Vol. XXV, No. 11 Printed in U.S.A.
JOURNAL OF THE AMERICAN GERIATRICS SOCIETY Copyright © 1977 by the American Geriatrics Society
Agnogenic Venous Mesenteric Thrombosis WILLIAM F. MITTY, JR, MD** and CLEMENTINO O. PURISIMA, MDt
St. Clare's Hospital and Health Center, New York, NY ABSTRACT: One of the most difficult diagnoses to establish is that of agnogenic venous mesenteric thrombosis (AVMT). This disorder occurs chiefly in elderly patients and, unless diagnosed promptly, leads to death in most instances. AVMT may follow surgical operations or occur during a prolonged illness. In the past five years at St. Clare's Hospital and Health Center in New York City, this diagnosis was established in five patients. In reviewing these cases, it was noted that some of the signs and symptoms such as those due to hypotension and shock with marked leukocytosis, were out of proportion to those usually observed when the preoperative diagnosis is being considered. The pathologic and radiologic characteristics of this disorder are outlined, and the recommended operative procedure for treatment is discussed. The importance is stressed of prompt, vigorous and prolonged anticoagulation therapy in order to minimize the chance of recurrence in the early postoperative period. Anticoagulant therapy is also effective preoperatively, if the disease is diagnosed sufficiently early. As greater numbers of elderly patients are being treated in hospitals, this dire complication should be uppermost in the minds of physicians and surgeons if a fatal outcome is to be avoided following a successful operative procedure.
Greek "agnes" (unknown), and is thus applied to diseases of unknown or obscure etiology. During the past five years, five cases of AVMT were diagnosed at the St. Clare's Hospital and Health Center of New York City. The highlights of these cases are presented, to show the dilemma which exists in making this diagnosis early enough to assure recovery.
Agnogenic venous mesenteric thrombosis (AVMT) is an entity first described by Beniviene (1) in the latter part of the fifteenth century. However, it was not appreciated by the medical profession until reports emanated from the clinics of Tiedman (2) in 1843 and Virchow (3) in 1847 and 1854. Elliot (4) in 1895 was the first author to report a patient surviving this pathologic condition. From that era to the present, there have been many articles (5) on mesenteric thrombosis but the authors failed to distinguish the arterial from the venous form. Donaldson and Stout (6) in 1935 first pointed out that venous mesenteric thrombosis was an entity separate from arterial thrombosis. According to Dorland's Medical Dictionary (25th edition), "agnogenic" is derived from the
CASE REPORTS
Patient 1 This 47-year-old man was admitted with the signs and symptoms of acute appendicitis. He underwent an exploratory celiotomy. The operative findings were hyperemia of the small bowel and a normal appendix; the appendix was removed. His recovery was complicated by severe abdominal pain on the first day postoperatively, and on the second day he had abdominal distention with marked tenderness and an elevated leukocyte count of 20,000 per cu mm. The patient went into a state of shock. A flat x-ray plate of the abdomen revealed dilatation of the small bowel. After he was successfully resuscitated he underwent an exploratory celiotomy. The sur-
* Presented at the 34th Annual Meeting of the American Geriatrics Society, Fairmont Hotel, San Francisco, CA, April 13-14, 1977. ** Chairman, Department of Surgery, St. Clare's Hospital and Health Center; Associate Professor of Clinical Surgery at New York University. Correspondence to be addressed to: William F. Mitty, Jr., MD, St. Clare's Hospital and Health Center, 415 West 51st Street, New York, NY 10019. t Resident in Surgery, St. Clare's Hospital and Health Center.
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geon found 2 liters of serosanguinous fluid and 19 ft of gangrenous small bowel. Resection of the affected segment was undertaken, with end-toend anastomosis. The postoperative course was uneventful except for persistent diarrhea which was controlled by Lomotil. The patient was not given heparin. At the present time he is alive and well.
Patient 2 This 55-year-old women was admitted because of a transcervical fracture of the left femur, for which she underwent a Smith Peterson nailing operation. Her postoperative course was uneventful until the twenty-first day, when abdominal pain developed and the patient went into a state of shock. On physical examination she had a board-like abdomen with distention. A flat x-ray plate of the abdomen revealed free air under both diaphragms. After she was resuscitated she underwent an exploratory celiotomy. At operation the surgeon found 3 liters of serosanguinous fluid, and gangrenous bowel from the mid -jejunum to 6 em above the ileo-cecal valve. Resection, with an end-to-end anastomosis, was per-
formed. The patient's postoperative course was virtually uneventful. Anticoagulant therapy was started on the second post-operative day. The patient was discharged on the twenty-third postoperative day. She has been symptom-free for the past eleven months.
