- Email: [email protected]
Acute esophageal necrosis associated with gastric volvulus
M Kram, L Gorenstein, D Eisen, et al.
Acute esophageal necrosis associated with gastric volvulus Michael Kram, MD, Lyall Gorenstein, MD, David Eisen, MD, Daniel Cohen, MD
Acute esophageal necrosis associated with gastric volvulus
We report a case of AEN associated with a gastric volvulus and speculate as to its etiology and offer a possible therapeutic alternative for this uncommon disorder. CASE REPORT
There have been several reports of acute esophageal necrosis (AEN) also known as “black esophagus.” It is defined as a dark pigmentation of the esophagus associated with histologic mucosal necrosis. Its cause is unknown, but many etiologies have been proposed including gastric outlet obstruction,1 ischemia,2-4 hypersensitivity to antibiotics,5,6 and viral infections.7 To our knowledge an association with gastric volvulus has not been described. From the Departments of Gastroenterology, Thoracic Surgery, Pathology and Radiology, Nyack Hospital, Nyack, New York. Reprint requests: Michael Kram, MD, Gastrointestinal Associates of Rockland, 500 New Hempstead Road, New City, NY 10956. Copyright © 2000 by the American Society for Gastrointestinal Endoscopy 0016-5107/2000/$12.00 + 0 37/4/104657 doi:10.1067/mge.2000.104657 610
GASTROINTESTINAL ENDOSCOPY
The patient was a 75-year-old woman without antecedent medical problems. She had been told of a hiatal hernia in the past. She presented to the emergency room with a 1-day history of vomiting “coffee ground” material. On admission her vital signs were normal and her abdomen was soft and nontender. Her admission hemoglobin was 10 mg/dL (normal 14.0-18.0 mg/dL). An urgent upper endoscopy revealed that a significant portion of the lower one third of her esophagus was black (Fig. 1); the proximal two thirds of the esophagus looked normal. There also appeared to be a large hiatal hernia and endoscopically a gastric volvulus was appreciated. The duodenum was intubated, and there were no other abnormalities present to account for GI bleeding. An upper GI series performed immediately after endoscopy revealed gastric volvulus, most likely of mesentero-axial orientation. The distal esophageal folds were thickened and irregular (Fig. 2). VOLUME 51, NO. 5, 2000
Acute esophageal necrosis associated with gastric volvulus
M Kram, L Gorenstein, D Eisen, et al.
Figure 1. Endoscopic view of black-appearing distal esophageal mucosa.
Figure 3. Photomicrograph showing esophageal necrosis involving the mucosa and submucosa. The deeper tissues and muscular layer are normal (H&E, orig. mag. ×40).
Figure 2. Upper GI x-ray series showing gastric volvulus. Laparotomy revealed that the entire stomach had herniated into the mediastinum through the esophageal hiatus. The stomach was reduced and a Nissen fundoplication was performed. The distal esophagus was edematous, but the serosal color was normal and did not appear ischemic. The esophageal tissue was boggy and while placing sutures into the esophagus during the fundoplication, barium from the upper GI series exuded from the esophageal wall. Because of difficulty in visualizing the distal esophagus, it was thought that a distal esophageal resection was necessary. A thoracotomy was then performed, and on inspection the esophageal muscularis propria appeared normal. When the esophagus was divided, the submucosal arteries were patent and bled vigorously; however, the mucosa was black and necrotic. Intraoperative frozen section was obtained and was interpreted as showing mucosal necrosis without the presence of viable squamous epithelium. The esophagus was therefore mobilized for another 5 cm where the mucosa, although discolVOLUME 51, NO. 5, 2000
ored, was not blackened as was the case for the more distal esophagus. At that level a 2-layer anastomosis was performed. On completion of the anastomosis, a flexible endoscope was passed into the esophagus to confirm mucosal viability at the anastomosis. External examination of the esophageal specimen (8 × 4 × 1 cm) showed the adventitial surface to be grossly unremarkable. On opening, the mucosal surface was entirely black without the presence of mass lesions. Multiple permanent histologic sections demonstrated similar findings with complete superficial necrosis involving the mucosa and submucosa (Fig. 3). No viable squamous epithelium was identified. The muscularis propria and deeper tissues of the esophagus were evaluated as being viable. Her postoperative course was complicated by aspiration pneumonia and adult respiratory distress syndrome. She required several weeks of ventilatory support, but then she made a full recovery. The esophagogastric anastomosis healed without incident.
