Diseases of the Esophagus

IDKD 2006 Diseases of the Esophagus M.S. Levine Gastrointestinal Radiology, Department of Radiology, University of Pennsylvania Medical Center, Philadelphia, PA, USA Introduction can also lead to longitudinal shortening of the esophagus and the development of fixed transverse folds, producing a This syllabus reviews the findings on esophagography for ‘stepladder’ appearance due to pooling of barium between a variety of esophageal diseases, including reflux the folds [5]. These folds should be differentiated from the esophagitis, Barrett’s esophagus, other types of esophagi- thin transverse striations (i.e., ‘feline’ esophagus) often seen tis, benign and malignant esophageal tumors, varices, as a transient finding at fluoroscopy due to contraction of the lower esophageal rings, esophageal intramural pseudodi- longitudinally oriented muscularis mucosae [6]. verticulosis, and esophageal motility disorders. Barrett’s Esophagus Reflux Esophagitis Barrett’s esophagus is a premalignant condition in which Reflux esophagitis is by far the most common inflamma- there is progressive columnar metaplasia of the distal tory disease involving the esophagus. The single most esophagus due to chronic reflux and reflux esophagitis. common sign of reflux esophagitis on double-contrast Barrett’s esophagus is thought to develop in about 10% of esophagrams is a finely nodular or granular appearance all patients with reflux esophagitis. Double-contrast in the distal third of the esophagus, with poorly defined esophagrams can be used to classify patients with reflux radiolucencies that fade peripherally due to edema and symptoms at high, moderate, or low risk for Barrett’s inflammation of the mucosa [1]. In other patients, barium esophagus, based on specific radiologic criteria [7]. Patients studies may reveal shallow ulcers and erosions in the dis- are classified at high risk when double-contrast esopha- tal esophagus contiguous with the gastroesophageal junc- grams reveal a mid-esophageal stricture or ulcer, or a retic- tion [2]. Reflux esophagitis may also be manifested by ular mucosal pattern (usually associated with a hiatal her- thickened longitudinal folds due to edema and inflamma- nia and/or gastroesophageal reflux) [7]. In such cases, en- tion that extend into the submucosa. However, thickened doscopy and biopsy should be performed for a definitive di- folds should be recognized as a nonspecific finding of agnosis. Patients are classified at moderate risk for Barrett’s esophagitis. Other patients with reflux esophagitis may esophagus when double-contrast studies reveal reflux have a single enlarged, chronically inflamed fold that esophagitis or peptic strictures in the distal esophagus [7]. arises at the gastric cardia and extends into the distal The decision for endoscopy in this group should be based esophagus as a smooth protuberance, also known as an on the severity of symptoms, age, and overall health of the inflammatory esophagogastric polyp [3]. These lesions patients. Finally, patients are classified at low risk for have no malignant potential, so endoscopy is not war- Barrett’s esophagus when double-contrast studies reveal no ranted when barium studies reveal typical findings of an structural abnormalities. The majority of patients are found inflammatory polyp in the distal esophagus. to be in this category, and the prevalence of Barrett’s esoph- Scarring from reflux esophagitis can lead to the develop- agus is so low that they can be treated empirically for their ment of a reflux-induced stricture (i.e., ‘peptic’ stricture) in reflux symptoms, without need for endoscopy [7]. the distal esophagus, almost always above a hiatal hernia. Such strictures typically appear as smooth, tapered segments of concentric narrowing, but asymmetric scarring can lead Infectious Esophagitis to asymmetric narrowing with focal sacculation or balloon- ing of the esophageal wall between areas of fibrosis. Other Candida Esophagitis peptic strictures may be manifested by short, ring-like areas of narrowing that could be mistaken for Schatzki rings in pa- Candida albicans is the most common cause of infec- tients with dysphagia [4]. Scarring from reflux esophagitis tious esophagitis. It usually occurs as an opportunistic in- 46 M.S.Levine fection in immunocompromised patients, particularly AIDS patients. Only about 50% of patients with Candida esophagitis are found to have thrush, so the absence of oropharyngeal disease in no way excludes this diagnosis. Candida esophagitis is usually manifested on double- contrast studies by multiple discrete plaque-like lesions that tend to be oriented longitudinally and are separated by normal mucosa [8]. Double-contrast esophagrams have a sensitivity of 90% in detecting Candida esophagitis [8], primarily because