Patient 3 This 57-year-old women was admitted to the medical service because of confusion and a transient ischemic attack. She was progressing nicely when on the twenty-eighth hospital day generalized abdominal pain developed, with bloody stools; blood was also noted in the Levin tube. Abdominal physical examination revealed moderate distention and generalized tenderness. The leukocyte count was 29,400 per cu mm. Plain xray films of the abdomen revealed multiple loops of dilated small bowel. At operation the surgeon found 2 liters of serosanguinous fluid and gangrene of the distal jejunum and right colon. A resection of the involved segment was performed, and an ileo colostomy effected. This patient was given heparin intravenously, but died on the third postoperative day.
Fig. 1. Operative specimen from a patient with AVMT. Gross pathologic lesions in the intestine.
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.'
Fig . 2 . Histologic section from affected intesti nal segment in a case of AVMT.
Patient 4 This 88-year-old woman was admitted to the hospital with diffuse abdominal pain, nausea, vomiting and marked dehydration of three days' duration. She had undergone no previous operative procedures. She was in a state of profound shock and her abdomen was markedly distended and tender. The leukocyte count was 19,400 per cu mm . A flat x-ray plate of the abdomen revealed distended loops of small and large bowel , which showed air-fluid levels. At operation the surgeon found 3 liters of serosanguinous fluid, with 15 ft of gangrenous jejunum and ileum. A resection was performed, and end-to-end anastomosis effected. Th is patient died two hours following the completion of the operation.
two days prior to surgical consultation. Abdominal examination revealed abdominal rigidity with diffuse tenderness. The leukocyte count was 23,500 per cu mm. His temperature was 102°F. A flat x-ray plate of the abdomen demonstrated distended loops of the distal small bowel, and the right and transverse colon. That day, at operation, the surgeon found 1 liter of serosangu inous fluid with gangrene of the distal small bowel and ascending colon. A colon ic resection with an ileocolostomy was performed to restore intestinal continuity. The postoperative course was complicated by an anastomatic disruption; the resulting peritonitis required resection of the previous anastomosis. The patient received heparin after the first operation. He died on the third day following the second operation (the tenth day after the initial operation).
Patient 5 DISCUSSION This 58-year-old man was admitted with fractures of his 3rd, 4th, 5th and 6th ribs. He had not undergone any previous operation. On the thirteenth hospital day he started to complain of abdominal pain, vomiting and bloody stools for
516
Characteristics In summary, the characteristics of our five cases were:
November 1977
AGNOGENIC VENOUS MESENTERIC THROMBOSIS
1. The average age of the patients was 60 years. 2. Three patients had had no previous operations; one had had an appendectomy; and one had undergone a Smith Peterson nailing procedure for hip fracture. 3. All patients complained of severe diffuse abdominal pain of one to three days' duration. 4. Physical examination in all patients revealed various degrees of abdominal distention, rigidity and tenderness. 5. All patients were in a state of shock. 6. Flat x-ray plates of the abdomen revealed moderate to severe abdominal distention of either the small bowel alone or both the small and large bowels. 7. The leukocyte count averaged 20,000 per cu mm, with a marked shift to the left. 8. In all patients the operative findings consisted of 1-3 liters of serosanguinous fluid and the presence of gangrenous bowel. 9. Three patients were given heparin postoperatively. 10. The mortality rate was 60 percent. The reported mortality rates for this pathologic process vary from 30 to 50 percent (7-9). Such a high mortality rate is often due to delay in diagnosis because the manifestations of AVMT are not generally recognized by the medical profession. Unfortunately, the diagnosis is usually made at operation or at autopsy. However, Clemett and Chang (10) reported on characteristic radiologic features and noted that the aggressive use of radiologic procedures should indicate the diagnosis in suspected cases. Agnogenic venous mesenteric thrombosis (AVMT) is a clinicopathologic process which has definitive characteristics that separate it from venous mesenteric thrombosis (VMT), e.g., as seen in women taking oral contraceptives (1114), or secondary to trauma, local infection, hernia, volvulus, malignant neoplasm (15), or concomitant arterial disease. It should also be distinguished etiologically from retrograde thrombosis secondary to portal hypertension in cirrhotic patients, which ultimately extends into the mesenteric veins, as described by Ottinger et al (16).