DISCUSSION AEN is a rare event. Often described as the black esophagus, AEN must be distinguished from other entities that can give the appearance endoscopically of a black esophagus such as melanosis,8 malignant melanoma,9 pseudomelanosis,10 and acanthosis GASTROINTESTINAL ENDOSCOPY
611
M Kram, L Gorenstein, D Eisen, et al.
nigricans.11 Other entities that can produce a similar appearance include ingestion of caustic substances and local necrosis secondary to various infections. Most cases of AEN have no known etiology, although ischemia, nasogastric tube trauma, infections and gastric outlet obstruction have been listed as causes.1 Other underlining conditions such as hyperglycemia, hypoxia and carcinoma have also been associated with AEN.8 Lacy et al.1 reported on 2 cases of AEN and reviewed cases published to date. Of 25 cases of AEN, 4 had an obvious cause that related to compromised blood supply to the esophagus. In the other 21 cases with no obvious cause, these investigators believed that a precipitating event was the development of an acute gastric outlet obstruction. They postulated that this transient outlet obstruction may result in the accumulation of large volumes of gastric secretions that continually bathe the esophagus for prolonged periods of time, thereby overwhelming the normal defense mechanisms of the esophagus, resulting in eventual mucosal necrosis. To our knowledge, our case is the first described association of a gastric volvulus with AEN. Gastric volvulus produces acute outlet obstruction, allowing the esophageal mucosa to be subjected to prolonged contact with acid and pepsin. In addition, the intraoperative and histologic findings of normal tissue in the deeper layers of the esophagus and the intraoperative finding of patent submucosal vessels indicate that mucosal necrosis resulted from a local insult, rather than occlusion of the main esophageal arterial branches. There are no prospective data available on the treatment of AEN. Management options include treatment of any underlying disorder, adequate hydration, H2 blockers or proton pump inhibitors, sucralfate, nasogastric tube intubation, urgent endoscopy, and use of antibiotics.1 In the majority of cases, esophageal necrosis appears to resolve without any long-term sequelae. Approximately 25% of patients treated conservatively develop long-term complications such as esophageal stricture. One third of patients with AEN die, either due to their
612
GASTROINTESTINAL ENDOSCOPY
Acute esophageal necrosis associated with gastric volvulus
underlying illness or as a direct result of this disorder.1 Surgical resection has been mentioned in only 1 previous case, where full-thickness esophageal necrosis was documented and was accompanied by colonic interposition.12 Given the high morbidity and mortality of this uncommon disorder and based on the outcome of our case, we believe that perhaps a more aggressive approach to this disorder should be undertaken. We recommend consideration of a gastric volvulus as a possible cause of black esophagus and an upper GI series on an urgent basis if the endoscopic findings raise any suspicion of this entity. Repair of the volvulus and esophageal resection would then be necessary. As demonstrated in our case, gastroesophageal continuity can be safely reestablished, provided there is no gross mediastinal contamination. REFERENCES 1. Lacy BE, Toor A, Bensen SP, Rothstein RI, Maheshwari Y. Acute esophageal necrosis: report of two cases and a review of the literature. Gastrointest Endosc 1999;49:527-32. 2. Lee KR, Stark E, Shaw FE. Esophageal infarction complicating spontaneous rupture of the thoracic aorta. JAMA 1977;237: 1233-4. 3. Cappell MS. Esophageal necrosis and perforation associated with the anticardiolipin syndrome. Am J Gastroenterol 1994; 89:1241-5. 4. Goldenberg SP, Wain SL, Marignani P. Acute necrotizing esophagitis. Gastroenterology 1990;98:493-6. 5. Mangan TF, Thompson Colley A, Wytock DH. Antibiotic-associated acute necrotizing esophagitis [letter]. Gastroenterology 1990;99:900. 6. Kikendall JW, Friedman AC, Oyewole MA, Fleischer D, Johnson LF. Pill-induced esophageal injury: case reports and review of the medical literature. Dig Dis Sci 1983;28:174-82. 7. Cattan P, Cuillerier E, Carnot F, Landi B, Dusoleil A, Barbier JP. Black esophagus associated with herpes esophagitis. Gastrointest Endosc 1999;49:105-7. 8. Sharma S, Venkateswaran S, Chacko A, Mathan M. Melanosis of the esophagus. Gastroenterology 1991;100:13-6. 9. Raven RW, Dawson I. Malignant melanoma of the esophagus. Br J Surg 1964;51:551-5. 10. Kimball MK. Pseudomelanosis of the esophagus. Gastrointest Endosc 1978;23:121-2. 11. Geller A, Aguilar H, Burgart L, Gostout CJ. The black esophagus. Am J Gastroenterol 1995;90:2210-2. 12. Moreto M, Ojembarrena E, Zaballa M, Tanago JG, Ibanez S. Idiopathic acute esophageal necrosis: not necessarily a terminal event. Endoscopy 1993;25:534-8.
