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Decide on the Spot

A not-so-black-and-white case of gastrointestinal bleeding

July 10, 2018 | Spyros Siakavellas, Konstantinos Papaxoinis and George Papatheodoridis

A 60-year-old woman presents at the Emergency Department complaining that she has been passing black, tarry stool since yesterday and had an episode of coffee-ground emesis some hours ago. It is the first time she has noticed these kinds of symptoms. Moreover, she reports episodes of epigastric pain on and off during the past week.

The patient has never undergone endoscopy. Her medical history includes diabetes mellitus, hypertension, hyperlipidaemia, gastro-oesophageal reflux disease (GORD), osteoarthritis and alcohol abuse. She admits that she occasionally uses nonsteroidal anti-inflammatory drugs (NSAIDs) to cope with episodes of pain caused by her osteoarthritis and that she took some in the past week.

On physical examination she is tachycardic (92 beats per minute) and hypotensive (82/57 mm Hg), but afebrile and her oxygen saturation level is normal. Her abdomen is mildly distended, with some tenderness during deep palpation and increased bowel sounds. Her blood test results at presentation are shown in Table 1. A variceal bleed was suspected, and an emergency upper gastrointestinal endoscopy was performed (see video).

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Case Question 1

What is your clinical diagnosis?

A. Oesophageal melanoma

B. Oesophageal infection (e.g. CMV, HSV, Candidiasis)

C. Acanthosis nigricans

D. Acute oesophageal necrosis (AEN)

  • Case question 1 answer and discussion
Case question 1 answer and discussion

Correct answer: D.

Discussion

Acute oesophageal necrosis (AEN), more frequently known as black oesophagus, was first described in 1990.1It has been also described as Gurvits syndrome, after the physician whodescribed itin depthand studied it thoroughly.2As seen in the case patient, AEN is characterized by the conspicuous endoscopic finding of diffuse and circumferential black ulcero-necrotic oesophageal mucosa, almost always involving the distal oesophagus and with an abrupt demarcation line at the gastro-oesophageal junction.3

Biopsy samples are not required for confirmation of diagnosis unless there is a high degree of suspicion for the presence of atypical forms of infection (e.g. CMV or HSV oesophagitis). When biopsy samples are taken tissue necrosis of variable depth is noted, with accompanying ulcerations and deposits of hemosiderin.4

AEN is a rare entity, with an incidence of only 0.1–0.28%, being encountered most frequently in the older population (mean age 67 years) and with a predilection for the male sex.5Around 220 cases have been presented so far worldwide.2The vast majority of patients (>90%) present with signs of upper gastrointestinal bleeding. Other less frequent symptoms include nausea, fever, dysphagia and abdominal pain.6–8

Case Question 2

Which of the conditions from the patient’s medical history is not associated with their diagnosis?

A. Diabetes mellitus
B. NSAID use
C. Alcohol abuse 
D. Hypoalbuminemia
E. Hypertension

 

  • Case question 2 answer and discussion
Case question 2 answer and discussion

Correct answer: B.

Discussion

The pathogenesis of AEN seems to be multifactorial.8It has been suggested that an event of transient or extended mucosal hypoperfusion (in the setting of underlying vascular disease), in combination with impaired local defence mechanisms, leads to the inability of the tissue to cope with increased exposure to toxic gastric contents (i.e. gastrin and pepsin). Thus, the damage to the mucosa is devastating, leading to variable degrees of necrosis. 

Several medical conditions/situations have been described to contribute to development of AEN:2,5,6

  • Diabetes mellitus (especially ketoacidosis)
  • Hypertension
  • Alcohol abuse
  • Malignancy
  • Malnutrition
  • Sepsis
  • Gastric volvulus or gastric outlet obstruction
  • Immunosuppression
  • Corrosive agent ingestion

Case Question 3

Which of the following measures is not recommended for the management of this patient?

A. Nil per os
B. Aggressive fluid resuscitation
C. Antibiotics 
D. IV acid suppression with PPIs
E. Glycaemic control

 

  • Case question 3 answer and discussion
Case question 3 answer and discussion

Correct answer: C.

