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Aortoesophageal fistula arising as a complication of prolonged nasogastric tube placement
CASE STUDIES
systemic and arteriovenous shunts within the liver in cirrhosis. Gastroenterology 93:129-134, 1987
imens show unexplained portal phlebosclerosis, particularly if venous intimal fibroplasia is so severe that elastic stains are required to reliably distinguish arteries from veins. Of course. the absence of these findings would not exclude the diagnosis of such a fistula. In cond%ions such as primary portal hypertension, portal vein changes tend to be much milder than those described in this report.
5. Hellekant C: Vascular complications following needle puncture of 1976 6. Okuda K. Musha H. Nakaiima Y, et al: Frequency of intrahepatic arteriovenous &la as a sequela To percutaneous nkedle’puncture 0; the liver. Gastroenterology 74:1204-1207, 1978 7. Takayasu K, Moriyama N, lshikawa T, et al: Large spontaneous intra-hepatic arterioportal fistula demonstrated by rapid sequential computed tomographic scan. Report of two cases. Gastroenterology 88:564-570, 1985 8: lnon AE, D’Agostino D: Portal hypertension secondary to congenital arteriooortal fistula. I Pediatr Gastroenterol Nutr 6:471-473, 1987 9. ‘Wee A, Lud&g J: Pericholangitis in chronic ulcerative colitis: Primary cholangitis of the small bile ducts? Ann Intern Med 102:581___ sclerosing ___ 587, 1Y85 10. Strode1 WE, Eckhauser FE, Lemmer JH, et al: Presentation and
the liver. Clinical angiography. Acta Radio1 Diagn 17:209-221,
REFERENCES 1. Derauf B1, Hunter DW, Sirr SA, et al: Peripheral embolization of diffuse hepatic &teriovenous malformations in a patient with hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radio1 10:80-83, 1987
perioperative
2. McKinnon WM, Smith RB, Davis SC, et al: Ruptured hepatic artery aneurysm with hepatic artery-to-portal vein fistula. Preservation of portal flow by autogenous vein reconstruction. Surgery 89:626-630, 1981 3. Itai Y, Furui S. Ohotoma K, et al: Dynamic CT feature of arterioportal shunts in hepatocellular carcinoma. AJR 146:723-727, 1986 4. Ohonishi K, Chin N, Sugita S, et al: Quantitative aspects of portal-
AORTOESOPHAGEAL TUBE PLACEMENT
IQP,
MD,
PHD,
of arterioportal
fistulas. Arch Surg 122:563-571,
11. Donovan AJ, Reynolds TB, Mikkelsen WP, et al: Systemic-portal arteriovenous fistulas: Pathological and hemodynamic observations in two patients. Surgery 66:474-482, 1969 12. Fulton RL, Wolfe1 DA: Hepatic artery-portal. vein arteriovenous I. Iistula. Arch Surg 100:307-309, 1976
FISTMA ARISING AS A COMPLICATION
KENT B. LEWANDROWSKI, MD, JAMES F. SOUTHERN,
management
I.,“’
OF PROLONGED
L. JEFFREY MEDEIROS, MD,
NASOGASTRIC AND MARSHALL JACOBS,
MD
ble angina pectoris. She described a 20-year history of exertional chest pain and systemic hypertension. During the past year, the chest pain had progressed to pain severe enough to awaken the patient most evenings. The patient denied prior gastrointestinal diseases or symptoms, including heartburn. Her medications at the time of hospital admission included propranolol, diltiazem, nitroglycerin, and furosemide. Physical examination was unremarkable. All laboratory studies including serum creatine kinase were normal. An ECG revealed changes consistent with a remote anteroseptal myocardial infarct and anterolateral T wave abnormalities. The patient underwent cardiac catheterization. Severe stenosis of the distal left main coronary artery and two severe stenoses of the proximal right coronary artery were identified. Immediately following catheterization, the patient developed severe hypotension with ECG changes consistent with ischemia which persisted despite administration of norepinephrine, dopamine, and placement of an intraaortic balloon pump. Emergency triple aortocoronary artery bypass surgery, using saphenous vein grafts, was then performed. At the time of surgery, both an endotracheal tube and an orogastric tube were placed, the latter for gastric decompression and administration of antacids. On the first postoperative day, persistent bloody chest tube drainage prompted mediastinal reexploration. No major bleeding sites were identified. The intraaortic balloon pump was removed the following day. The mechanical ventilator was discontinued and the endotracheal tube was removed after 1 week. The orogastric tube was replaced with a nasogastric tube for enteral nutrition. A nasogastric tube remained in place (it was changed once) until the last day of the patient’s hospital stay. One day before death, the patient developed hematemesis which necessitated transfusions of multiple units of blood. Once the bleeding was controlled, upper pharyngoesophagogastroscopy was performed and revealed deep esophageal ulcers immediately distal to the level of the aortic arch and abundant blood within the stomach. The possibility of aortoesophageal fistula was suspected, the patient was informed, and correc-
