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Esophageal-atrial perforation due to recurrent esophagitis 18 years after esophageal bypass surgery
Esophageal-atrial perforation due to recurrent esophagitis 18 years after esophageal bypass surgery A 62-year-old man presented with a grand mal seizure, progressive abdominal distention, and refractory hypotension 18 years after colonic bypass of a benign stricture of the low middle third of the esophagus. He died 3 hours after admission to the hospital. The patient had a history of liniment ingestion in childhood plus a long history (If dysphagia and substernal pain. Autopsy disclosed a large ulcer of the anterior wall of the distal esophagus, which had eroded through the posterior wall of the left atrium. Histologic examination revealed chronic esophagitis with fibrous obliteration of the esophageal wall, pericardium, and left atrial myocardium near the site of perforation. Foreign material was present within small arteries of multiple viscera, and in several of these fragments transverse striations were demonstrated. Esophageal-atrial perforation is a rare but fatal complication of chronic esophageal ulceration. The clinical and pathological features of this and previously reported cases of nontraumatic esophageal-atrial perforation are reviewed.
George F. Murphy, M.D.,* A. Kevin Raymond, M.D.,* and J. Gordon Scannell, M.D.,** Boston, Mass.
N
ontraumatic esophageal-atrial perforation is a rare and invariably fatal complication of benign esophageal ulceration.':" We report a case of esophageal-atrial perforation occurring 18 years subsequent to colonic interposition bypass of a lower esophageal stricture.
Case report A 62-year-old man was admitted to the Emergency Ward of the Massachusetts General Hospital because of a grand mal seizure. Immediately prior to admission he was noted to be diaphoretic, trembling, and unable to move. By the time of arrival in the emergency ward, generalized tonic-clonic movements and fecal incontinence were observed. Prior to this episode the patient was in excellent health except for intermittent epigastric burning. Twenty years before the present admission, retrostemal pain and dysphagia for many years, superimposed upon a history of ingestion of liniment as a child, led to a clinical From the Departments of Pathology and Surgery, Harvard Medical School. Massachusetts General Hospital, Boston, Mass. 02114. Received for publication Jan. 31, 1979. Accepted for publication March 9, 1979. Addressfor reprints: J. Gordon Scannell, M.D., Departmentof Surgery, Massachusetts General Hospital, Boston, Mass. 02114. *Department of Pathology. **Department of Surgery.
diagnosis of benign stricture of the low middle third of the esophagus. Active peptic esophagitis was not demonstrated at that time. Because of increasing dysphagia not responding to repeated bouginage and the relatively high level of the stricture, an esophageal bypass procedure was performed in 1960. A segment of transverse colon was used, passed in isoperistaltic fashion substernally with an end-to-end anastomosis to the cervical esophagus. The latter was transected and the distal end turned in and allowed to retract into the mediastinum. The lower end of the colonic bypass was anastomosed to the anterior surface of the stomach with an end-to-side cologastrostomy. The lower end of the esophagus and cardia were not explored. The patient did well following this procedure, with relief of his symptoms of esophageal obstruction. However, in 1976 he noted return of the burning pain, this time epigastric in nature. An upper gastrointestinal barium study at this time demonstrated a persistent hiatus hernia and gastric reflux into the bypassed lower segment of the esophagus (Fig. 1).* The symptoms were sufficiently controlled by the conventional measures of antacids and elevation of the head of the bed, so that no further surgical intervention was considered. Physical examination disclosed an acutely ill man with a systolic blood pressure of 88 mm Hg and an irregular pulse rate of 120 beats per minute. The abdomen was soft with frequent borborygmi. Guaiac examination of the stool was positive. Neurologic examination showed generalized hyperreflexia with clonis, bilateral Babinski signs, a left-sided *Courtesy of Dr. Caesar Briefer, Cambridge, Mass.
0022-5223179/080181+04$00.40/0 © 1979 The C. V. Mosby Co.