2. The involved bowel is congested, cyanotic and thickened by hemorrhagic fluid. 3. Absence of mucosal necrosis in many cases, especially when the disease is diagnosed early (7). 4. A large amount of serosanguinous peritoneal fluid. 5. Functional dilatation of the small bowel proximal to the affected thrombotic portion. 6. Mesenteric thickening secondary to edema, and discoloration secondary to hemorrhage (15). 7. The presence of arterial pulsations. 8. In cut sections, the thrombi are easily extracted. 9. The presence of fresh as well as organized thrombi, which indicates an intermittent as well as a progressive thrombosis (17). 10. Venous thrombi may extend into the mesentery of normal-appearing bowel (7, 11). 11. Partial thrombosis ofthe larger veins, with normal-appearing bowel (13). Venous obstruction may be only marginal, with spontaneous resolution, thus avoiding the catastrophes which usually befall these patients. It is believed that the thrombi usually originate in the larger arcuate veins and then are propagated to occlude the collateral blood flow; this, in
Pathologic findings AVMT, when diagnosed either in the operating room or the autopsy room, has certain manifestations. Represented grossly in Figure 1 and microscopically in Figure 2, these are as follows: 1. Segmental distribution.
Fig. 3. Flat x-ray plate of the abdomen, showing smallbowel obstruction.
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Vol. XXV
F ig. 4. Roentgenogram with contrast medium in small intestines, illustrating characteristic find ings in a patient with AVMT.
turn, causes the intestinal lesions observed clinically by physicians (18).
Clinical findings The clinical manifestations usually noted in AVMT patients are: 1. Symptoms that are insidious and progressive. 2. Prodromal period ranging from several hours to many days (12, 17). 3. The abdominal pain is disproportionate to the physical signs that abdominal examination elicits (8). 4. Nausea and vomiting are secondary symptoms. 5. Either constipation or diarrhea may be present. Alteration in bowel habits is not characteristic of the disease. In some series constipation is more common than diarrhea but in other series (17) diarrhea is more common. Melena is a key differential and prognostic sign in distinguishing pure mechanical obstruction from vascular impairment.
518
6. Moderate to marked leukocytosis is the only notable and consistent laboratory finding (7). 7. Abdominal distention is present in about 50 percent of the cases. 8. The absence of tympany indicates that the bowel at the affected site is filled with fluid and blood. 9. Body temperature may be normal in the early stages of the disease, but fever appears as the physiopathologic process progresses into frank gangrene and peritonitis. 10. Shock and peritonitis are late manifestations.
Radiologic findings In 1975 Clemett and Chang (10) first introduced the concept of studying patients with suspected venous mesenteric thrombosis by the use of contrast media introduced into the small intestine. In their series of 12 cases they reported 91 percent accuracy in substantiating this diagnosis. Previously (8), plain x-ray films of the abdo-
November 1977
AGNOGENIC VENOUS MESENTERIC THROMBOSIS
Fig. 5. Roentgenogram with contrast medium in small intestines, illustrating characteristic findings in a patient with AVMT.
