Intramural esophageal dissection with perforation.

Intramural esophageal dissection (IED) is an uncommon but well-defined clinical entity characterized by a mucosal tear and intramural extension of a false lumen, with or without a re-entry point. The first description of this condition is credited to Marks and Keet1 and has been referred to by a number of terms, including intramucosal esophageal dissection, intramural esophageal rupture, esophageal apoplexy, and submucosal hematoma. In an earlier report, Phan and Heitmiller2 supported use of the term IED, as it was descriptive and did not imply an etiology. The cause of IED is either primary mucosal injury with secondary intramural dissection or primary intramural dissection (usually thought to be bleeding) with secondary perforation into the lumen. In either case, the entity has been traditionally found in elderly women, often those who are taking anticoagulants or who have coagulopathy. However, it is now clear that IED can be found in a wide range of patients. In the case report by Soulellis and associates,3 IED was found in a previously healthy 45-year-old Asian man. In a separate report, Lauzon and Heitmiller4 described a case of IED in a previously healthy male 19-year-old college student. In most cases, IED is associated with mucosal trauma during eating, and its diagnosis is confirmed by contrast esophagogram or endoscopy; in other words, the injury occurs prior to the diagnostic procedure. In the case described by Soulellis and colleagues,3 the patient, who presented with esophageal obstruction from an impacted grape, appeared to develop IED during endoscopic manipulation with air insufflation. According to the case report, the endoscopists saw the mucosal perforation develop with intramural dissection (from air) as they attempted to remove the impacted food material. Their description suggests that the injury was iatrogenic, as the injury appeared under direct endoscopic view. Perhaps this explains why the case was associated with extravasation of air into the mediastinum, a finding that is rarely associated with IED. Skinner and Belsey5 were the first to suggest that esophageal perforation can range in severity. Degrees of perforation (from least virulent to most virulent) include air leakage only, contained perforation with self-drainage, free mediastinal leakage, and free leakage with extension into the pleural space. The patient under the care of Soulellis and coworkers3 experienced air leakage only. Contrast esophagography is the most effective examination for determining the type of perforation. Air leakage only, with no contrast extravasation, can be observed and resolves spontaneously. Contained perforation can be managed nonoperatively, as described by Cameron and coworkers.6 Free, noncontained perforation should be promptly addressed, preferably by primary repair. Is the patient described by Soulellis and associates3 the first reported case of IED with perforation? There are two reasons that this may not be the case. The first is that the IED in this patient may have been iatrogenically caused, as the patient may have presented merely with an impacted food bolus and not with IED. If so, the pathogenesis and possible outcome of this clinical entity, admittedly IED, could be different than the IEDs previously reported in the literature. The other reason is that there appears to be evidence of earlier descriptions of IED associated with esophageal perforation. Postlethwaite,7 in a comprehensive review of intramural rupture and intramural hematoma, reported, “Operation in 7 patients was performed because complete perforation or rupture was suspected. In only 1 patient was operation really beneficial.” This description suggests that there was true perforation in at least 1 previous patient and that this injury required surgical intervention. The clinical characteristics and settings associated with the presentation of IED can vary greatly. The diagnosis of IED must be considered in all patients who present with acute onset odynophagia after eating. As mentioned above, the diagnosis is best confirmed by contrast esophagogram or endoscopy. The advantage of contrast radiology studies is the study will both confirm the diagnosis and determine whether leakage is present or not. In the vast majority of cases, there is no risk of perforation, and patients can be managed nonoperatively with complete resolution. As Soulellis and colleagues3 and Postlethwaite7 have shown, esophageal perforation can be associated with IED and should be considered as well.