Aortoesophageal fist&a is a rare disorder that may result from many causes. In this report, we describe the unique case of a 71year-old woman who developed an aortoesophagealfktula following prolonged placement of a nasogastric tube. The presence of dense fibrous adhesions between the aorta and esophagus may have facilitated the development of aortoesophageal fistula in this patient. HUM PATHOL 20:709-711. 0 1989 by W.B. Saunders Company. Aortoesophageal fistula is a rare disorder that may result from many causes, including primary diseases of the aorta (eg, thoracic aortic aneurysm) or esophagus (eg, squamous cell carcinoma), ingestion of a foreign body, penetrating trauma, and iatrogenically induced injury, such as a complication of a contiguous surgical procedure or surgical instrumentation (eg, rigid esophagoscopy).‘-’ In this report, we describe an aortoesophageal fistula that resulted from prolonged placement of a nasogastric tube. Although nasogastric tube placement has resulted in esophageal perforation and mediastinitis,2 to our knowledge, aortoesophageal fistula arising as a complication of nasogastric tube placement is unique. A role for aortoesophageal fibrous adhesions as a factor that facilitated the development of the aortoesophageal fistula is proposed. CASE REPORT A 71-year-old woman was admitted to the Massachusetts General Hospital (Boston) for the treatment of unstaReceived January 27, 1989, from the Departments of Pathology and Cardiovascular Surgery, Massachusetts General Hospital and Harvard Medical School, Boston. Accepted for publication February 16, 1989. Kqr words: aortoesophageal fistula, nasogastric tube, cardiac surgery. Address correspondence and reprint requests to Kent B. Lewandrowski, MD, Department of Patholoav, -, Massachusetts Genera1 Hospital, Boston, GA 02114. 0 1989 by W.B. Saunders Company. 0046-8177/89/2007-0015$5.00/0
709
HUMAN PATHOLOGY
tive surgery was recommended. However, the patient refused an additional operation. The next day, 30 days after hospital admission, massive hematemesis developed. Despite placement of a Sengstaken-Blakemore tube and numerous blood transfusions, the patient developed progressive hypotension and died. Autopsy Findings Dense fibrous adhesions filled the spaces between the descending thoracic aorta, esophagus, trachea, and the right mainstem bronchus. Almost the entire lengths of the esophagus and aorta were firmly adherent to each other. Two ulcers were found within the esophagus. The deepest ulcer, 1.5 X 0.5 cm, was located 5.0 cm distal to the level of origin of the left subclavian artery. The ulcer (Fig 1) penetrated deeply through the walls of the esophagus and descending thoracic aorta, communicating with the aortic lumen and forming a fistula. The fistula was 0.2 cm in diameter where it entered the aortic lumen. The fistula tract contained clotted blood. A second esophageal ulcer, 0.5 cm in diameter, eroded through the esophagus and into the membranous portion of the right mainstem bronchus, but did not completely penetrate through the bronchial wall. Also present at the gastroesophageal junction and along the greater curvature of the stomach were multiple mucosal erosions, each approximately 0.5 cm, which appeared to outline the shape of a nasogastric tube. The stomach was distended by 1,700 mL of blood. Microscopically, the aortoesophageal fistula was pyramidal, with its base corresponding to the esophageal lumen and its apex corresponding to the aortic lumen (Fig 2). The tract was lined by necrotic debris, acute and chronic inflammatory cells, and granulation tissue. Abundant grampositive and -negative bacteria were found predominantly at the base of the fistula. The aortoesophageal adhesions were composed of dense fibosis with scant, small foci of lymphocytes and macrophages. There were no acute inflammatory cells or granulation tissue within the fibrous scar tissue. Additional autopsy findings included dense pericardial fibrosis (attributed to the patient’s prior open heart surgery), severe coronary artery atherosclerosis with over 80% occlusion of the left main and right coronary arteries, healed and healing myocardial infarcts, cardiomegaly (501 g) with biatrial and left ventricular dilatation, severe atherosclerosis of cerebral arteries, and organizing bronchopneumonia of the right lower lobe. The coronary artery
Volume 20, No. 7 (July 1989)