I8 I
The Journal of
182 Murphy, Raymond, Scannell
Thoracic and Cardiovascular Surgery
showed degenerative changes with loss of cross strianons (Fig. 3, insetB). The ulcer base contained fibrin and clusters of gram-positive cocci and was separated from the endothelial surface of the left atrium by a thin septum of fibrous tissue (Fig. 3). Fibrin was deposited at the site of perforation, which was located at the most attenuated portion of this intervening fibrous septum. There was no inflammation in the distal portion of the interposed segment of colon. Irregularly shaped fragments of para-aminosalicylic acid- positive material and gram-positive cocci were present within small arteries and arterioles of the heart, liver, kidneys, and spleen. Transverse striations were visible in some fragments by phosphotungstic acid-hematoxylin (PTAH) staining (Fig. 4).
Discussion
Fig. I. Barium study 2 years prior to esophageal-atrial perforation to evaluate symptoms of epigastric buming demonstrates reflux into distal segment of surgically bypassed esophagus (arrow). gaze preference, and spontaneous bilateral decerebrate posturing. Electrocardiogram disclosed atrial fibrillation and a right bundle branch block. An admission hematocrit valve was 42%. Intravenous Dilantin was slowly administered. Despite vigorous fluid management and vasopressor administration, hypotension persisted. The abdomen became increasingly distended. A repeat hematocrit value was 19o/c. A paracentesis was negative for intraperitoneal blood. Brisk oropharyngeal bleeding developed before the patient died 3 hours after admission. Postmortem examination revealed an intact substernal segment of transverse colon which joined the proximal esophagus to the anterior wall of the stomach. A 3.5 by 2.0 by 1.5 em deep ulcer was present in the anterior wall of the esophagus, 2.0 em above the esophageal-gastric junction (Fig. 2, A). The edges of the ulcer were elevated and rounded, and the base contained dark red material. A centrally located 0.2 by 0.3 em defect in the ulcer base communicated with the left atrial chamber. The rim of this defect consisted of hyperemic endocardium of the posterior wall of the left atrium and was located 2.5 em above the mitral valve anulus (Fig. 2, B). The stomach, small intestine, and colon were filled with approximately 3 L of unclotted blood. Microscopic examination revealed lymphocytic infiltration and fibrosis of the distal esophagus. The ulcer crater was surrounded by edematous mucosa lined by columnar epithelial cells (Fig. 3, insetA). The submucosa, adjacent pericardium, and left atrial myocardium were replaced by granulation tissue and dense fibrous connective tissue infiltrated by numerous lymphocytes and plasma cells. Residual myocytes
Nontraumatic perforation of the esophagus into the left atrium is a rare occurrence. To our knowledge, only six cases have been previously documented in the literature':" (Table I), four men and two women, with an age range of 20 to 78 years and a median age of 48 years. The majority had a history of epigastric discomfort or dysphagia of many years' duration. Most had radiographically documented chronic esophagitis with associated hiatus hernia or esophageal stricture. Hernatemesis was a common presenting manifestation of perforation, and all patients showed diffuse or focal neurologic signs. Death ensued from 5 hours to 6 days after presentation. Pathological examination disclosed chronic esophagitis with ulceration into the left atrium in five cases, and esophageal carcinoma was found in one patient. I Since esophageal-pericardial fistulas are significantly more common" than esophageal-atrial perforations, it is likely that chronic periesophageal fibrosis with obliteration of the adjacent pericardium is a prerequisite to the formation of a direct esophageal-atrial communication. Excluded from consideration here are two instances of traumatic esophageal-atrial perforations, one secondary to a bullet wound' and the second to mechanical intraesophageal trauma. H The case described herein conforms to the clinical and pathological features of those previously documented. Symptoms of chronic epigastric burning recurred 16 years after surgical bypass of a benign esophageal stricture and anteceded the formation of the esophageal-atrial perforation by 2 years. Hematemesis was not a presenting feature, presumably related to impaired emesis secondary to abnormal motility within the interposed segment of colon. This case illustrates that reflux esophagitis and its complications may arise in patients after esophageal bypass procedures in which the distal esophagus and esophagogastric junction are left intact. In the present instance small arteries of multiple or-
Volume 78
Recurrent esophagitis after esophageal bypass
Number 2
I 83
August, 1979
Fig. 2. A, Deep ulcer of distal esophagus with small perforation centrally located within the base (arrow). B, Perforation (arrow) of region of posterior wall of left atrium adjacent to ulcer base.