of their ability to demonstrate these mucosal plaques. During the past two decades, a much more fulminant form of candidiasis has been encountered in patients with AIDS, who may present with a grossly irregu- lar or ‘shaggy’ esophagus caused by innumerable coales- cent pseudomembranes and plaques with trapping of barium between these lesions (Fig. 1) [9]. Other patients with Fig. 2. Herpes esophagitis. Double- contrast esophagram shows multi- achalasia or scleroderma may develop a ‘foamy’ esopha- ple small, discrete ulcers with sur- gus with innumerable tiny bubbles layering out in the bar- rounding mounds of edema (ar- ium column; this phenomenon presumably results from rows) in mid-esophagus the yeast form of fungal infection [10]. When typical findings of Candida esophagitis are encountered on double- contrast esophagrams, these patients can be treated with clinical setting, small, discrete ulcers without plaques antifungal agents without the need for endoscopy. should be highly suggestive of herpes esophagitis, as ul- ceration in candidiasis almost always occurs on a back- Herpes Esophagitis ground of diffuse plaque formation. As the disease pro- gresses, however, herpes esophagitis may be manifested The herpes simplex virus type 1 is another common by a combination of ulcers and plaques, mimicking cause of infectious esophagitis. Most affected patients are Candida esophagitis [12]. immunocompromised, but herpes esophagitis may occasionally develop as an acute, self-limited disease in oth- Cytomegalovirus Esophagitis erwise healthy individuals [11]. Viral infection initially leads to the development of small vesicles that rupture to Cytomegalovirus (CMV) is another cause of infectious form discrete, punched-out ulcers on the mucosa. As a re- esophagitis that occurs in patients with AIDS. CMV sult, herpes esophagitis may be manifested on double- esophagitis may be manifested on double-contrast studies contrast studies by multiple superficial ulcers on a nor- by multiple small ulcers or, even more commonly, by one mal background mucosa (Fig. 2) [12]. In the appropriate or more giant, flat ulcers that are several centimeters or more in length [13]. Herpetic ulcers rarely become this large, so the presence of one or more giant ulcers should suggest CMV esophagitis in patients with AIDS. However, the differential diagnosis also includes giant human immunodeficiency virus (HIV) ulcers in the esophagus (see next section). Because CMV is treated with toxic antiviral agents such as ganciclovir, endoscopy is required to con- firm the presence of CMV before treating these patients. Human Immunodeficiency Virus Esophagitis HIV infection can lead to the development of giant esophageal ulcers indistinguishable from those caused by CMV. Double-contrast esophagrams typically reveal one or more giant ulcers surrounded by a radiolucent rim of Fig. 1. Advanced Candida eso- edema, sometimes associated with a cluster of small phagitis in a patient with AIDS. satellite ulcers (Fig. 3) [14]. Occasionally, these individ- Double-contrast esophagram shows uals may have associated palatal ulcers or a characteris- ‘shaggy’ esophagus of fulminant tic rash on the upper body. The diagnosis is established esophageal candidiasis due to innumerable plaques and pseudomem- by obtaining endoscopic biopsy specimens, brushings, or branes with trapping of barium be- cultures to rule out CMV esophagitis as the cause of the tween lesions ulcers. Unlike CMV ulcers, HIV-related esophageal ul- Diseases of the Esophagus 47 ogy is uncertain, but this condition most likely develops as a result of an inflammatory response to ingested food allergens. Most adults with IEE are young men with long- standing dysphagia and recurrent food impactions [16]. They classically have an atopic history (e.g., asthma, al- lergic rhinitis) and peripheral eosinophilia, but IEE fre- quently occurs as an isolated condition [16]. Affected individuals are treated with topical steroids (swallowing me- tered doses of aerosolized steroid preparations) and pro- Fig. 3. HIV ulcer in a patient with AIDS. Double-contrast esopha- tein-free diets with varying degrees of success. gram shows large, flat ulcer in pro- IEE may be manifested on esophagography by segmen- file (arrows) in distal esophagus. tal strictures in the esophagus. The strictures often contain Although CMV esophagitis could distinctive ring-like indentations, resulting in a so-called produce identical findings, endo- ‘ringed’ esophagus [16]. Other patients with IEE may have scopic brushings and biopsies re- vealed no evidence of CMV diffuse esophageal narrowing, resulting in a ‘small-cal- iber’ esophagus [16]. A ringed esophagus has also been described in congenital esophageal stenosis. Affected

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