men had revealed evidence of small-bowel obstruction but were otherwise nonspecific (see Fig. 3). In some investigative studies (19, 20) of plain films of the abdomen, however, certain signs of bowel obstruction were noted (rigid, fixed, thickwelled loops of bowel) which were not truly characteristic at AVMT. According to Clemett and Chang (10), a specific diagnosis can be made by the use of contrast media in the gastrointestinal tract in suspected cases of AVMT. The characteristics, seen in Figures 4 and 5, are as follows: 1. Marked thickening of the bowel wall and valvulae conniventes due to congestion and edema-the most prominent finding. 2. Mesenteric thickening, contributing to the separation of bowel loops. 3. The transition zone between involved and uninvolved bowel segments is usually long, and is characterized by progressive thickening of the valvulae to the point of obliteration and progressive thickening of the bowel wall. 4. Pseudotumors or "thumb-prints" secondary to focal hemorrhage are found occasionally. Selective superior mesenteric arteriography may be of help in making the differential diag-
nosis in AVMT. Arteries to the affected bowel segment show intense spasm and very slow, or no blood flow. There is marked and prolonged staining of the thickened bowel wall in the transition zone. No circulation is evident in the more severely affected bowel. There is no venous drainage from the involved bowel segment. Treatment
At present, the treatment of choice for AVMT is surgical intervention, unless the diagnosis can be made early by the radiologic procedures advocated by Clemett and Chang (10). Only in the early phases of the disease can anticoagulants reverse the process and obviate operative procedures. However, the use of anticoagulants in preoperative treatment has not had sufficient clinical trial to warrant its advocacy as the sole method of therapy. The surgical treatment of AVMT involves wide resection of the diseased bowel segment. Nevertheless, the incidence of postoperative recurrence is reported to be 29 percent, and most recurrences occur within eleven days after operation. Apparently the use of anticoagulation therapy postop-
519
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eratively can prevent recurrence in almost all cases (9). The mortality rate is between 50 and 60 percent (7, 8). Inahara (21) treated AVMT successfully by thrombectomy without resection. . SUMMARY In the five cases of AVMT presented here, the average age of the patients was 60 years. The chief presenting signs and symptoms were severe abdominal pain, shock, and leukocytosis. At operation, the surgeon found a large amount of serosanguinous fluid and extensive gangrene of the bowel. The findings in a flat x-ray plate of the abdomen are not specifically diagnostic of AVMT, but contrast air studies of the intestinal tract reveal certain characteristics which indicate the diagnosis preoperatively in about 93 percent of the patients. Treatment can be divided into three phases: 1) if early diagnosis is established, anticoagulant therapy may reverse the course of the disease; 2) iffrank gangrene ofthe bowel is present, surgical resection is the treatment of choice; and 3) in the postoperative period, anticoagulant therapy should be given to prevent a recurrence. AVMT carries a mortality rate between 50 and 60 percent. To prevent this appalling mortality, the physician should exercise a high index of suspicion so that in the early phases of AVMT the diagnosis can be established and treatment started promptly and vigorously. REFERENCES 1. Albutt C: The Historical Relations of Medicine and Surgery. London, Macmillan & Co., Ltd., 1905.
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2. Tiedman F: Von der Verengerung and Schliessung der Pulsadern in Krankheiten. Leipzig, K. Gross, 1843. 3. Moore T: Mesenteric vascular occlusion, Brit J Surg 28: 347, 1941. 4. Elliot JW: The operative relief of gangrene of the intestine due to occlusion of the mesenteric vessels, Ann Surg 21: 9, 1895. 5. Jackson JM, Allen CA and Quimby WC: Mesenteric embolism and thrombosis, JAMA 42: 1469, 1904; 43: 25, 194~ 43: 110, 1904; 43: 183, 1904. 6. Donaldson JK and Stout BF: Mesenteric thrombosis, Am J Surg 29: 208, 1935. 7. Naitove A and Weismann RE: Primary mesenteric venous thrombosis, Ann Surg 161: 516, 1965. 8. Mathew JE and White RR: Primary mesenteric venous occlusive disease, Am J Surg 122: 579, 1971. 9. Jenson CB and Smith GA: Clinical study of 51 cases of mesenteric infarction, Surgery 40: 930, 1956. 10. Clemett AR and Chang J: The radiologic diagnosis of spontaneous mesenteric venous thrombosis, Am J Gastroenterol 63: 209, 1975. 11. Civetta JM and Kolodny M: Mesenteric venous thrombosis associated with oral contraceptives, Gastroenterology 58: 713, 1970. 12. Rose MB: Superior mesenteric vein thrombosis and oral contraceptives, Postgrad Med J 48: 430, 1972. 13. Miller DR: Unusual focal mesenteric venous thrombosis associated with contraceptive mediation, Ann Surg 173: 135, 1971. 14. Ellis DL and Heifetz CJ: Mesenteric venous thrombosis in two women taking oral contraceptives, Am J Surg 125: 641, 1973. 15. Bussey CD: Primary mesenteric venous thrombosis, Arch Surg 71: 688, 1955. 16. Ottinger LW and Austen WG: Study of 136 patients with mesenteric infarction, Surg Gynec Obst 124: 251, 1967. 17. Berry FB and Bougas JA: Agnogenic venous mesenteric thrombosis, Ann Surg 132: 450, 1950. 18. Hessen I: Roentgen examination in cases of occlusion of the mesenteric vessels, Acta Radiol 44: 293, 1955. 19. Nelson SW and Eggleston W: Findings on plain roentgenograms of the abdomen associated with mesenteric vascular occlusion with a possible new sign of mesenteric venous thrombosis, Am J Roentgenol 83: 886, 1960. 20. Wang CC and Reeves JD: Mesenteric vascular disease, Am J Roentgenol 83: 886, 1960. 21. Inahara T: Acute mesenteric venous thrombosis; treatment by thrombectomy, Ann Surg 174: 956, 1971.
