Black esophagus: Acute esophageal necrosis syndrome

Acute esophageal necrosis (AEN), commonly referred to as "black esophagus", is a rare clinical entity arising from a combination of ischemic insult seen in hemodynamic compromise and low-flow states, corrosive injury from gastric contents in the setting of esophago-gastroparesis and gastric outlet obstruction, and decreased function of mucosal barrier systems and reparative mechanisms present in malnourished and debilitated physical states. AEN may arise in the setting of multiorgan dysfunction, hypoperfusion, vasculopathy, sepsis, diabetic ketoacidosis, alcohol intoxication, gastric volvulus, traumatic transection of the thoracic aorta, thromboembolic phenomena, and malignancy. Clinical presentation is remarkable for upper gastrointestinal bleeding. Notable symptoms may include epigastric/abdominal pain, vomiting, dysphagia, fever, nausea, and syncope. Associated laboratory f indings may reflect anemia and leukocytosis. The hallmark of this syndrome is the development of diffuse circumferential black mucosal discoloration in the distal esophagus that may extend proximally to involve variable length of the organ. Classic "black esophagus" abruptly stops at the gastroesophageal junction. Biopsy is recommended but not required for the diagnosis. Histologically, necrotic debris, absence of viable squamous epithelium, and necrosis of esophageal mucosa, with possible involvement of submucosa and muscularis propria, are present. Classif ication of the disease spectrumis best described by a staging system. Treatment is directed at correcting coexisting clinical conditions, restoring hemodynamic stability, nil-per-os restriction, supportive red blood cell transfusion, and intravenous acid suppression with proton pump inhibitors. Complications include perforation with mediastinal infection/abscess, esophageal stricture and stenosis, superinfection, and death. A high mortality of 32% seen in the setting of AEN syndrome is usually related to the underlying medical co-morbidities and diseases.

Acute esophageal necrosis: A systematic review and pooled analysis

BACKGROUND Acute esophageal necrosis(AEN)is a rare entity with multifactorial etiology,usually presenting with signs of upper gastrointestinal bleeding.AIM To systematically review all available data on demographics,clinical features,outcomes and management of this medical condition.METHODS A systematic literature search was performed with respect to the PRISMA statement(end-of-search date:October 24,2018).Data on the study design,interventions,participants and outcomes were extracted by two independent reviewers.RESULTS Seventy-nine studies were included in this review.Overall,114 patients with AEN were identified,of whom 83 were males and 31 females.Mean patient age was 62.1±16.1.The most common presenting symptoms were melena,hematemesis or other manifestations of gastric bleeding(85%).The lower esophagus was most commonly involved(92.9%).The most widely implemented treatment modality was conservative treatment(75.4%),while surgical or endoscopic intervention was required in 24.6%of the cases.Mean overall followup was 66.2±101.8 d.Overall 29.9%of patients died either during the initial hospital stay or during the follow-up period.Gastrointestinal symptoms on presentation[Odds ratio 3.50(1.09-11.30),P=0.03]and need for surgical or endoscopic treatment[surgical:Odds ratio 1.25(1.03-1.51),P=0.02;endoscopic:Odds ratio 1.4(1.17-1.66),P<0.01]were associated with increased odds of complications.A sub-analysis separating early versus late cases(after 2006)revealed a significantly increased frequency of surgical or endoscopic intervention(9.7%vs 30.1%respectively,P=0.04)CONCLUSION AEN is a rare condition with controversial pathogenesis and unclear optimal management.Although the frequency of surgical and endoscopic intervention has increased in recent years,outcomes have remained the same.Therefore,further research work is needed to better understand how to best treat this potentially lethal disease.

Acute esophageal necrosis as a complication of diabetic ketoacidosis:A case report

BACKGROUND Acute esophageal necrosis(AEN)is a rare condition that has been associated with low volume states,microvascular disease,gastrointestinal(GI)mucosal damage,and impaired GI motility.It has been linked in case reports with diabetic ketoacidosis(DKA)and is commonly associated with GI bleeding(GIB).CASE SUMMARY We report a case of endoscopy confirmed AEN as a complication of DKA in a 63-year-old Caucasian male without any overt GIB and a chief complaint of epigastric pain.Interestingly,there was no apparent trigger for DKA other than a newly started ketogenic diet two days prior to symptom onset.A possible potentiating factor for AEN beyond DKA is the recent start of a Glucagon-like peptide-1 receptor agonist(GLP-1 RA),though they have not been previously connected to DKA or AEN.The patient was subsequently treated with high dose proton pump inhibitors,GLP-1 RA was discontinued,and an insulin regimen was instituted.The patient’s symptoms improved over the course of several weeks following discharge and repeat endoscopy showed well healing esophageal mucosa.CONCLUSION This report highlights AEN in the absence of overt GIB,emphasizing the importance of early consideration of EGD.