Table I. Summary of reported cases of nontraumatic esophageal-atrial perforation Chronic symptoms
Source lyear Hindenlang I (1881)
52, M
Epigastric pain, dysphagia (3 rno.)
Prolla, Taebel, and Kirsner'' (1967) Laubscher" (1970)
63, M
Dysphagia (8 yr.)
20, M
Clinical diagnosis
Chronic esophagitis, stricture History of trauma
Presenting signs
Postmortem findings
Syncope, coma; hematemesis
4 days
Focal neurologic deficits; hematemesis Syncope; hematemesis Syncope
41 hr. I day
Hojgaard and Raaschou-Nielson' ( 1970)
78, F
Epigastric burning (2 yr.) Dysphagia (12 yr.)
Itabashi and Granada'; (1972)
23, M
Vomiting (3-7 yr.)
Chronic esophagitis
Hyperreflexia; hematemesis
II hr.
Mott and Austin" (1976)
51, F
Dysphagia, hematemesis
Chronic esophagitis, hiatus hernia
Focal neurologic deficits; syncope
5 hr.
Murphy, Raymond, and Scannell (1979)
62, M
Dysphagia (18 yr.), epigastric burning (2 yr.)
Stricture (surgically bypassed), reflux esophagitis, hiatus hernia
Grand mal seizure
3 hr.
Esophageal stenosis, hiatus hernia
gans contained fragments of striated muscle and grampositive cocci identical to those colonizing the base of the esophageal ulcer. Systemic food embolization has been documented previously in one patient with an esophageal-atrial perforation;' and emboli composed of necrotic debris," septic squamous cell emboli, 1 and
6 days
Esophageal carcinoma; septic squamous cell emboli in brain Benign esophageal ulcer Benign esophageal ulcer Chronic esophagitis with stricture; meningoencephalitis Chronic esophagitis with ulcer; systemic food emboli Benign esophageal ulcer; systemic emboli composed of necrotic debris Chronic esophagitis with ulcer; systemic striated muscle emboli
meningoencephalitis" have been associated with this condition. The origin of the intra-arterial striated muscle fibers in our case is presumably from previously ingested gastric contents, although fragmentation and embolization of degenerating left atrial myocardium cannot be excluded, Neurologic signs are frequently
The Journal of
I 84
Murphy, Raymond, Scannell
Thoracic and Cardiovascular Surgery
Fig. 3. Ulcer base (b) is separated from left atrial endocardium (e) by dense fibrous tissue (hematoxylin and eosin, orig. mag. x II). Fibrin is deposited at the site of perforation (arrow). The surrounding esophageal mucosa is replaced by columnar epithelial cells and infiltrated by lymphocytes (inset A, hematoxylin and eosin, orig. mag. x III). The left atrial myocardium in this region shows degeneration and fibrous replacement of myocytes (inset B, hematoxylin and eosin, orig. mag. x 278).
cause of the occurrence of massive gastrointestinal hemorrhage and systemic embolization of septic material from the stomach or ulcer base. REFERENCES
2
3 4
5 Fig. 4. A splenic arteriole contains fragments of muscle exhibiting prominent transverse striations (phosphotungstic acid-hematoxylin, orig. mag. x256).
presenting features of esophageal-atrial perforation, and food emboli have been documented in cerebral tissues." In summary, esophageal-atrial perforation should be considered in patients with chronic reflux esophagitis who present with hypotension, hematemesis, and nonspecific neurologic signs. Rapid clinical recognition and surgical intervention is theoretically mandatory be-
6
7
8
Hindenlang C: Carcinoma oesophagi mit perforation in den L. Vorhof. OtschMedWochenschr7:105-113, 1881 Prolla JC, Taebel OW, Kirsner JB: Perforation of an esophagogastric anastomotic ulcer into the left atrium. Gastroenterology 52:871-874, 1967 Laubscher HA: Esophagocardiac fistula. N Engl J Med 282: 794- 795, 1970 Hojgaard K, Raaschou-Nielsen J: Oesophago-cardiac fistula. Acta Pathol Microbiol Scand Suppl 212: 114-116, 1970 Itabashi HH, Granada LO: Cerebral food embolism secondary to esophageal-cardiac perforation. JAMA 219: 373-375, 1972 Mott LJM, Austin GE: Cerebral embolization resulting from esophageal-atrial fistula. Arch Intern Med 136:718720, 1976 Bliznak J, Ramsey JO: Atrio-esophageal fistula secondary to gunshot wound of the chest. Milit Med 136:584-585. 1971 Roquefeuil B, Kienlen J, Viguie E, Markarian E, Hollea G: Rupture du bas oesophage consecutive a la mise en place d'une sonde gastrique avec fistule oeso-auriculaire gauche rapidement mortelle. Ann Anesth Franc X V, 2:129-134, 1974
George F. Murphy, M.D.,* A. Kevin Raymond, M.D.,* and J. Gordon Scannell, M.D.,** Boston, Mass.