bypass grafts were patent. There was minimal atherosclerosis of the thoracic aorta without evidence of aneurysm formation and no plaques of atherosclerosis were found near the fistula site. DISCUSSION A classic clinical presentation, first described by Chiari in 1914,‘B2 is often observed in patients with aortoesophageal fistula. Affected individuals develop transient hematemesis (referred to as “sentinel” or “signal” hematemesis),
FIGURE 2. Low-power photomicrograph of the aortoesophageal fistula (above center of field). The esophageal lumen is on the left. The aortic lumen is on the right. (Hematoxyiin-eosin stain; magnification x 6).
710
CASESTUDIES (particularly if acute) is usually not followed by fistula development. lo Esophageal contents leaking from a site of perforation usually follow the path of least resistance, along soft tissue planes and between vital structures such as the aorta.‘O Thus, we hypothesize that the presence of dense aortoesophageal fibrous adhesions was essential to the development of the aortoesophageal fistula by bringing the aorta and esophagus into close proximity and by confining and directing the ulcer base and the esophageal contents into the wall of the aorta until the aortic wall was eroded and a fistula formed. The origin of the dense periaortic and periesophageal fibrosis in this case is not clear. The fibrous tissue contained scant chronic inflammation, similar to that of the pericardial fibrosis that we attributed to prior open heart surgery. However, no dissection was performed in the region of the esophagus and aorta. Postoperatively, the patient had abundant bleeding that necessitated reexploration and search for bleeding sites. The postoperative hemorrhage may have dissected along the soft tissue planes between the esophagus and aorta, inciting an intense librosing process due to the presence of highly fibrogenic blood.
asymptomatic for an interval that may last hours to weeks (the “latency period”), and then exsanguinate.2,4~5~s.9 This clinical presentation occurred in our case. On the 29th hospital day, the patient developed hematemesis which was controlled. Twenty-four hours later, the patient developed massive hematemesis, progressive hypotension, and died. The aortoesophageal fistula appears to have arisen as a complication of a penetrating esophageal ulcer. The fistula was pyramidal; its 0.5-cm base corresponded to the lumen of the esophagus and its 0.2 cm apex was located at the aortic lumen. Numerous other ulcers and erosions involved the mucosa and submucosa of the esophagus, while the thoracic aorta was almost normal. Only mild atherosclerosis was found, no plaques were found at or near the fistula site, and there was no evidence of aortic aneurysm. Prolonged placement of the nasogastric tube appears to be the most likely cause of the esophageal ulcer in this case. Nasogastric tubes are known to damage both esophageal and gastric mucosa and mucosal erosions are commonly found at autopsy in patients with nasogastric tubes in place. The mucosal erosions commonly demonstrate a linear pattern outlining the pathway of the nasogastric tube, as was observed. In addition, the patient had no prior history of heartburn or other complaints which might suggest that these ulcers were present before placement of the nasogastric tube, nor was there evidence of another cause of esophageal ulceration such as infection by Candidu sp or herpesvirus. The sequence of events from initial mucosal ulceration by the nasogastric tube to the formation of the aortoesophageal fistula is speculative. Prolonged episodes of hypotension combined with vasopressor treatment are known to cause ischemia and necrosis of the esophageal mucosa. The necrotic regions then ulcerated, perhaps secondary to pressure contact from the nasogastric tube. Pressure from the tube may have also been responsible for the depth of the ulcers. The mixed bacterial flora found at the ftstula base, consistent with contamination from the oropharynx, may have also played a role in ulcer development by their release of damaging enzymes. The ulcer eventually penetrated completely through the wall of the esophagus. However, these factors alone do not explain the development of the aortoesophageal fistula, since esophageal perforation are
REFERENCES 1. Lui RC, Johnson FE, Horovitz JH, et al: Aortoesophageal fist&: Case report and literature review. J Vast Surg 6:379-3&X?, 1987 2. Carter R, Molder GA, Snyder EN J r, et al: Aortoesophageal fistula. Am J Surg 136:26-28, 1978 3. Alrenga DP: Fatal hemorrhage complicating carcinoma of the esophanus: Reoort of four cases. Am 1 Gastroenterol 65:422-426, 1976 4. Yonago RH, lben AB, Mark JBD: Aortic bypass in the management of aortoesophageal fist&. Ann Thorac Surg 7:235-237, 1969 5. Cte&teko G, Mok CK: Aorta-esophageal fist& induced by a foreign body. The first recorded survival. J Thorac Cardiovasc Surg 80:233-235, ”
.