VOLUME 51, NO. 5, 2000
Acute esophageal necrosis associated with gastric volvulus Michael Kram, MD, Lyall Gorenstein, MD, David Eisen, MD, Daniel Cohen, MD
Acute esophageal necrosis associated with gastric volvulus
We report a case of AEN associated with a gastric volvulus and speculate as to its etiology and offer a possible therapeutic alternative for this uncommon disorder. CASE REPORT
There have been several reports of acute esophageal necrosis (AEN) also known as “black esophagus.” It is defined as a dark pigmentation of the esophagus associated with histologic mucosal necrosis. Its cause is unknown, but many etiologies have been proposed including gastric outlet obstruction,1 ischemia,2-4 hypersensitivity to antibiotics,5,6 and viral infections.7 To our knowledge an association with gastric volvulus has not been described. From the Departments of Gastroenterology, Thoracic Surgery, Pathology and Radiology, Nyack Hospital, Nyack, New York. Reprint requests: Michael Kram, MD, Gastrointestinal Associates of Rockland, 500 New Hempstead Road, New City, NY 10956. Copyright © 2000 by the American Society for Gastrointestinal Endoscopy 0016-5107/2000/$12.00 + 0 37/4/104657 doi:10.1067/mge.2000.104657 610
GASTROINTESTINAL ENDOSCOPY
The patient was a 75-year-old woman without antecedent medical problems. She had been told of a hiatal hernia in the past. She presented to the emergency room with a 1-day history of vomiting “coffee ground” material. On admission her vital signs were normal and her abdomen was soft and nontender. Her admission hemoglobin was 10 mg/dL (normal 14.0-18.0 mg/dL). An urgent upper endoscopy revealed that a significant portion of the lower one third of her esophagus was black (Fig. 1); the proximal two thirds of the esophagus looked normal. There also appeared to be a large hiatal hernia and endoscopically a gastric volvulus was appreciated. The duodenum was intubated, and there were no other abnormalities present to account for GI bleeding. An upper GI series performed immediately after endoscopy revealed gastric volvulus, most likely of mesentero-axial orientation. The distal esophageal folds were thickened and irregular (Fig. 2). VOLUME 51, NO. 5, 2000
Acute esophageal necrosis associated with gastric volvulus
M Kram, L Gorenstein, D Eisen, et al.
Figure 1. Endoscopic view of black-appearing distal esophageal mucosa.
Figure 3. Photomicrograph showing esophageal necrosis involving the mucosa and submucosa. The deeper tissues and muscular layer are normal (H&E, orig. mag. ×40).
Figure 2. Upper GI x-ray series showing gastric volvulus. Laparotomy revealed that the entire stomach had herniated into the mediastinum through the esophageal hiatus. The stomach was reduced and a Nissen fundoplication was performed. The distal esophagus was edematous, but the serosal color was normal and did not appear ischemic. The esophageal tissue was boggy and while placing sutures into the esophagus during the fundoplication, barium from the upper GI series exuded from the esophageal wall. Because of difficulty in visualizing the distal esophagus, it was thought that a distal esophageal resection was necessary. A thoracotomy was then performed, and on inspection the esophageal muscularis propria appeared normal. When the esophagus was divided, the submucosal arteries were patent and bled vigorously; however, the mucosa was black and necrotic. Intraoperative frozen section was obtained and was interpreted as showing mucosal necrosis without the presence of viable squamous epithelium. The esophagus was therefore mobilized for another 5 cm where the mucosa, although discolVOLUME 51, NO. 5, 2000
ored, was not blackened as was the case for the more distal esophagus. At that level a 2-layer anastomosis was performed. On completion of the anastomosis, a flexible endoscope was passed into the esophagus to confirm mucosal viability at the anastomosis. External examination of the esophageal specimen (8 × 4 × 1 cm) showed the adventitial surface to be grossly unremarkable. On opening, the mucosal surface was entirely black without the presence of mass lesions. Multiple permanent histologic sections demonstrated similar findings with complete superficial necrosis involving the mucosa and submucosa (Fig. 3). No viable squamous epithelium was identified. The muscularis propria and deeper tissues of the esophagus were evaluated as being viable. Her postoperative course was complicated by aspiration pneumonia and adult respiratory distress syndrome. She required several weeks of ventilatory support, but then she made a full recovery. The esophagogastric anastomosis healed without incident.