JOURNAL OF THE AMERICAN GERIATRICS SOCIETY Copyright © 1977 by the American Geriatrics Society
Agnogenic Venous Mesenteric Thrombosis WILLIAM F. MITTY, JR, MD** and CLEMENTINO O. PURISIMA, MDt
St. Clare's Hospital and Health Center, New York, NY ABSTRACT: One of the most difficult diagnoses to establish is that of agnogenic venous mesenteric thrombosis (AVMT). This disorder occurs chiefly in elderly patients and, unless diagnosed promptly, leads to death in most instances. AVMT may follow surgical operations or occur during a prolonged illness. In the past five years at St. Clare's Hospital and Health Center in New York City, this diagnosis was established in five patients. In reviewing these cases, it was noted that some of the signs and symptoms such as those due to hypotension and shock with marked leukocytosis, were out of proportion to those usually observed when the preoperative diagnosis is being considered. The pathologic and radiologic characteristics of this disorder are outlined, and the recommended operative procedure for treatment is discussed. The importance is stressed of prompt, vigorous and prolonged anticoagulation therapy in order to minimize the chance of recurrence in the early postoperative period. Anticoagulant therapy is also effective preoperatively, if the disease is diagnosed sufficiently early. As greater numbers of elderly patients are being treated in hospitals, this dire complication should be uppermost in the minds of physicians and surgeons if a fatal outcome is to be avoided following a successful operative procedure.
Greek "agnes" (unknown), and is thus applied to diseases of unknown or obscure etiology. During the past five years, five cases of AVMT were diagnosed at the St. Clare's Hospital and Health Center of New York City. The highlights of these cases are presented, to show the dilemma which exists in making this diagnosis early enough to assure recovery.
Agnogenic venous mesenteric thrombosis (AVMT) is an entity first described by Beniviene (1) in the latter part of the fifteenth century. However, it was not appreciated by the medical profession until reports emanated from the clinics of Tiedman (2) in 1843 and Virchow (3) in 1847 and 1854. Elliot (4) in 1895 was the first author to report a patient surviving this pathologic condition. From that era to the present, there have been many articles (5) on mesenteric thrombosis but the authors failed to distinguish the arterial from the venous form. Donaldson and Stout (6) in 1935 first pointed out that venous mesenteric thrombosis was an entity separate from arterial thrombosis. According to Dorland's Medical Dictionary (25th edition), "agnogenic" is derived from the
CASE REPORTS
Patient 1 This 47-year-old man was admitted with the signs and symptoms of acute appendicitis. He underwent an exploratory celiotomy. The operative findings were hyperemia of the small bowel and a normal appendix; the appendix was removed. His recovery was complicated by severe abdominal pain on the first day postoperatively, and on the second day he had abdominal distention with marked tenderness and an elevated leukocyte count of 20,000 per cu mm. The patient went into a state of shock. A flat x-ray plate of the abdomen revealed dilatation of the small bowel. After he was successfully resuscitated he underwent an exploratory celiotomy. The sur-
* Presented at the 34th Annual Meeting of the American Geriatrics Society, Fairmont Hotel, San Francisco, CA, April 13-14, 1977. ** Chairman, Department of Surgery, St. Clare's Hospital and Health Center; Associate Professor of Clinical Surgery at New York University. Correspondence to be addressed to: William F. Mitty, Jr., MD, St. Clare's Hospital and Health Center, 415 West 51st Street, New York, NY 10019. t Resident in Surgery, St. Clare's Hospital and Health Center.