1980 6. Valtonen EJ, Koiv’uniemi A: Aortoesophageal fist& complicating carcinoma of the esophagus. Report of observations in two cases. J Thorac Cardiovasc Surg 53:448-452, 1967 7. Lefkowitz M, Elsas LJ, Levine RJ: Candida infection complicating oeotic esoohaeeal ulcer. Arch Intern Med 113:672-675, 1964 ’ * 8. Sldop ED, Thompson JC: Aorta-esophageal fist&: Report of a case and review of the literature. Gastroenterology 53:768-777, 1967 9. Baron RL, Koehler RE, Gutierrez FRyit al: Clinical and radiographic manifestations of aortoesophageal fistulas. Radiology 141:599-605, 198 1 10. Berry BE, Ochsner JL: Perforation of the esophagus. A 30 year review. J Thorac Cardiovasc Surg 65:1-7, 1973
IMMUNOHISTOCHEMICAL CHARACTERIZATION OF THE HISTIOCYTES LYMPHADENOPATHY: ANALYSIS OF AN EXTRANODAL CASE PHILLIP LOPEZ, DO,
AND MELINDA L. ESTES,
IN SINUS HISTIOCYTOSIS
WITH MASSIVE
MD
We studied the morphologic, antigenic, and enzymatic characteristics of the histiocytesin an isolated extranodal case of sinus histiocytosiswith massive lymphudenopathy(SHML) involving the CNS. To our knowledge, this represents the first immunohistochemically documented case of CNS SHML. The histiocytes exhibited the Received November 7, 1988, from the Department of Pathology, Cleveland Clinic Foundation. Accepted for publication February 28,1989. Kqr worok sinus histiocytosis with massive lymphadenopathy, immunohistochemical, CNS, histiocvtes. Address correspondence and bequests to Melinda L. Estes, MD, Department of Pathology, Cleveland Clinic Foundation, 1 Clinic Center Dr (L24), Cleveland, OH 441955138. 0 1989 by W.B. Saunders Company. 0046-8177/89/2007-0016$5.00/O
S-l 00( +), CD1 1c( +), a-l -antichymotrypsin(+) immunophenotype, which suggests that the histiocytesof SHML coexpressphenotypic characteristics of histiocytes of the mononuclear phagocytic system, ana’ histiocytes of the interdigitating reticulum cell and Langerhan cell lineages. To examine the argument that the histiocytes in SHML may represent ordinary tissue macrophages of granulomatous inflammation, we compared the immunophenotypic characteristicsof the histiocytesfound in SHML to thosefound in xanthogranulomatouspyelonephritis (XP). We found that the histiocytesof XP have immunophenotypic characteristicsof histiocytes belonging to the mononuclear phagocytic system lineage. The present study demonstratesthat the histiocytesof SHML are distinct from the histiocytesof another xanthogranulomatous disorder, supporting the concept that SHML is a distinct clinicopathologic entity. HUM PATHOL 20:71 I-715. 0 1989 by W.B. Saunders Company.
