 and 20 mm may or may not have dysphagia (about 50%) and greater than 20 mm will rarely have dysphagia.

Image What “syndrome” has been associated with Schatzki’s ring?

“Steakhouse syndrome,” which refers to the occurrence of acute dysphagia due to food impaction.

Image What pathogenetic mechanisms have been implicated in the formation of Schatzki’s ring?

Pill-induced, gastroesophageal reflux disease, and congenital.

Image True/False: Endoscopy is the best diagnostic test to detect an esophageal ring.

False. The barium esophagram is a more sensitive test. The use of a barium tablet or marshmallow may help even further to identify a subtle ring and to estimate its luminal diameter.

Image How can a muscular ring be differentiated from Schatzki’s ring radiographically?

On barium swallow, the caliber of the muscular ring varies, and the stenosis may disappear with full distension. The Schatzki’s ring does not vary in appearance.

Image What is the usual histology of a Schatzki’s ring?

As the rings are most often located at the gastroesophageal junction, the upper surface usually has squamous epithelium, and the lower surface is covered with columnar cells.

Image What are typical clinical signs of Schatzki’s ring?

Age greater than 40, intermittent solid dysphagia, and worse when eating is hurried.

Image What percent of patients with Schatzki’s ring remain symptom-free after esophageal dilation at 1-, 2-, and 3-year follow-up?

68%, 35%, and 11%, respectively. Usually, passage of a large caliber dilator (eg, 16–19 mm) is most helpful.

Image What endoscopic treatment options may be helpful for Schatzki’s rings that have been refractory to esophageal dilatation?

Four-quadrant biopsies of the ring or four-quadrant incisions of the ring using a needle-knife papillotome.

Image Where is the most common location of an esophageal web?

Esophageal webs can appear anywhere in the esophagus but tend to occur most commonly in the proximal part.

Image What percentage of patients with dysphagia will be found to have an esophageal web?

5%–15%.

Image What is the Plummer–Vinson or Paterson–Kelly syndrome?

Esophageal web that is associated with glossitis, iron-deficiency anemia, and koilonychia.

Image What types of cancers have been associated with Plummer–Vinson syndrome?

Pharyngeal and proximal esophageal squamous cell cancers.

Image True/False: Esophageal webs that are associated with iron deficiency improve with iron supplements.

False. The esophageal webs do not seem to consistently improve with iron therapy.

Image What dermatological diseases have been associated with esophageal webs?

Cicatricial pemphigoid and epidermolysis bullosa. Other associated skin diseases include Stevens–Johnson syndrome and psoriasis.

Image After allogeneic bone marrow transplantation, what complication has been associated with the development of an esophageal web?

Graft-versus-host disease.

Image True/False: Esophageal webs are more common in women.

True.

Image What esophageal disorders have been associated with webs?

Inlet patch, Zenker’s diverticulum, and esophageal duplication cyst.

Image True/False: Zenker’s diverticulum is the most common esophageal diverticulum.

False. Although Zenker’s diverticulum is commonly thought of as an esophageal diverticulum, it actually forms in a hypopharyngeal location; in Killian’s triangle just proximal to the cricopharyngeus muscle.

Image What is the estimated prevalence of Zenker’s diverticulum in the general population?

0.01%–0.11%.

Image At what age does a Zenker’s diverticulum commonly present?

Almost half of the cases will present during the seventh to eighth decade of life.

Image How commonly does squamous cell carcinoma occur in a Zenker’s diverticulum?

It is seen in approximately 0.4% of patients.

Image What surgical techniques are used to treat a Zenker’s diverticulum?

Diverticulopexy, diverticulectomy, and cricopharyngeal myotomy.

Image True/False: Endoscopic approaches have also been described to treat a Zenker’s diverticulum.

True. Endoscopic incision of the septum between the diverticulum and the esophageal lumen is included as a treatment option for the Zenker’s diverticulum.

Image What is the most common cause of midesophageal diverticula?

Esophageal motor dysfunction resulting in high intraluminal pressure, outpouching, and the formation of pulsion diverticula.

Image True/False: Most patients with midesophageal diverticula complain of dysphagia.

False. In most patients, the diverticula are asymptomatic and are incidentally discovered during barium esophagram. In a small percentage of patients, they can cause dysphagia and chest pain.

Image What is a traction diverticulum?

Midesophageal diverticula were once considered to arise as a result of traction due to paraesophageal inflammation, most commonly from tuberculosis and fungal diseases.

Image What is the likely cause of epiphrenic diverticula?

As with midesophageal diverticula, esophageal motor disorders are believed to be the underlying mechanism for epiphrenic diverticula, which occur just proximal to the lower esophageal sphincter (LES).

Image What esophageal motility abnormalities have been documented in association with epiphrenic diverticula?

Achalasia, diffuse esophageal spasm, hypertensive lower esophageal sphincter, and nutcracker esophagus.

Image What percent of dysphagia cases are due to esophageal diverticula?

Less than 5%.

Image What is esophageal intramural pseudodiverticulosis?

Multiple, small (1–3 mm), flask-shaped outpouching of the esophagus.

Image What is the pathogenesis of esophageal intramural pseudodiverticulosis?

Cystic dilations of the esophageal gland ducts.

Image What infection can be detected in about one half of patients with esophageal intramural pseudodiverticulosis?

Esophageal candidiasis.

Image What esophageal lesion is almost always associated with esophageal intramural pseudodiverticulosis?

Esophageal stricture located in the upper or midesophagus. The pseudodiverticula are often observed distal to the stricture.

Image What is the incidence of an inlet patch?

It ranges between 4% and10%.

Image What type of gastric mucosa can be found in an inlet patch?

Gastric corpus or fundic mucosa that can include functional parietal and chief cells.

Image What complications have been described in association with an inlet patch?

Uncommonly, proximal esophageal stricture, ulcer, and esophageal adenocarcinoma.

Image What symptom has been suggested to be associated with an inlet patch?

Globus sensation. Obliteration of the patch may result in symptom resolution.

• • • SUGGESTED READINGS • • •

Kinottenbelt G, Skinner A, Seefelder C. Tracheo-oesophageal fistula (TOF) and oesophageal atresia (OA). Best Pract Res Clin Anaesthesiol. 2010 Sep;24(3):387-401.

Tobin RW. Esophageal rings, webs, and diverticula. J Clin Gastroenterol. 1998 Dec;27(4):285-295.

Poyrazoglu OK, Bahcecioglu IH, Dagli AF, Ataseven H, Celebi S, Yalniz M. Heterotopic gastric mucosa (inlet patch): endoscopic prevalence, histopathological, demographical and clinical characteristics. Int J Clin Pract. 2009 Feb;63(2):287-291.

Levitt B, Richter JE. Dysphagia lusoria: a comprehensive review. Dis Esophagus. 2007;20(6):455-460.