Digestive Endoscopy (2005) 17, 89–92
CASE REPORT
ACUTE ESOPHAGEAL NECROSIS MARCOS CARNEIRO,* MANUEL LESCANO,* LUIZA ROMANELLO,† JOSÉ MÓDENA,† FABIANA CARNEIRO,‡ LEANDRA RAMALHO,‡ ANA MARTINELLI* AND ALEX FRANÇA* ‡
*Division of Gastroenterology, Department of Internal Medicine, †Gastrointestinal Endoscopy Unit and Department of Pathology, University Hospital, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Ribeirão Preto, SP, Brazil
Acute esophageal necrosis (AEN) is defined as a diffuse blackened aspect of the esophagus observed by upper gastrointestinal endoscopy associated with the histopathological findings of necrosis of the esophageal mucosa. In general, the condition is present in severely compromised patients. Its cause remains unknown but is probably multifactorial. In the present case report we describe the clinical, endoscopic and histopathological characteristics of black esophagus observed in three patients and present a review of the literature.
Key words: acute esophageal necrosis, black esophagus.
INTRODUCTION Acute esophageal necrosis (AEN), also called ‘black esophagus’, is defined as a blackened aspect of the esophagus observed by upper gastrointestinal endoscopy associated with the histopathological finding of necrosis of the esophageal mucosa. The first endoscopic description was reported in 1990 by Goldenberg et al.1 and several cases have been published in the literature since then. AEN is frequently associated with severe clinical conditions such as hypovolemic shock, septic shock, hyperglycemia, hypothermia, and liver disease, with impairment of the hemodynamic equilibrium of the patient. Its cause continues to be unknown and its morbidity and mortality are mainly related to the main disease. In the present report we describe the clinical, endoscopic and histopathological characteristics of three patients with a diagnosis of AEN.
CASE REPORT Case 1 A 55-year-old man with cirrhosis of the liver was admitted to University Hospital, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Brazil with a history of fever (40∞C), diarrhea and deterioration of general condition of 11 days duration. The patient was hospitalized for 5 days, being treated with chloramphenicol. He was referred to our service after clinical worsening. Physical examination revealed a patient in poor general condition, pale, dehydrated, afebrile, with a pulse of 126 b.p.m., hypotense, tachypneic, and with no evidence of abdominal disease. Laboratory tests revealed hemoglobin 5.2 g/dL, hematocrit 16%, leukocytes 2700 cells/ Correspondence: Alex Vianey Callado França, Departamento de Clínica Médica, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Av. Bandeirantes, 3900, Monte Alegre, Ribeirão Preto, SP 14048-900, Brazil. Email:
[email protected] Received 20 May 2004; accepted 5 July 2004.
mm3, platelets 27 000/mL, sodium 136 mEq/L, potassium 3.0 mEq/L, urea 26 mg/dL, creatinine 1.1 mg/dL, glycemia 247 mg/dL, amylase 27.4 U/L, aspartate aminotransferase (AST) 39 U/L, alkaline phosphatase 74 U/L, gammaglutamyl transpeptidase 142.4 U/L, total bilirubin 3.0 mg/dL, International Normalized Ratio (INR) 1.6, albumin 2.7 g/dL, negative antibody to human immunodeficiency virus (anti-HIV) and to hepatitis C virus (anti-HCV), and negative hepatitis B surface antigen (HbsAg). A chest X-ray showed no abnormalities. An abdominal ultrasound revealed signs of chronic hepatic disease and a small amount of ascitic fluid volume. Endoscopy revealed a blackened and friable esophagus throughout the extension of the organ, with cotton-like pinpoint spots in the upper third, hiatus hernia, enanthematous pangastritis with antral erosions, and erosive duodenitis (Fig. 1). An esophageal biopsy revealed necrosis of the mucosa (Fig. 2) with the presence of spores and pseudohyphae suggestive of Candida sp. (Fig. 3). The initial treatment consisted of intravenous (i.v.) administration of fluids and red blood cell concentrates, and oral administration of omeprazole and fluconazole. The patient developed hospital pneumonia, which was treated with cefepime and amikacin i.v., and was discharged on the 14th day of hospitalization. Case 2 A 64-year-old man with cirrhosis of the liver and chronic pancreatitis secondary to alcoholism was admitted to University Hospital, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Brazil with a history of hematochezia of 4 h duration. Physical examination revealed the patient to be pale, hydrated, icteric, afebrile, with a pulse of 116 b.p.m., arterial pressure of 120/60 mmHg, eupneic, with collateral abdominal circulation, vascular spiders on the trunk, and presence of fresh blood revealed by rectal examination. Laboratory tests showed hemoglobin 5.6 g/dL, hematocrit 20%, leukocytes 4900 cells/mm3, platelets 140 000/mL, sodium 138.5 mEq/L, potassium 3.9 mEq/L, urea 19 mg/dL, creatinine 0.7 mg/dL, glycemia 64 mg/dL, AST 103 U/L, total
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Fig. 1. Endoscopic view of the esophagus showing the organ circumferentially blackened and friable, with cotton-like pinpoint spots in the upper third.