711
systemic and arteriovenous shunts within the liver in cirrhosis. Gastroenterology 93:129-134, 1987
imens show unexplained portal phlebosclerosis, particularly if venous intimal fibroplasia is so severe that elastic stains are required to reliably distinguish arteries from veins. Of course. the absence of these findings would not exclude the diagnosis of such a fistula. In cond%ions such as primary portal hypertension, portal vein changes tend to be much milder than those described in this report.
5. Hellekant C: Vascular complications following needle puncture of 1976 6. Okuda K. Musha H. Nakaiima Y, et al: Frequency of intrahepatic arteriovenous &la as a sequela To percutaneous nkedle’puncture 0; the liver. Gastroenterology 74:1204-1207, 1978 7. Takayasu K, Moriyama N, lshikawa T, et al: Large spontaneous intra-hepatic arterioportal fistula demonstrated by rapid sequential computed tomographic scan. Report of two cases. Gastroenterology 88:564-570, 1985 8: lnon AE, D’Agostino D: Portal hypertension secondary to congenital arteriooortal fistula. I Pediatr Gastroenterol Nutr 6:471-473, 1987 9. ‘Wee A, Lud&g J: Pericholangitis in chronic ulcerative colitis: Primary cholangitis of the small bile ducts? Ann Intern Med 102:581___ sclerosing ___ 587, 1Y85 10. Strode1 WE, Eckhauser FE, Lemmer JH, et al: Presentation and
the liver. Clinical angiography. Acta Radio1 Diagn 17:209-221,
REFERENCES 1. Derauf B1, Hunter DW, Sirr SA, et al: Peripheral embolization of diffuse hepatic &teriovenous malformations in a patient with hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radio1 10:80-83, 1987
perioperative
2. McKinnon WM, Smith RB, Davis SC, et al: Ruptured hepatic artery aneurysm with hepatic artery-to-portal vein fistula. Preservation of portal flow by autogenous vein reconstruction. Surgery 89:626-630, 1981 3. Itai Y, Furui S. Ohotoma K, et al: Dynamic CT feature of arterioportal shunts in hepatocellular carcinoma. AJR 146:723-727, 1986 4. Ohonishi K, Chin N, Sugita S, et al: Quantitative aspects of portal-
AORTOESOPHAGEAL TUBE PLACEMENT
IQP,
MD,
PHD,
of arterioportal
fistulas. Arch Surg 122:563-571,
11. Donovan AJ, Reynolds TB, Mikkelsen WP, et al: Systemic-portal arteriovenous fistulas: Pathological and hemodynamic observations in two patients. Surgery 66:474-482, 1969 12. Fulton RL, Wolfe1 DA: Hepatic artery-portal. vein arteriovenous I. Iistula. Arch Surg 100:307-309, 1976
FISTMA ARISING AS A COMPLICATION
KENT B. LEWANDROWSKI, MD, JAMES F. SOUTHERN,
management
I.,“’
OF PROLONGED
L. JEFFREY MEDEIROS, MD,
NASOGASTRIC AND MARSHALL JACOBS,
MD
ble angina pectoris. She described a 20-year history of exertional chest pain and systemic hypertension. During the past year, the chest pain had progressed to pain severe enough to awaken the patient most evenings. The patient denied prior gastrointestinal diseases or symptoms, including heartburn. Her medications at the time of hospital admission included propranolol, diltiazem, nitroglycerin, and furosemide. Physical examination was unremarkable. All laboratory studies including serum creatine kinase were normal. An ECG revealed changes consistent with a remote anteroseptal myocardial infarct and anterolateral T wave abnormalities. The patient underwent cardiac catheterization. Severe stenosis of the distal left main coronary artery and two severe stenoses of the proximal right coronary artery were identified. Immediately following catheterization, the patient developed severe hypotension with ECG changes consistent with ischemia which persisted despite administration of norepinephrine, dopamine, and placement of an intraaortic balloon pump. Emergency triple aortocoronary artery bypass surgery, using saphenous vein grafts, was then performed. At the time of surgery, both an endotracheal tube and an orogastric tube were placed, the latter for gastric decompression and administration of antacids. On the first postoperative day, persistent bloody chest tube drainage prompted mediastinal reexploration. No major bleeding sites were identified. The intraaortic balloon pump was removed the following day. The mechanical ventilator was discontinued and the endotracheal tube was removed after 1 week. The orogastric tube was replaced with a nasogastric tube for enteral nutrition. A nasogastric tube remained in place (it was changed once) until the last day of the patient’s hospital stay. One day before death, the patient developed hematemesis which necessitated transfusions of multiple units of blood. Once the bleeding was controlled, upper pharyngoesophagogastroscopy was performed and revealed deep esophageal ulcers immediately distal to the level of the aortic arch and abundant blood within the stomach. The possibility of aortoesophageal fistula was suspected, the patient was informed, and correc-
