Chapter 312 Obstructing and Motility Disorders of the Esophagus
Obstructing lesions classically produce dysphagia to solids earlier and more noticeably than to liquids and can manifest when the infant liquid diet begins to incorporate solids; this is in contrast to dysphagia from dysmotility, in which swallowing of liquids is affected as early as, or earlier than, solids. In most instances of dysphagia, evaluation begins with fluoroscopy, which may include videofluoroscopic evaluation of swallowing, particularly if aspiration is a primary symptom. Secondary studies are often endoscopic if intrinsic obstruction is suspected or manometric if dysmotility is suspected; other imaging studies may be used in particular cases. Congenital lesions can require surgery, whereas webs and peptic strictures might respond adequately to endoscopic (or bougie) dilation. Peptic strictures, once dilated, should prompt consideration of fundoplication for ongoing prophylaxis.
Esophageal duplication cysts are the most commonly encountered foregut duplications. These cysts are lined by intestinal epithelium, have a well-developed smooth muscle wall, and are attached to the normal gastrointestinal tract. Two thirds occur on the right side of the esophagus. The most common presentation is respiratory distress caused by compression of the adjacent airways. Dysphagia is a common symptom in older children. Upper gastrointestinal bleeding can occur as a result of acid-secreting gastric mucosa in the duplication wall. Neuroenteric cysts might contain glial elements and are associated with vertebral anomalies. Diagnosis is made on either barium swallow or chest CT. Treatment is surgical; laparoscopic approach to excision is also possible.
Enlarged mediastinal or subcarinal lymph nodes, caused by infection (tuberculosis, histoplasmosis) or neoplasm (lymphoma), are the most common external masses that compress the esophagus and produce obstructive symptoms. Vascular anomalies can also compress the esophagus; dysphagia lusoria is a term denoting the dysphagia produced by a developmental vascular anomaly, which is often an aberrant right subclavian artery or right-sided or double aortic arch (Chapter 426.1).
Intrinsic narrowing of the esophageal lumen can be congenital or acquired. The etiology is suggested by the location, the character of the lesion, and the clinical situation. The lower esophagus is the most common location for peptic strictures, which are generally somewhat ragged and several cm long. Thin membranous rings, including the Schatzki ring at the squamocolumnar junction, can also occlude this area. In the mid esophagus, congenital narrowing may be associated with the esophageal atresia–tracheoesophageal fistula complex, in which some of the lesions might incorporate cartilage and might be impossible to dilate safely; alternatively, reflux esophagitis can induce a ragged and extensive narrowing that appears more proximal than the usual peptic stricture, often because of an associated hiatal hernia. Congenital webs or rings can narrow the upper esophagus. The upper esophagus can also be narrowed by an inflammatory stricture occurring after a caustic ingestion or due to epidermolysis bullosa. Cricopharyngeal achalasia can appear radiographically as a cricopharyngeal “bar” posteriorly in the upper esophagus. Eosinophilic esophagitis (EoE) is currently one of the most common causes for esophageal obstructive symptoms. Although the pathogenesis of obstructive EoE is not yet completely explained and seems to vary among individual patients, endoscopy or radiology demonstrate stricture formation in some children with EoE, and in others a noncompliant esophagus is evident, with thickened wall layers demonstrable by ultrasonography.
Erdeve O, Kologlu M, Atasay B, Arsan S. Primary cricopharyngeal achalasia in a newborn treated by balloon dilatation. Int J Pediatr Otorhinolaryngol. 2007;71:165-168.
Marlais M, Fishman JR, Fell JME, et al. UK incidence of achalasia: an 11-year national epidemiological study. Arch Dis Child. 2011;96:192-194.
Mishra A, Wang M, Pemmaraju VR, et al. Esophageal remodeling develops as a consequence of tissue specific IL-5–induced eosinophilia. Gastroenterology. 2008;134:204-214.
Nurko S, Rosen R. Esophageal dysmotility in patients who have eosinophilic esophagitis. Gastrointest Endosc Clin N Am. 2008;18:73-89. ix