Discussion

Although the oesophagus is the organ most obviously affected by this condition, treatment should target the underlying medical conditions and restoring hemodynamic stability. Treatment involves bowel rest, aggressive fluid resuscitation (with blood transfusion as needed) and high-dose IV acid suppression with PPIs to decrease oesophageal acid exposure. In severe cases total parenteral nutrition may be necessary.9 

Use of antibiotics (including antivirals and antifungals) is generally not recommended. Antibiotics may even be detrimental, as cases complicated by C. difficileinfection have shown, unless there is a confirmed diagnosis of, or a high degree of clinical suspicion for, underlying significant infection or sepsis, perforation or the patient is immunocompromised.3Surgery is required only in highly selected cases (e.g. those complicated by perforation, abscess formation or a stricture). Complications are rare, but they usually involve stenosis or stricture formation. Prognosis is poor, with a high mortality rate of 32% due to the severity of the comorbidities present, despite specific AEN-related mortality being quite low at 6%. Treatment should, therefore, be prompt, aggressive and aimed at the conditions that have caused the AEN, to achieve better recovery rates.6

References

  1. Goldenberg SP, Wain SL and Marignani P. Acute necrotizing esophagitis. Gastroenterology 1990; 98: 493–496. https://www.gastrojournal.org/article/0016-5085(90)90844-Q/pdf?referrer=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2F
  2. Manno V, et al. Acute esophageal necrosis (black esophagus): a case report and literature review. Acta Diabetol 2017; 54: 1061–1063. https://link.springer.com/article/10.1007/s00592-017-1028-4
  3. Gurvits GE, et al. Black esophagus: new insights and multicenter international experience in 2014. Dig Dis Sci 2015; 60: 444–453.
  4. Kalva NR, et al. An unusual cause of acute upper gastrointestinal bleeding: acute esophageal necrosis. Case Rep Gastrointest Med 2016; 2016: 6584363. https://www.hindawi.com/journals/crigm/2016/6584363/
  5. Bonaldi M, et al. Black esophagus: acute esophageal necrosis, clinical case and review of literature. J Surg Case Rep 2017; 2017: rjx037. https://academic.oup.com/jscr/article/2017/3/rjx037/3058866
  6. Averbukh LD, Mavilia MG and Gurvits GE. Acute esophageal necrosis: A case series. Cureus 2018; 10: e2391. https://www.cureus.com/articles/11644-acute-esophageal-necrosis-a-case-series
  7. Galanopoulos M, et al. Black esophagus: An uncommon cause of upper gastrointestinal bleeding. Intern Emerg Med 2016; 11: 1019–1020. https://link.springer.com/article/10.1007/s11739-015-1360-z
  8. Choksi V, et al. "Black esophagus" or Gurvits syndrome: A rare complication of diabetic ketoacidosis. Case Rep Gastrointest Med 2017; 2017: 4815752. https://www.hindawi.com/journals/crigm/2017/4815752/
  9. Brar TS, Helton R and Zaidi Z. Total parenteral nutrition successfully treating black esophagus secondary to hypovolemic shock. Case Rep Gastrointest Med 2017; 2017: 4396870. https://www.hindawi.com/journals/crigm/2017/4396870/
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  • About the Authors
About the Authors

Spyros Siakavellas is a Gastroenterology Fellow in the Academic Department of Gastroenterology, Laiko General Hospital of Athens, Greece. In 2018 he joined the UEG E-learning team as a Web Editor. He was trained in medicine and completed his PhD thesis on “The role of the TL1A/DcR3 axis in IBD” at the Medical School of Athens University. He has trained in internal medicine and gastroenterology in the UK and in Greece. His research interests include IBD and hepatology.

Konstantinos Papaxoinis is a Consultant in the Academic Department of Gastroenterology in the Laiko General Hospital of Athens, Greece. He was trained in medicine at the Medical School of Athens University. He has trained in gastroenterology in the UK and in Greece. His research interests include gastrointestinal oncology and capsule endoscopy.

George Papatheodoridis is Professor in Medicine and Gastroenterology at Athens Medical School and Director of the Academic Gastroenterology Department of Laiko Hospital of Athens, Greece. He was trained in medicine and completed his PhD thesis on “Acute on Chronic Hepatitis B” at the Medical School of Athens University. He was trained in gastroenterology at Tzaneion Hospital of Piraeus, Greece, and had a 2-year research fellowship in hepatology at the Royal Free Hospital in London, UK. He has served as member of the EASL Scientific Committee/Governing Board and is a member of EASL and AASLD. He is President of the Hellenic Association for Study of Liver, President of the Hellenic Foundation of Gastroenterology & Nutrition and Chair of Scientific Committee for Viral Hepatitis of the Hellenic Center for Prevention of Diseases. He is also a member of the committee for the Hellenic National Plan for hepatitis C. His main research interest has been focused on viral hepatitis.

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