Aortoesophageal fist&a is a rare disorder that may result from many causes. In this report, we describe the unique case of a 71year-old woman who developed an aortoesophagealfktula following prolonged placement of a nasogastric tube. The presence of dense fibrous adhesions between the aorta and esophagus may have facilitated the development of aortoesophageal fistula in this patient. HUM PATHOL 20:709-711. 0 1989 by W.B. Saunders Company. Aortoesophageal fistula is a rare disorder that may result from many causes, including primary diseases of the aorta (eg, thoracic aortic aneurysm) or esophagus (eg, squamous cell carcinoma), ingestion of a foreign body, penetrating trauma, and iatrogenically induced injury, such as a complication of a contiguous surgical procedure or surgical instrumentation (eg, rigid esophagoscopy).‘-’ In this report, we describe an aortoesophageal fistula that resulted from prolonged placement of a nasogastric tube. Although nasogastric tube placement has resulted in esophageal perforation and mediastinitis,2 to our knowledge, aortoesophageal fistula arising as a complication of nasogastric tube placement is unique. A role for aortoesophageal fibrous adhesions as a factor that facilitated the development of the aortoesophageal fistula is proposed. CASE REPORT A 71-year-old woman was admitted to the Massachusetts General Hospital (Boston) for the treatment of unstaReceived January 27, 1989, from the Departments of Pathology and Cardiovascular Surgery, Massachusetts General Hospital and Harvard Medical School, Boston. Accepted for publication February 16, 1989. Kqr words: aortoesophageal fistula, nasogastric tube, cardiac surgery. Address correspondence and reprint requests to Kent B. Lewandrowski, MD, Department of Patholoav, -, Massachusetts Genera1 Hospital, Boston, GA 02114. 0 1989 by W.B. Saunders Company. 0046-8177/89/2007-0015$5.00/0
709
HUMAN PATHOLOGY
tive surgery was recommended. However, the patient refused an additional operation. The next day, 30 days after hospital admission, massive hematemesis developed. Despite placement of a Sengstaken-Blakemore tube and numerous blood transfusions, the patient developed progressive hypotension and died. Autopsy Findings Dense fibrous adhesions filled the spaces between the descending thoracic aorta, esophagus, trachea, and the right mainstem bronchus. Almost the entire lengths of the esophagus and aorta were firmly adherent to each other. Two ulcers were found within the esophagus. The deepest ulcer, 1.5 X 0.5 cm, was located 5.0 cm distal to the level of origin of the left subclavian artery. The ulcer (Fig 1) penetrated deeply through the walls of the esophagus and descending thoracic aorta, communicating with the aortic lumen and forming a fistula. The fistula was 0.2 cm in diameter where it entered the aortic lumen. The fistula tract contained clotted blood. A second esophageal ulcer, 0.5 cm in diameter, eroded through the esophagus and into the membranous portion of the right mainstem bronchus, but did not completely penetrate through the bronchial wall. Also present at the gastroesophageal junction and along the greater curvature of the stomach were multiple mucosal erosions, each approximately 0.5 cm, which appeared to outline the shape of a nasogastric tube. The stomach was distended by 1,700 mL of blood. Microscopically, the aortoesophageal fistula was pyramidal, with its base corresponding to the esophageal lumen and its apex corresponding to the aortic lumen (Fig 2). The tract was lined by necrotic debris, acute and chronic inflammatory cells, and granulation tissue. Abundant grampositive and -negative bacteria were found predominantly at the base of the fistula. The aortoesophageal adhesions were composed of dense fibosis with scant, small foci of lymphocytes and macrophages. There were no acute inflammatory cells or granulation tissue within the fibrous scar tissue. Additional autopsy findings included dense pericardial fibrosis (attributed to the patient’s prior open heart surgery), severe coronary artery atherosclerosis with over 80% occlusion of the left main and right coronary arteries, healed and healing myocardial infarcts, cardiomegaly (501 g) with biatrial and left ventricular dilatation, severe atherosclerosis of cerebral arteries, and organizing bronchopneumonia of the right lower lobe. The coronary artery
Volume 20, No. 7 (July 1989)
bypass grafts were patent. There was minimal atherosclerosis of the thoracic aorta without evidence of aneurysm formation and no plaques of atherosclerosis were found near the fistula site. DISCUSSION A classic clinical presentation, first described by Chiari in 1914,‘B2 is often observed in patients with aortoesophageal fistula. Affected individuals develop transient hematemesis (referred to as “sentinel” or “signal” hematemesis),