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geon found 2 liters of serosanguinous fluid and 19 ft of gangrenous small bowel. Resection of the affected segment was undertaken, with end-toend anastomosis. The postoperative course was uneventful except for persistent diarrhea which was controlled by Lomotil. The patient was not given heparin. At the present time he is alive and well.
Patient 2 This 55-year-old women was admitted because of a transcervical fracture of the left femur, for which she underwent a Smith Peterson nailing operation. Her postoperative course was uneventful until the twenty-first day, when abdominal pain developed and the patient went into a state of shock. On physical examination she had a board-like abdomen with distention. A flat x-ray plate of the abdomen revealed free air under both diaphragms. After she was resuscitated she underwent an exploratory celiotomy. At operation the surgeon found 3 liters of serosanguinous fluid, and gangrenous bowel from the mid -jejunum to 6 em above the ileo-cecal valve. Resection, with an end-to-end anastomosis, was per-
formed. The patient's postoperative course was virtually uneventful. Anticoagulant therapy was started on the second post-operative day. The patient was discharged on the twenty-third postoperative day. She has been symptom-free for the past eleven months.
Patient 3 This 57-year-old women was admitted to the medical service because of confusion and a transient ischemic attack. She was progressing nicely when on the twenty-eighth hospital day generalized abdominal pain developed, with bloody stools; blood was also noted in the Levin tube. Abdominal physical examination revealed moderate distention and generalized tenderness. The leukocyte count was 29,400 per cu mm. Plain xray films of the abdomen revealed multiple loops of dilated small bowel. At operation the surgeon found 2 liters of serosanguinous fluid and gangrene of the distal jejunum and right colon. A resection of the involved segment was performed, and an ileo colostomy effected. This patient was given heparin intravenously, but died on the third postoperative day.
Fig. 1. Operative specimen from a patient with AVMT. Gross pathologic lesions in the intestine.
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.'
Fig . 2 . Histologic section from affected intesti nal segment in a case of AVMT.
Patient 4 This 88-year-old woman was admitted to the hospital with diffuse abdominal pain, nausea, vomiting and marked dehydration of three days' duration. She had undergone no previous operative procedures. She was in a state of profound shock and her abdomen was markedly distended and tender. The leukocyte count was 19,400 per cu mm . A flat x-ray plate of the abdomen revealed distended loops of small and large bowel , which showed air-fluid levels. At operation the surgeon found 3 liters of serosanguinous fluid, with 15 ft of gangrenous jejunum and ileum. A resection was performed, and end-to-end anastomosis effected. Th is patient died two hours following the completion of the operation.
two days prior to surgical consultation. Abdominal examination revealed abdominal rigidity with diffuse tenderness. The leukocyte count was 23,500 per cu mm. His temperature was 102°F. A flat x-ray plate of the abdomen demonstrated distended loops of the distal small bowel, and the right and transverse colon. That day, at operation, the surgeon found 1 liter of serosangu inous fluid with gangrene of the distal small bowel and ascending colon. A colon ic resection with an ileocolostomy was performed to restore intestinal continuity. The postoperative course was complicated by an anastomatic disruption; the resulting peritonitis required resection of the previous anastomosis. The patient received heparin after the first operation. He died on the third day following the second operation (the tenth day after the initial operation).
