Acute oesophageal necrosis: an important differential in the elderly population with haematemesis

Abstract

Acute oesophageal necrosis is a rare cause of haematemesis associated with high mortality and morbidity in elderly patients with multiple comorbidities. Acute oesophageal necrosis is thought to be caused by a combination of hypoperfusion, vascular disease and duodenal disease causing transient gastric outlet obstruction and therefore reflux of gastric contents. The subsequent necrosis is associated with significant morbidity and mortality. We present a case of an 83-year-old man presenting with sepsis secondary to gallstone cholangitis, who developed haematemesis 2 days post admission. Oesopho-gastro-duodenoscopy demonstrated necrosis to the oesophagus and duodenal ulceration. This was the first case of acute oesophageal necrosis observed within our hospital. We review the literature on the management of acute oesophageal necrosis and discuss the impact of acute oesophageal necrosis and its complications on the patient’s long-term outcome.

Background

Acute oesophageal necrosis (AON), also known as necrotising oesophagitis and black oesophagus, was first described by Goldenberg in 1990.¹ Gurvits² then formulated the distinct clinical ‘Gurvits syndrome’ in 2007. It is a rare syndrome with an estimated prevalence of 0.2%, with only around 100 cases reported in the literature.^2–4

AON predominantly presents with an episode of haematemesis in a patient who has presented to hospital with a preceding clinical event such as sepsis.² The underlying pathophysiology of AON is thought to be multifactorial involving hypotension and pre-existing vascular disease resulting in predisposition to necrosis, impaired mucosal healing and severe reflux of gastric contents inflicting mucosal injury.^{2 5} In Gurvitis’ 2014⁶ multicentre case review, it was noted that 90% of patients had tachycardia and hypotension, preceding diagnosis, which supports the theory of necrosis happening secondary to ischaemia in the vulnerable distal oesophagus. Interestingly, it was also noted that duodenal disease was linked to more extensive AON in the oesophagus which could result in transient gastric outlet obstruction increasing gastro-oesophageal reflux.⁶ The proposed pathophysiology of reflux of gastric contents is further supported by Davide et al⁷ finding three cases in Zollinger-Ellison syndrome. Therefore, it predominantly effects elderly men, with multiple comorbidities.

We report the first case observed within our hospital, to highlight the need to consider AON as a differential diagnosis in elderly men with multiple comorbidities experiencing haematemesis.

Case presentation

An 83-year-old man with a background of type 2 diabetes mellitus presented to A&E with confusion and fever. On examination, he was generally unwell, but remained normotensive with a baseline blood pressure of 150 mmHg systolic. Initial investigations revealed elevated inflammatory markers and significantly deranged liver function tests. Of note, alkaline phosphatase was 1268 U/L, bilirubin 90 µmol/L and alanine transferase 290 U/L. He was initiated on broad-spectrum antimicrobial therapy and admitted under gastroenterology with suspected ascending cholangitis. A CT scan of the abdomen and pelvis revealed a distended common bile duct (CBD) of 9 mm with multiple calculi within the gallbladder and CBD. This finding supported the admission diagnosis; therefore, antimicrobial therapy was continued and an endoscopic retrograde cholangiopancreatography was requested.

Unfortunately, his condition deteriorated prior to undergoing the procedure. Two days post admission, he began to experience intermittent vomiting of gastric contents, subsequently followed by coffee ground haematemesis with an absence of melena. Throughout this physical deterioration, he was tachycardic and his systolic blood pressure fluctuated between 120 mmHg and 150 mmHg. His repeat biochemistry showed a significant deterioration in inflammatory markers (white cell count increased from 10.0×10⁹/L to 28.8×10⁹/L), liver function tests (bilirubin increased from 44 µmol/L to 128 µmol/L) and urea (7.0 mmol/L to 16 mmol/L); haemoglobin reduced from 111 g/L to 83 g/L 4 days following the episode of haematemesis, but stabilised without requiring a blood transfusion.

Antibiotic treatment was escalated and intravenous proton pump inhibitor was initiated and he was made nil by mouth (NBM) in preparation for an oesophago-gastro-duodenosocopy (OGD) to investigate the cause of the vomiting.

OGD revealed a black discolouration of the distal oesophagus with deep black ulcers and diffuse oozing of blood with a sharp distal transition to normal mucosa at the gastro-oesophageal junction (figure 1). In addition, multiple Forrest III, duodenal ulcers were observed. The OGD demonstrated the haematemesis was secondary to AON rather than the duodenal ulceration as there was active bleeding directly observed in the oesophagus whereas the duodenal ulceration showed evidence of healing. No biopsies were taken during the procedure due to concerns of causing oesophageal perforation. This OGD was diagnostic for AON.

Figure 1.

Initial oesophago-gastro-duodenosocopy showing circumferential blackening of mucosa, diagnostic for acute oesophageal necrosis.