FIGURE 2. Low-power photomicrograph of the aortoesophageal fistula (above center of field). The esophageal lumen is on the left. The aortic lumen is on the right. (Hematoxyiin-eosin stain; magnification x 6).
710
CASESTUDIES (particularly if acute) is usually not followed by fistula development. lo Esophageal contents leaking from a site of perforation usually follow the path of least resistance, along soft tissue planes and between vital structures such as the aorta.‘O Thus, we hypothesize that the presence of dense aortoesophageal fibrous adhesions was essential to the development of the aortoesophageal fistula by bringing the aorta and esophagus into close proximity and by confining and directing the ulcer base and the esophageal contents into the wall of the aorta until the aortic wall was eroded and a fistula formed. The origin of the dense periaortic and periesophageal fibrosis in this case is not clear. The fibrous tissue contained scant chronic inflammation, similar to that of the pericardial fibrosis that we attributed to prior open heart surgery. However, no dissection was performed in the region of the esophagus and aorta. Postoperatively, the patient had abundant bleeding that necessitated reexploration and search for bleeding sites. The postoperative hemorrhage may have dissected along the soft tissue planes between the esophagus and aorta, inciting an intense librosing process due to the presence of highly fibrogenic blood.
asymptomatic for an interval that may last hours to weeks (the “latency period”), and then exsanguinate.2,4~5~s.9 This clinical presentation occurred in our case. On the 29th hospital day, the patient developed hematemesis which was controlled. Twenty-four hours later, the patient developed massive hematemesis, progressive hypotension, and died. The aortoesophageal fistula appears to have arisen as a complication of a penetrating esophageal ulcer. The fistula was pyramidal; its 0.5-cm base corresponded to the lumen of the esophagus and its 0.2 cm apex was located at the aortic lumen. Numerous other ulcers and erosions involved the mucosa and submucosa of the esophagus, while the thoracic aorta was almost normal. Only mild atherosclerosis was found, no plaques were found at or near the fistula site, and there was no evidence of aortic aneurysm. Prolonged placement of the nasogastric tube appears to be the most likely cause of the esophageal ulcer in this case. Nasogastric tubes are known to damage both esophageal and gastric mucosa and mucosal erosions are commonly found at autopsy in patients with nasogastric tubes in place. The mucosal erosions commonly demonstrate a linear pattern outlining the pathway of the nasogastric tube, as was observed. In addition, the patient had no prior history of heartburn or other complaints which might suggest that these ulcers were present before placement of the nasogastric tube, nor was there evidence of another cause of esophageal ulceration such as infection by Candidu sp or herpesvirus. The sequence of events from initial mucosal ulceration by the nasogastric tube to the formation of the aortoesophageal fistula is speculative. Prolonged episodes of hypotension combined with vasopressor treatment are known to cause ischemia and necrosis of the esophageal mucosa. The necrotic regions then ulcerated, perhaps secondary to pressure contact from the nasogastric tube. Pressure from the tube may have also been responsible for the depth of the ulcers. The mixed bacterial flora found at the ftstula base, consistent with contamination from the oropharynx, may have also played a role in ulcer development by their release of damaging enzymes. The ulcer eventually penetrated completely through the wall of the esophagus. However, these factors alone do not explain the development of the aortoesophageal fistula, since esophageal perforation are
REFERENCES 1. Lui RC, Johnson FE, Horovitz JH, et al: Aortoesophageal fist&: Case report and literature review. J Vast Surg 6:379-3&X?, 1987 2. Carter R, Molder GA, Snyder EN J r, et al: Aortoesophageal fistula. Am J Surg 136:26-28, 1978 3. Alrenga DP: Fatal hemorrhage complicating carcinoma of the esophanus: Reoort of four cases. Am 1 Gastroenterol 65:422-426, 1976 4. Yonago RH, lben AB, Mark JBD: Aortic bypass in the management of aortoesophageal fist&. Ann Thorac Surg 7:235-237, 1969 5. Cte&teko G, Mok CK: Aorta-esophageal fist& induced by a foreign body. The first recorded survival. J Thorac Cardiovasc Surg 80:233-235, ”
.
