2. AEN (BLACK ESOPHAGUS, NECROTIZING
ESOPHAGITIS)
CRITERIA:
•ACUTE PRESENTATION WITH ENDOSCOPIC FINDINGS CONSISTING OF A
CIRCUMFERENTIAL BLACK ESOPHAGUS WITH OR WITHOUT EXUDATES.
•DISTAL ESOPHAGEAL INVOLVEMENT THAT CAN EXTEND PROXIMALLY, BUT ENDS SHARPLY
AT THE GASTROESOPHAGEAL BORDER.
•UNIFORM HISTOLOGIC FINDINGS AFFECTING THE MUCOSA AND SUBMUCOSA AND
CONSISTING OF DIFFUSE AND SEVERE NECROSIS, WITHOUT RECOGNIZABLE STRATIFIED
SQUAMOUS CELLS, OCCASIONAL DERANGED MUSCLE FIBERS, HYPEREMIA AND
SCATTERED THROMBOSED VESSELS WITHOUT SPECIFIC CAUSATIVE AGENTS.
•OCCURRENCE IN THE ABSENCE OF CAUSTIC OR OTHER INJURIOUS AGENTS
3. PATHOGENESIS
• UNKNOWN. THE PRESUMED OVERALL UNDERSTANDING IS THAT OF A
"TWO HIT" PHENOMENON. INITIAL EVENT ( LOW FLOW VASCULAR STATE),
WHICH THEN PREDISPOSES THE ESOPHAGEAL MUCOSA TO A SEVERE
TOPICAL INJURY ( REFLUX OF ACID AND PEPSIN).
• ISCHEMIA LIKELY HAS A ROLE BASED UPON HISTOPATHOLOGIC AND
CLINICAL DATA
• IN SUPPORT OF THIS HYPOTHESIS IS THE OBSERVATION THAT
TEMPORARY REDUCTION OF ESOPHAGEAL BLOOD PERFUSION CAN
RESULT IN EXTENSIVE ESOPHAGEAL NECROSIS, WHICH RESOLVES
RAPIDLY WHEN PERFUSION IS RESTORED
• FURTHERMORE, AEN TENDS TO OCCUR IN THE DISTAL THIRD OF THE
ESOPHAGUS, WHICH IS RELATIVELY HYPOVASCULAR COMPARED WITH
OTHER ESOPHAGEAL SEGMENTS
• FINALLY, THE NECROSIS OF THE MUCOSA AND SUBMUCOSA,
MICROSCOPIC THROMBOSIS AND RAPID REGRESSION ARE SIMILAR TO
5. DIFFERENTIAL DIAGNOSIS
• MELANOSIS — IN PATIENTS WITH UNDERLYING CHRONIC ESOPHAGITIS. SEEN IN THE
DISTAL ESOPHAGUS.
• PSEUDOMELANOSIS — DUE TO TISSUE DEPOSITION OF PSEUDOMELANIN, A "WEAR AND
TEAR" PIGMENT DERIVED FROM LYSOSOMAL DEGRADATION. HISTOLOGICALLY IT IS
SEEN AS BROWN PIGMENT WITHIN MACROPHAGES
• MELANOMA — RARE. IT USUALLY ORIGINATES IN THE MID AND LOWER ESOPHAGUS
• ACANTHOSIS NIGRICANS — VELVETY, VERRUCOUS, HYPERPIGMENTED SKIN AND
MUCOSAL PLAQUES.CAN BE BENIGN, IT CAN ALSO BE A PARANEOPLASTIC
PHENOMENON, COMMONLY ASSOCIATED WITH INTRA-ABDOMINAL MALIGNANCIES
• COAL DUST AND EXOGENOUS DYE INGESTION — MOST COMMON EXOGENOUS
PIGMENT TO DEPOSIT IN HUMAN BODY TISSUES
• PSEUDOMEMBRANOUS ESOPHAGITIS — ASSOCIATION WITH SERIOUS SYSTEMIC
ILLNESS. A THIN, YELLOW OR BLACK, CONCENTRIC MEMBRANE COATS THE DISTAL (AND
LESS COMMONLY ENTIRE) ESOPHAGUS. THE MEMBRANE CAN BE DISLODGED
REVEALING A FRIABLE UNDERLYING MUCOSA
6. TREATMENT
• ADEQUATE HYDRATION AND TREATMENT OF THE UNDERLYING ILLNESS.
• AGGRESSIVE ACID SUPPRESSION, WITH INTRAVENOUS PROTON PUMP INHIBITORS.
• ORAL INTAKE SHOULD BE AVOIDED FOR AT LEAST 24 HOURS AFTER WHICH SUCRALFATE
SUSPENSION SHOULD BE CONSIDERED BECAUSE OF ITS THEORETICAL ROLE IN THE
PREVENTION OF FURTHER ESOPHAGEAL INJURY DUE TO ITS CYTOPROTECTIVE EFFECTS AND
ITS ABILITY TO BIND PEPSIN AND STIMULATE MUCUS SECRETION
• NASOGASTRIC TUBES SHOULD BE WITHHELD UNLESS USED TO DECOMPRESS A GASTRIC
OUTLET OBSTRUCTION OR IF PERSISTENT VOMITING IS PRESENT.
• A DECISION REGARDING ANTIBIOTICS SHOULD BE MADE ON AN INDIVIDUAL BASIS, ESPECIALLY
IN THE SETTING OF PATIENTS WHO ARE CRITICALLY ILL OR APPEAR TO BE SEPTIC.
• NEED FOR REPEAT ENDOSCOPY SHOULD BE GUIDED BY THE PATIENT'S CLINICAL COURSE.
• AS A GENERAL RULE, THE ENDOSCOPIC FINDINGS REVERT TO NORMAL FAIRLY RAPIDLY.
• IT IS IMPORTANT TO MONITOR FOR COMPLICATIONS SUCH AS ESOPHAGEAL STENOSIS, WHICH
MAY PRESENT AS DYSPHAGIA, AND MAY REQUIRE REPEAT ENDOSCOPY FOR DIAGNOSIS AND
THERAPY.
7. PROGNOSIS
• HIGH MORTALITY RELATED TO THE UNDERLYING
ILLNESSES IN PATIENTS WITH ACUTE
ESOPHAGEAL NECROSIS (AEN) (AND THE
LIMITED NUMBER OF REPORTED CASES)
OBSCURES A DETAILED UNDERSTANDING OF ITS
NATURAL HISTORY.