Treatment

On the basis of the diagnosis of AON, conservative management was initiated. This included increasing the dose of intravenous proton pump inhibitor to 40 mg three times a day and the patient remaining NBM. Due to the requirement for the patient to remain NBM for a prolonged time period, a nasojejunal tube was inserted endoscopically to allow enteral feeding. Of note, albumin was low throughout the admission.

A further OGD was performed on day 12 to identify whether the mucosa had healed, as the literature reports normal oesophageal mucosa can be observed as early as 1 to 2 weeks post diagnosis,⁵ and whether oral nutrition could be commenced, an important factor for patient comfort. On endoscopy, the mucosa showed partial healing (figure 2). Oral hydration with clear fluids was initiated and well tolerated; therefore, he advanced to a liquid diet. This was deemed clinically appropriate but also had a significant impact on improving patient morale during a prolonged hospital admission with limited visitation due to the COVID-19 pandemic.

Figure 2.

Oesophago-gastro-duodenosocopy showing partial healing of the oesophageal mucosa 12 days post diagnosis.

Outcome

Following 6 days of oral feeding, the patient developed vomiting of gastric contents. OGD revealed a distal oesophageal stricture not passable with the endoscope. Therefore, a nasogastric tube was sited to allow nutrition while the patient awaited a radiologically inserted gastrostomy (RIG) for long-term feeding. The patient was discharged home following RIG placement, but the hospital admission had resulted in a general deterioration in the patient’s frailty resulting in increased care needs. An outpatient barium swallow confirmed the presence of a 3 cm distal oesophageal stricture which was scheduled for outpatient endoscopic dilatation, 8 weeks post discharge to allow complete healing of ulceration.

Discussion

AON is a rare condition with predominance in elderly men with multiple comorbidities, most commonly diabetes mellitus and hypertension. Both present in the case described. Diagnosis is made endoscopically on observation of blackening of the oesophageal mucosa abruptly ending at the z-line in the absence of ingestion of corrosive materials. The distal portion of the oesophagus is most frequently affected due to increased vulnerability from the absence of collateral vascular supply increasing the risk of watershed infarcts; however, AON has been observed in all regions of the oesophagus.^{2 8} Biopsy can be considered to exclude differential diagnosis such as infection, acanthosis nigricans and melanoma, but is often avoided due to concerns of oesophageal perforation which is associated with increased mortality.^{2 5 6}

Clinical presentation is almost universally haematemesis, as in the case described,² although incidental cases on endoscopy have been reported.⁷ Case reports in the literature report a wide range of preceding clinical events: acute cardiac event, sepsis, abdominal pain and acute limb ischaemia.2–4 9 10 AON has also been observed in critically ill patients in the intensive care unit with diabetic ketoacidosis, thoracic aortic aneurysm and following trauma.^11–13

The aetiology of the necrosis in the case described is not obvious as overt hypotension was not observed; however, his systolic blood pressure did reduce substantially which could have contributed to the ischaemic changes, and alternatively he could have experienced unrecorded transient hypotension. In addition, he had duodenal disease which is associated with severe AON, but gastric outlet obstruction was not observed on OGD.

Management across the literature supports treatment of underlying conditions and conservative management with the use of intravenous proton pump inhibitors, fluid resuscitation, NBM and glycaemic control. Antibiotic use is controversial and only recommended when there is evidence of superimposed infection.^{4 6} With regards to nutrition, it is advised malnourished patients receive parenteral nutrition to aid mucosal healing and that nasogastric tube placement should be avoided due to perforation risk; however, this can be mitigated through endoscopic guidance permitting the use of enteral nutrition.^{2 8 14} In the case we have described, it was felt nutrition was an important aspect in patient management considering the link between AON, low albumin and therefore malnutrition.⁶

AON has a high mortality rate of 32%, largely due to underlying pathologies, with AON specific mortality at 6%. It also has a significant morbidity rate with 7% developing oesophageal perforation and 10% oesophageal strictures.² There is limited evidence to suggest a patient is more likely to develop stricture formation if they have duodenal disease; this is supported in the case we have described.⁶ Specific patient outcomes in the literature vary from a 72-year-old man who developed AON following admission with septic cellulitis showing significant improvement following 2 weeks of conservative treatment including oral nutrition.¹² However, conservatively managed AON was fatal in a patient who presented with an unruptured thoracic aortic aneurysm, but prior to development of AON, the patient was already requiring intensive care support and had developed other complications of hospital admission such as pulmonary embolism.⁹

This case highlights the morbidity associated with AON through the general deterioration of the patient during the hospital admission, and despite recovering from AON, it has resulted in life-changing complications particularly with regards to mobility and nutrition.

Learning points.

• Acute oesophageal necrosis should be considered as a differential diagnosis in the multi-comorbid geriatric male population experiencing haematemesis.

• Nutrition is an important aspect of management to promote mucosal healing.

• Nasogastric/jejunal tube insertion should be performed under endoscopic guidance to reduce risk of perforation.

Ethics statements

Patient consent for publication

Obtained.