1980 6. Valtonen EJ, Koiv’uniemi A: Aortoesophageal fist& complicating carcinoma of the esophagus. Report of observations in two cases. J Thorac Cardiovasc Surg 53:448-452, 1967 7. Lefkowitz M, Elsas LJ, Levine RJ: Candida infection complicating oeotic esoohaeeal ulcer. Arch Intern Med 113:672-675, 1964 ’ * 8. Sldop ED, Thompson JC: Aorta-esophageal fist&: Report of a case and review of the literature. Gastroenterology 53:768-777, 1967 9. Baron RL, Koehler RE, Gutierrez FRyit al: Clinical and radiographic manifestations of aortoesophageal fistulas. Radiology 141:599-605, 198 1 10. Berry BE, Ochsner JL: Perforation of the esophagus. A 30 year review. J Thorac Cardiovasc Surg 65:1-7, 1973
IMMUNOHISTOCHEMICAL CHARACTERIZATION OF THE HISTIOCYTES LYMPHADENOPATHY: ANALYSIS OF AN EXTRANODAL CASE PHILLIP LOPEZ, DO,
AND MELINDA L. ESTES,
IN SINUS HISTIOCYTOSIS
WITH MASSIVE
MD
We studied the morphologic, antigenic, and enzymatic characteristics of the histiocytesin an isolated extranodal case of sinus histiocytosiswith massive lymphudenopathy(SHML) involving the CNS. To our knowledge, this represents the first immunohistochemically documented case of CNS SHML. The histiocytes exhibited the Received November 7, 1988, from the Department of Pathology, Cleveland Clinic Foundation. Accepted for publication February 28,1989. Kqr worok sinus histiocytosis with massive lymphadenopathy, immunohistochemical, CNS, histiocvtes. Address correspondence and bequests to Melinda L. Estes, MD, Department of Pathology, Cleveland Clinic Foundation, 1 Clinic Center Dr (L24), Cleveland, OH 441955138. 0 1989 by W.B. Saunders Company. 0046-8177/89/2007-0016$5.00/O
S-l 00( +), CD1 1c( +), a-l -antichymotrypsin(+) immunophenotype, which suggests that the histiocytesof SHML coexpressphenotypic characteristics of histiocytes of the mononuclear phagocytic system, ana’ histiocytes of the interdigitating reticulum cell and Langerhan cell lineages. To examine the argument that the histiocytes in SHML may represent ordinary tissue macrophages of granulomatous inflammation, we compared the immunophenotypic characteristicsof the histiocytesfound in SHML to thosefound in xanthogranulomatouspyelonephritis (XP). We found that the histiocytesof XP have immunophenotypic characteristicsof histiocytes belonging to the mononuclear phagocytic system lineage. The present study demonstratesthat the histiocytesof SHML are distinct from the histiocytesof another xanthogranulomatous disorder, supporting the concept that SHML is a distinct clinicopathologic entity. HUM PATHOL 20:71 I-715. 0 1989 by W.B. Saunders Company.
711