N
ontraumatic esophageal-atrial perforation is a rare and invariably fatal complication of benign esophageal ulceration.':" We report a case of esophageal-atrial perforation occurring 18 years subsequent to colonic interposition bypass of a lower esophageal stricture.
Case report A 62-year-old man was admitted to the Emergency Ward of the Massachusetts General Hospital because of a grand mal seizure. Immediately prior to admission he was noted to be diaphoretic, trembling, and unable to move. By the time of arrival in the emergency ward, generalized tonic-clonic movements and fecal incontinence were observed. Prior to this episode the patient was in excellent health except for intermittent epigastric burning. Twenty years before the present admission, retrostemal pain and dysphagia for many years, superimposed upon a history of ingestion of liniment as a child, led to a clinical From the Departments of Pathology and Surgery, Harvard Medical School. Massachusetts General Hospital, Boston, Mass. 02114. Received for publication Jan. 31, 1979. Accepted for publication March 9, 1979. Addressfor reprints: J. Gordon Scannell, M.D., Departmentof Surgery, Massachusetts General Hospital, Boston, Mass. 02114. *Department of Pathology. **Department of Surgery.
diagnosis of benign stricture of the low middle third of the esophagus. Active peptic esophagitis was not demonstrated at that time. Because of increasing dysphagia not responding to repeated bouginage and the relatively high level of the stricture, an esophageal bypass procedure was performed in 1960. A segment of transverse colon was used, passed in isoperistaltic fashion substernally with an end-to-end anastomosis to the cervical esophagus. The latter was transected and the distal end turned in and allowed to retract into the mediastinum. The lower end of the colonic bypass was anastomosed to the anterior surface of the stomach with an end-to-side cologastrostomy. The lower end of the esophagus and cardia were not explored. The patient did well following this procedure, with relief of his symptoms of esophageal obstruction. However, in 1976 he noted return of the burning pain, this time epigastric in nature. An upper gastrointestinal barium study at this time demonstrated a persistent hiatus hernia and gastric reflux into the bypassed lower segment of the esophagus (Fig. 1).* The symptoms were sufficiently controlled by the conventional measures of antacids and elevation of the head of the bed, so that no further surgical intervention was considered. Physical examination disclosed an acutely ill man with a systolic blood pressure of 88 mm Hg and an irregular pulse rate of 120 beats per minute. The abdomen was soft with frequent borborygmi. Guaiac examination of the stool was positive. Neurologic examination showed generalized hyperreflexia with clonis, bilateral Babinski signs, a left-sided *Courtesy of Dr. Caesar Briefer, Cambridge, Mass.
0022-5223179/080181+04$00.40/0 © 1979 The C. V. Mosby Co.
I8 I
The Journal of
182 Murphy, Raymond, Scannell
Thoracic and Cardiovascular Surgery
showed degenerative changes with loss of cross strianons (Fig. 3, insetB). The ulcer base contained fibrin and clusters of gram-positive cocci and was separated from the endothelial surface of the left atrium by a thin septum of fibrous tissue (Fig. 3). Fibrin was deposited at the site of perforation, which was located at the most attenuated portion of this intervening fibrous septum. There was no inflammation in the distal portion of the interposed segment of colon. Irregularly shaped fragments of para-aminosalicylic acid- positive material and gram-positive cocci were present within small arteries and arterioles of the heart, liver, kidneys, and spleen. Transverse striations were visible in some fragments by phosphotungstic acid-hematoxylin (PTAH) staining (Fig. 4).