Patient 5 DISCUSSION This 58-year-old man was admitted with fractures of his 3rd, 4th, 5th and 6th ribs. He had not undergone any previous operation. On the thirteenth hospital day he started to complain of abdominal pain, vomiting and bloody stools for
516
Characteristics In summary, the characteristics of our five cases were:
November 1977
AGNOGENIC VENOUS MESENTERIC THROMBOSIS
1. The average age of the patients was 60 years. 2. Three patients had had no previous operations; one had had an appendectomy; and one had undergone a Smith Peterson nailing procedure for hip fracture. 3. All patients complained of severe diffuse abdominal pain of one to three days' duration. 4. Physical examination in all patients revealed various degrees of abdominal distention, rigidity and tenderness. 5. All patients were in a state of shock. 6. Flat x-ray plates of the abdomen revealed moderate to severe abdominal distention of either the small bowel alone or both the small and large bowels. 7. The leukocyte count averaged 20,000 per cu mm, with a marked shift to the left. 8. In all patients the operative findings consisted of 1-3 liters of serosanguinous fluid and the presence of gangrenous bowel. 9. Three patients were given heparin postoperatively. 10. The mortality rate was 60 percent. The reported mortality rates for this pathologic process vary from 30 to 50 percent (7-9). Such a high mortality rate is often due to delay in diagnosis because the manifestations of AVMT are not generally recognized by the medical profession. Unfortunately, the diagnosis is usually made at operation or at autopsy. However, Clemett and Chang (10) reported on characteristic radiologic features and noted that the aggressive use of radiologic procedures should indicate the diagnosis in suspected cases. Agnogenic venous mesenteric thrombosis (AVMT) is a clinicopathologic process which has definitive characteristics that separate it from venous mesenteric thrombosis (VMT), e.g., as seen in women taking oral contraceptives (1114), or secondary to trauma, local infection, hernia, volvulus, malignant neoplasm (15), or concomitant arterial disease. It should also be distinguished etiologically from retrograde thrombosis secondary to portal hypertension in cirrhotic patients, which ultimately extends into the mesenteric veins, as described by Ottinger et al (16).
2. The involved bowel is congested, cyanotic and thickened by hemorrhagic fluid. 3. Absence of mucosal necrosis in many cases, especially when the disease is diagnosed early (7). 4. A large amount of serosanguinous peritoneal fluid. 5. Functional dilatation of the small bowel proximal to the affected thrombotic portion. 6. Mesenteric thickening secondary to edema, and discoloration secondary to hemorrhage (15). 7. The presence of arterial pulsations. 8. In cut sections, the thrombi are easily extracted. 9. The presence of fresh as well as organized thrombi, which indicates an intermittent as well as a progressive thrombosis (17). 10. Venous thrombi may extend into the mesentery of normal-appearing bowel (7, 11). 11. Partial thrombosis ofthe larger veins, with normal-appearing bowel (13). Venous obstruction may be only marginal, with spontaneous resolution, thus avoiding the catastrophes which usually befall these patients. It is believed that the thrombi usually originate in the larger arcuate veins and then are propagated to occlude the collateral blood flow; this, in
Pathologic findings AVMT, when diagnosed either in the operating room or the autopsy room, has certain manifestations. Represented grossly in Figure 1 and microscopically in Figure 2, these are as follows: 1. Segmental distribution.
Fig. 3. Flat x-ray plate of the abdomen, showing smallbowel obstruction.
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Vol. XXV
F ig. 4. Roentgenogram with contrast medium in small intestines, illustrating characteristic find ings in a patient with AVMT.
turn, causes the intestinal lesions observed clinically by physicians (18).
Clinical findings The clinical manifestations usually noted in AVMT patients are: 1. Symptoms that are insidious and progressive. 2. Prodromal period ranging from several hours to many days (12, 17). 3. The abdominal pain is disproportionate to the physical signs that abdominal examination elicits (8). 4. Nausea and vomiting are secondary symptoms. 5. Either constipation or diarrhea may be present. Alteration in bowel habits is not characteristic of the disease. In some series constipation is more common than diarrhea but in other series (17) diarrhea is more common. Melena is a key differential and prognostic sign in distinguishing pure mechanical obstruction from vascular impairment.
518
6. Moderate to marked leukocytosis is the only notable and consistent laboratory finding (7). 7. Abdominal distention is present in about 50 percent of the cases. 8. The absence of tympany indicates that the bowel at the affected site is filled with fluid and blood. 9. Body temperature may be normal in the early stages of the disease, but fever appears as the physiopathologic process progresses into frank gangrene and peritonitis. 10. Shock and peritonitis are late manifestations.
