Acute esophageal necrosis: possible association with terlipressin

A 75-year-old man was admitted to our department with abdominal pain, hematemesis, and melena. His significant medical history included erosive gastritis, alcohol-related chronic liver disease, and chronic pancreatitis. He was not receiving any medication. His blood pressure was low (80/50 mmHg); results of laboratory testing showed macrocytic anemia and liver dysfunction (hemoglobin 11.8 g/dL, mean cell volume [MCV] 106.4 fL, international normalized ratio [INR] 1.53). After a second episode of hematemesis, his hemoglobin dropped to 8.9 g/dL and he was treated by infusion of a colloidal solution, two units of packed red blood cells, a proton pump inhibitor, and terlipressin (2 mg every 4 hours).

Endoscopy showed a black mucosa ([Fig. 1 a]) that started from the upper esophagus and ended abruptly at the cardia. At that level, we identified an ulcer extending circumferentially in which there was a large exposed vessel ([Fig. 1 b]), which was treated by application of a Hemoclip. The stomach and duodenum were intact. Brushings were negative for cytomegalovirus. Broad-spectrum antibiotics, antifibrinolytic drugs, and parenteral nutrition were commenced; terlipressin was stopped.

Fig. 1 Endoscopic views in a 75-year-old man with hematemesis and melena showing: a black mucosa consistent with acute esophageal necrosis; b an exposed vessel in an ulcer at the cardia.

Endoscopy at day 8 showed a clear margin between the intact proximal esophagus and its lower portion ([Fig. 2 a]). The luminal circumference decreased craniocaudally, ending in a stricture at the cardia ([Fig. 2 b]). At day 16, the distal esophagus appeared stenotic but was passable and enteral nutrition was resumed.

Fig. 2 Views during a second endoscopy performed after 8 days showing: a a clear margin between the intact proximal esophagus and the damaged middle third of the esophagus; b a stricture of the cardia.

The patient was discharged 25 days after admission. A month later, endoscopy revealed almost complete restoration of the mucosa. Notably, at the cardia we observed a Schatzki ring ([Fig. 3]). A further endoscopy 8 months later showed no abnormal esophageal findings.

Fig. 3 View of the lower esophagus during an endoscopy performed a month after discharge showing a Schatzki ring.

Acute esophageal necrosis is characterized by a circumferential mucosal blackening involving the distal esophagus and occasionally extending upstream that stops abruptly at the gastroesophageal junction [1]. Ulceration of the cardia, as in this case, is uncommon; however, similar cases have been reported [2].

Ischemia, impaired mucosal defenses, and chemical insult seem to contribute to its pathogenesis [3]. The distal esophagus has been shown to be less vascularized in angiographic studies [2] [3], arguably making it susceptible to local hypoperfusion caused by low splanchnic blood flow. In the case described, such a state could have resulted from hemorrhage and hypotension.

Furthermore, because of the signs of liver dysfunction and the history of alcohol abuse, which suggested variceal bleeding, the patient received terlipressin, a splanchnic vasoconstrictor that may have reduced microcirculatory perfusion, further contributing to the local ischemia [4]. Although cutaneous necrosis following terlipressin treatment has been reported [5], this is the first reported case of a possible association with acute esophageal necrosis.

Endoscopy_UCTN_Code_CCL_1AB_2AC_3AH

• References

• 1 Moretó M, Ojembarrena E, Zaballa M et al. Idiopathic acute esophageal necrosis: not necessarily a terminal event. Endoscopy 1993; 25: 534-538
• 2 Burtally A, Gregoire P. Acute esophageal necrosis and low-flow state. Can J Gastroenterol 2007; 21: 245-247
• 3 Gurvits GE. Black esophagus: acute esophageal necrosis syndrome. World J Gastroenterol 2010; 16: 3219-3225
• 4 Asfar P, Bracht H, Radermacher P. Impact of vasopressin analogues on the gut mucosal microcirculation. Best Pract Res Clin Anaesthesiol 2008; 22: 351-358
• 5 Lu YY, Wei KC, Wu CS. Terlipressin-induced extensive skin necrosis: a case report and published work review. J Dermatol 2012; 39: 866-868