Discussion
Fig. I. Barium study 2 years prior to esophageal-atrial perforation to evaluate symptoms of epigastric buming demonstrates reflux into distal segment of surgically bypassed esophagus (arrow). gaze preference, and spontaneous bilateral decerebrate posturing. Electrocardiogram disclosed atrial fibrillation and a right bundle branch block. An admission hematocrit valve was 42%. Intravenous Dilantin was slowly administered. Despite vigorous fluid management and vasopressor administration, hypotension persisted. The abdomen became increasingly distended. A repeat hematocrit value was 19o/c. A paracentesis was negative for intraperitoneal blood. Brisk oropharyngeal bleeding developed before the patient died 3 hours after admission. Postmortem examination revealed an intact substernal segment of transverse colon which joined the proximal esophagus to the anterior wall of the stomach. A 3.5 by 2.0 by 1.5 em deep ulcer was present in the anterior wall of the esophagus, 2.0 em above the esophageal-gastric junction (Fig. 2, A). The edges of the ulcer were elevated and rounded, and the base contained dark red material. A centrally located 0.2 by 0.3 em defect in the ulcer base communicated with the left atrial chamber. The rim of this defect consisted of hyperemic endocardium of the posterior wall of the left atrium and was located 2.5 em above the mitral valve anulus (Fig. 2, B). The stomach, small intestine, and colon were filled with approximately 3 L of unclotted blood. Microscopic examination revealed lymphocytic infiltration and fibrosis of the distal esophagus. The ulcer crater was surrounded by edematous mucosa lined by columnar epithelial cells (Fig. 3, insetA). The submucosa, adjacent pericardium, and left atrial myocardium were replaced by granulation tissue and dense fibrous connective tissue infiltrated by numerous lymphocytes and plasma cells. Residual myocytes
Nontraumatic perforation of the esophagus into the left atrium is a rare occurrence. To our knowledge, only six cases have been previously documented in the literature':" (Table I), four men and two women, with an age range of 20 to 78 years and a median age of 48 years. The majority had a history of epigastric discomfort or dysphagia of many years' duration. Most had radiographically documented chronic esophagitis with associated hiatus hernia or esophageal stricture. Hernatemesis was a common presenting manifestation of perforation, and all patients showed diffuse or focal neurologic signs. Death ensued from 5 hours to 6 days after presentation. Pathological examination disclosed chronic esophagitis with ulceration into the left atrium in five cases, and esophageal carcinoma was found in one patient. I Since esophageal-pericardial fistulas are significantly more common" than esophageal-atrial perforations, it is likely that chronic periesophageal fibrosis with obliteration of the adjacent pericardium is a prerequisite to the formation of a direct esophageal-atrial communication. Excluded from consideration here are two instances of traumatic esophageal-atrial perforations, one secondary to a bullet wound' and the second to mechanical intraesophageal trauma. H The case described herein conforms to the clinical and pathological features of those previously documented. Symptoms of chronic epigastric burning recurred 16 years after surgical bypass of a benign esophageal stricture and anteceded the formation of the esophageal-atrial perforation by 2 years. Hematemesis was not a presenting feature, presumably related to impaired emesis secondary to abnormal motility within the interposed segment of colon. This case illustrates that reflux esophagitis and its complications may arise in patients after esophageal bypass procedures in which the distal esophagus and esophagogastric junction are left intact. In the present instance small arteries of multiple or-
Volume 78
Recurrent esophagitis after esophageal bypass
Number 2
I 83
August, 1979
Fig. 2. A, Deep ulcer of distal esophagus with small perforation centrally located within the base (arrow). B, Perforation (arrow) of region of posterior wall of left atrium adjacent to ulcer base.
Table I. Summary of reported cases of nontraumatic esophageal-atrial perforation Chronic symptoms
Source lyear Hindenlang I (1881)
52, M
Epigastric pain, dysphagia (3 rno.)
Prolla, Taebel, and Kirsner'' (1967) Laubscher" (1970)
63, M
Dysphagia (8 yr.)
20, M
Clinical diagnosis
Chronic esophagitis, stricture History of trauma
Presenting signs
Postmortem findings
Syncope, coma; hematemesis
4 days
Focal neurologic deficits; hematemesis Syncope; hematemesis Syncope
41 hr. I day
Hojgaard and Raaschou-Nielson' ( 1970)
78, F
Epigastric burning (2 yr.) Dysphagia (12 yr.)