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Fig. 3. Biopsy of the esophagus shown in Fig. 1. Gomori methenamine silver nitrate stain shows pseudohyphae and ovoid spores in necrosis suggesting Candida sp. (original magnification ¥ 40).
the duodenal bulb on upper and lower walls. An esophageal biopsy demonstrated necrosis of the mucosa and of the lamina propria. The patient was given i.v. fluid replacement and infusion of a red blood cell concentrate and omeprazol, conventional treatment for hepatic encephalopathy, was started. On the sixth day of hospitalization, a new endoscopy revealed partial regression of the blackened aspect of the esophagus in the lower two thirds, as well as hypertensive gastropathy and active duodenal ulcers. Clinical and laboratory improvement occurred and the patient was discharged on the 12th day for ambulatory follow up. Sixty days later he was re-hospitalized with hepatic encephalopathy and died 20 days later due to hepatic insufficiency. Case 3 Fig. 2. Biopsy of the esophagus shown in Fig. 1. Esophageal necrosis. No stratified squamous cells are seen. (HE; original magnification ¥ 20).
bilirubin 5.4 mg/dL, INR 2.2, and negative to anti-HIV, to anti-HCV antibodies and to HbsAg. Endoscopy revealed edematous esophagitis, hiatus hernia, enanthematous pangastritis with antral erosions, a healed gastric ulcer in the prepyloric region, erosive duodenitis, a healed duodenal ulcer, and angiodysplasia in the antrum and on the anterosuperior wall of the duodenal bulb (hemostasis was obtained with 50% glucose and epinephrine 1 : 1000). The initial treatment consisted of i.v. fluid replacement and a red blood cell concentrate. On the third day of hospitalization, the patient presented melena, mental confusion and flapping. A second endoscopy was performed, revealing a blackened and thickened esophagus throughout the extension of the organ, a healed gastric ulcer in the antrum, and two active ulcers in
An 83-year-old diabetic woman with pleural and peritoneal carcinomatosis due to an ovarian neoplasia was admitted to University Hospital, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Brazil with a history of hematemesis of 12 h duration. Physical examination revealed the patient to be dehydrated, pale, eupneic, with a pulse of 114 b.p.m., arterial pressure of 80/40 mmHg, and a large volume of abdominal ascites. Laboratory tests revealed hemoglobin 11.3 g/dL, hematocrit 33%, leukocytes 5400 cells/mm3, platelets 119 000/mL, sodium 154 mEq/L, potassium 3.6 mEq/L, urea 241 mg/dL, creatinine 3.2 mg/dL, glycemia 156 mg/dL, INR 1.5. Endoscopy revealed a blackened esophagus in the proximal two thirds, with superficial bleeding, multiple plane confluent erosions on all walls, hiatus hernia, hemorrhagic pangastritis, and five deep ulcers with thick fibrin distributed throughout all the walls of the duodenal bulb, preventing the passage of the endoscope to the second duodenal portion. Treatment consisted of clinical support measures and the patient died 3 days later due to multiple organ failure.
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DISCUSSION Acute esophageal necrosis is a condition seldom reported in the literature. The first endoscopic description was reported by Goldenberg et al. in 1990,1 although post-mortem studies had already been reported.2–6 In those studies, the frequency ranged from 0 to 10.3%,7–9 while in endoscopic studies the frequency ranged from 0.01% to 0.2%.10–12 The main clinical manifestation of this entity is hemorrhage of the gastrointestinal tract, but the patients may also present abdominal pain, nausea and vomiting,10–12 dysphagia5,6,13,14 or anemia.12 There is a predominance of elderly male patients, with an age range of 23–86 years.11,12,15 The diagnosis is based on typical endoscopic appearance and on histopathological findings after the exclusion of ingestion of corrosive substances. Other conditions that should be considered in the differential diagnosis are melanosis, pseudomelanosis, acanthosis nigricans,16 melanoma17 and exposure to coal.18,19 These conditions can be excluded by the absence of true necrosis and ulceration detected by histopathological examination. The etiology of AEN remains undefined and is probably multifactorial. Despite the extensive vascularization of the esophagus,20,21 most investigators suggest an ischemic origin of AEN. The preferential location of the lesion is the distal segment of the esophagus, which has been shown to be less vascularized in anatomical studies22 and by angiographic examination.23 In addition, this disease usually develops in elderly patients with associated cardiovascular diseases1,7,9,11,12,24,25 and in states of low flow10–12,26,27 which are more vulnerable to ischemic injury. Finally, the histopathological finding of vascular thrombosis4,10,12,28 and the association with antiphospholipid syndrome28 support this hypothesis. Gastroesophageal reflux secondary to gastric emptying difficulties, as is the case for patients with gastric volvulus, duodenal ulcer, pylorus obstruction or a gastric tube1,10,29,30 is another possible etiology of AEN. In this case, the esophageal mucosa of the distal segment is exposed to large amounts of reflux material, such as H+ ions, bile salts, pepsin and trypsin, which reduce the defense mechanisms of the esophageal mucosa.31–34 Other possible etiologies are mucosal hypersensitivity reaction associated with erythema multiforme and ingestion of medications,13,35,36 trauma due to nasogastric tube insertion1 and herpes infection.14 Frequently associated clinical conditions are postoperative situations,1,5,10,24,25 hypothermia,4,37 neoplasias,9–12,14,23 esophageal candidiasis,12 hyperglycemia,10–12,14,24 malnutrition10–12 and hepatic disease.7,12,27,38 There is no endoscopic treatment for this condition. Clinical therapy in order to prevent an increase in the mucosal lesion is based on the treatment of the main disease,11,14,29 appropriate hydration11,12,29 and administration of antacids, of an H2 receptor antagonist, or a proton pump inhibitor.10–12,14 Sucralfate may also be used11 and parenteral nutrition may be given when necessary.12,24 The prognosis for AEN patients is variable. Mortality is 35–50% and depends on the severity of the associated clinical conditions.9–11 The major complication is stenosis of the esophagus, which occurs in approximately 15% of patients,12 requiring repeated sessions of esophageal dilatation.1,10,11,31 A mediastinal abscess has also been reported.10 Esophagectomy may also be necessary in cases of massive necrosis of the
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organ and of esophageal stenosis that does not respond to endoscopic dilatation.1,10 In conclusion, AEN should be part of the differential endoscopic diagnosis of critically ill patients submitted to upper gastrointestinal endoscopy.
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