DISCUSSION AEN is a rare event. Often described as the black esophagus, AEN must be distinguished from other entities that can give the appearance endoscopically of a black esophagus such as melanosis,8 malignant melanoma,9 pseudomelanosis,10 and acanthosis GASTROINTESTINAL ENDOSCOPY
611
M Kram, L Gorenstein, D Eisen, et al.
nigricans.11 Other entities that can produce a similar appearance include ingestion of caustic substances and local necrosis secondary to various infections. Most cases of AEN have no known etiology, although ischemia, nasogastric tube trauma, infections and gastric outlet obstruction have been listed as causes.1 Other underlining conditions such as hyperglycemia, hypoxia and carcinoma have also been associated with AEN.8 Lacy et al.1 reported on 2 cases of AEN and reviewed cases published to date. Of 25 cases of AEN, 4 had an obvious cause that related to compromised blood supply to the esophagus. In the other 21 cases with no obvious cause, these investigators believed that a precipitating event was the development of an acute gastric outlet obstruction. They postulated that this transient outlet obstruction may result in the accumulation of large volumes of gastric secretions that continually bathe the esophagus for prolonged periods of time, thereby overwhelming the normal defense mechanisms of the esophagus, resulting in eventual mucosal necrosis. To our knowledge, our case is the first described association of a gastric volvulus with AEN. Gastric volvulus produces acute outlet obstruction, allowing the esophageal mucosa to be subjected to prolonged contact with acid and pepsin. In addition, the intraoperative and histologic findings of normal tissue in the deeper layers of the esophagus and the intraoperative finding of patent submucosal vessels indicate that mucosal necrosis resulted from a local insult, rather than occlusion of the main esophageal arterial branches. There are no prospective data available on the treatment of AEN. Management options include treatment of any underlying disorder, adequate hydration, H2 blockers or proton pump inhibitors, sucralfate, nasogastric tube intubation, urgent endoscopy, and use of antibiotics.1 In the majority of cases, esophageal necrosis appears to resolve without any long-term sequelae. Approximately 25% of patients treated conservatively develop long-term complications such as esophageal stricture. One third of patients with AEN die, either due to their
612
GASTROINTESTINAL ENDOSCOPY
Acute esophageal necrosis associated with gastric volvulus
underlying illness or as a direct result of this disorder.1 Surgical resection has been mentioned in only 1 previous case, where full-thickness esophageal necrosis was documented and was accompanied by colonic interposition.12 Given the high morbidity and mortality of this uncommon disorder and based on the outcome of our case, we believe that perhaps a more aggressive approach to this disorder should be undertaken. We recommend consideration of a gastric volvulus as a possible cause of black esophagus and an upper GI series on an urgent basis if the endoscopic findings raise any suspicion of this entity. Repair of the volvulus and esophageal resection would then be necessary. As demonstrated in our case, gastroesophageal continuity can be safely reestablished, provided there is no gross mediastinal contamination. REFERENCES 1. Lacy BE, Toor A, Bensen SP, Rothstein RI, Maheshwari Y. Acute esophageal necrosis: report of two cases and a review of the literature. Gastrointest Endosc 1999;49:527-32. 2. Lee KR, Stark E, Shaw FE. Esophageal infarction complicating spontaneous rupture of the thoracic aorta. JAMA 1977;237: 1233-4. 3. Cappell MS. Esophageal necrosis and perforation associated with the anticardiolipin syndrome. Am J Gastroenterol 1994; 89:1241-5. 4. Goldenberg SP, Wain SL, Marignani P. Acute necrotizing esophagitis. Gastroenterology 1990;98:493-6. 5. Mangan TF, Thompson Colley A, Wytock DH. Antibiotic-associated acute necrotizing esophagitis [letter]. Gastroenterology 1990;99:900. 6. Kikendall JW, Friedman AC, Oyewole MA, Fleischer D, Johnson LF. Pill-induced esophageal injury: case reports and review of the medical literature. Dig Dis Sci 1983;28:174-82. 7. Cattan P, Cuillerier E, Carnot F, Landi B, Dusoleil A, Barbier JP. Black esophagus associated with herpes esophagitis. Gastrointest Endosc 1999;49:105-7. 8. Sharma S, Venkateswaran S, Chacko A, Mathan M. Melanosis of the esophagus. Gastroenterology 1991;100:13-6. 9. Raven RW, Dawson I. Malignant melanoma of the esophagus. Br J Surg 1964;51:551-5. 10. Kimball MK. Pseudomelanosis of the esophagus. Gastrointest Endosc 1978;23:121-2. 11. Geller A, Aguilar H, Burgart L, Gostout CJ. The black esophagus. Am J Gastroenterol 1995;90:2210-2. 12. Moreto M, Ojembarrena E, Zaballa M, Tanago JG, Ibanez S. Idiopathic acute esophageal necrosis: not necessarily a terminal event. Endoscopy 1993;25:534-8.
VOLUME 51, NO. 5, 2000