Itabashi and Granada'; (1972)
23, M
Vomiting (3-7 yr.)
Chronic esophagitis
Hyperreflexia; hematemesis
II hr.
Mott and Austin" (1976)
51, F
Dysphagia, hematemesis
Chronic esophagitis, hiatus hernia
Focal neurologic deficits; syncope
5 hr.
Murphy, Raymond, and Scannell (1979)
62, M
Dysphagia (18 yr.), epigastric burning (2 yr.)
Stricture (surgically bypassed), reflux esophagitis, hiatus hernia
Grand mal seizure
3 hr.
Esophageal stenosis, hiatus hernia
gans contained fragments of striated muscle and grampositive cocci identical to those colonizing the base of the esophageal ulcer. Systemic food embolization has been documented previously in one patient with an esophageal-atrial perforation;' and emboli composed of necrotic debris," septic squamous cell emboli, 1 and
6 days
Esophageal carcinoma; septic squamous cell emboli in brain Benign esophageal ulcer Benign esophageal ulcer Chronic esophagitis with stricture; meningoencephalitis Chronic esophagitis with ulcer; systemic food emboli Benign esophageal ulcer; systemic emboli composed of necrotic debris Chronic esophagitis with ulcer; systemic striated muscle emboli
meningoencephalitis" have been associated with this condition. The origin of the intra-arterial striated muscle fibers in our case is presumably from previously ingested gastric contents, although fragmentation and embolization of degenerating left atrial myocardium cannot be excluded, Neurologic signs are frequently
The Journal of
I 84
Murphy, Raymond, Scannell
Thoracic and Cardiovascular Surgery
Fig. 3. Ulcer base (b) is separated from left atrial endocardium (e) by dense fibrous tissue (hematoxylin and eosin, orig. mag. x II). Fibrin is deposited at the site of perforation (arrow). The surrounding esophageal mucosa is replaced by columnar epithelial cells and infiltrated by lymphocytes (inset A, hematoxylin and eosin, orig. mag. x III). The left atrial myocardium in this region shows degeneration and fibrous replacement of myocytes (inset B, hematoxylin and eosin, orig. mag. x 278).
cause of the occurrence of massive gastrointestinal hemorrhage and systemic embolization of septic material from the stomach or ulcer base. REFERENCES
2
3 4
5 Fig. 4. A splenic arteriole contains fragments of muscle exhibiting prominent transverse striations (phosphotungstic acid-hematoxylin, orig. mag. x256).
presenting features of esophageal-atrial perforation, and food emboli have been documented in cerebral tissues." In summary, esophageal-atrial perforation should be considered in patients with chronic reflux esophagitis who present with hypotension, hematemesis, and nonspecific neurologic signs. Rapid clinical recognition and surgical intervention is theoretically mandatory be-
6
7
8
Hindenlang C: Carcinoma oesophagi mit perforation in den L. Vorhof. OtschMedWochenschr7:105-113, 1881 Prolla JC, Taebel OW, Kirsner JB: Perforation of an esophagogastric anastomotic ulcer into the left atrium. Gastroenterology 52:871-874, 1967 Laubscher HA: Esophagocardiac fistula. N Engl J Med 282: 794- 795, 1970 Hojgaard K, Raaschou-Nielsen J: Oesophago-cardiac fistula. Acta Pathol Microbiol Scand Suppl 212: 114-116, 1970 Itabashi HH, Granada LO: Cerebral food embolism secondary to esophageal-cardiac perforation. JAMA 219: 373-375, 1972 Mott LJM, Austin GE: Cerebral embolization resulting from esophageal-atrial fistula. Arch Intern Med 136:718720, 1976 Bliznak J, Ramsey JO: Atrio-esophageal fistula secondary to gunshot wound of the chest. Milit Med 136:584-585. 1971 Roquefeuil B, Kienlen J, Viguie E, Markarian E, Hollea G: Rupture du bas oesophage consecutive a la mise en place d'une sonde gastrique avec fistule oeso-auriculaire gauche rapidement mortelle. Ann Anesth Franc X V, 2:129-134, 1974