Radiologic findings In 1975 Clemett and Chang (10) first introduced the concept of studying patients with suspected venous mesenteric thrombosis by the use of contrast media introduced into the small intestine. In their series of 12 cases they reported 91 percent accuracy in substantiating this diagnosis. Previously (8), plain x-ray films of the abdo-
November 1977
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Fig. 5. Roentgenogram with contrast medium in small intestines, illustrating characteristic findings in a patient with AVMT.
men had revealed evidence of small-bowel obstruction but were otherwise nonspecific (see Fig. 3). In some investigative studies (19, 20) of plain films of the abdomen, however, certain signs of bowel obstruction were noted (rigid, fixed, thickwelled loops of bowel) which were not truly characteristic at AVMT. According to Clemett and Chang (10), a specific diagnosis can be made by the use of contrast media in the gastrointestinal tract in suspected cases of AVMT. The characteristics, seen in Figures 4 and 5, are as follows: 1. Marked thickening of the bowel wall and valvulae conniventes due to congestion and edema-the most prominent finding. 2. Mesenteric thickening, contributing to the separation of bowel loops. 3. The transition zone between involved and uninvolved bowel segments is usually long, and is characterized by progressive thickening of the valvulae to the point of obliteration and progressive thickening of the bowel wall. 4. Pseudotumors or "thumb-prints" secondary to focal hemorrhage are found occasionally. Selective superior mesenteric arteriography may be of help in making the differential diag-
nosis in AVMT. Arteries to the affected bowel segment show intense spasm and very slow, or no blood flow. There is marked and prolonged staining of the thickened bowel wall in the transition zone. No circulation is evident in the more severely affected bowel. There is no venous drainage from the involved bowel segment. Treatment
At present, the treatment of choice for AVMT is surgical intervention, unless the diagnosis can be made early by the radiologic procedures advocated by Clemett and Chang (10). Only in the early phases of the disease can anticoagulants reverse the process and obviate operative procedures. However, the use of anticoagulants in preoperative treatment has not had sufficient clinical trial to warrant its advocacy as the sole method of therapy. The surgical treatment of AVMT involves wide resection of the diseased bowel segment. Nevertheless, the incidence of postoperative recurrence is reported to be 29 percent, and most recurrences occur within eleven days after operation. Apparently the use of anticoagulation therapy postop-
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eratively can prevent recurrence in almost all cases (9). The mortality rate is between 50 and 60 percent (7, 8). Inahara (21) treated AVMT successfully by thrombectomy without resection. . SUMMARY In the five cases of AVMT presented here, the average age of the patients was 60 years. The chief presenting signs and symptoms were severe abdominal pain, shock, and leukocytosis. At operation, the surgeon found a large amount of serosanguinous fluid and extensive gangrene of the bowel. The findings in a flat x-ray plate of the abdomen are not specifically diagnostic of AVMT, but contrast air studies of the intestinal tract reveal certain characteristics which indicate the diagnosis preoperatively in about 93 percent of the patients. Treatment can be divided into three phases: 1) if early diagnosis is established, anticoagulant therapy may reverse the course of the disease; 2) iffrank gangrene ofthe bowel is present, surgical resection is the treatment of choice; and 3) in the postoperative period, anticoagulant therapy should be given to prevent a recurrence. AVMT carries a mortality rate between 50 and 60 percent. To prevent this appalling mortality, the physician should exercise a high index of suspicion so that in the early phases of AVMT the diagnosis can be established and treatment started promptly and vigorously. REFERENCES 1. Albutt C: The Historical Relations of Medicine and Surgery. London, Macmillan & Co., Ltd., 1905.
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2. Tiedman F: Von der Verengerung and Schliessung der Pulsadern in Krankheiten. Leipzig, K. Gross, 1843. 3. Moore T: Mesenteric vascular occlusion, Brit J Surg 28: 347, 1941. 4. Elliot JW: The operative relief of gangrene of the intestine due to occlusion of the mesenteric vessels, Ann Surg 21: 9, 1895. 5. Jackson JM, Allen CA and Quimby WC: Mesenteric embolism and thrombosis, JAMA 42: 1469, 1904; 43: 25, 194~ 43: 110, 1904; 43: 183, 1904. 6. Donaldson JK and Stout BF: Mesenteric thrombosis, Am J Surg 29: 208, 1935. 7. Naitove A and Weismann RE: Primary mesenteric venous thrombosis, Ann Surg 161: 516, 1965. 8. Mathew JE and White RR: Primary mesenteric venous occlusive disease, Am J Surg 122: 579, 1971. 9. Jenson CB and Smith GA: Clinical study of 51 cases of mesenteric infarction, Surgery 40: 930, 1956. 10. Clemett AR and Chang J: The radiologic diagnosis of spontaneous mesenteric venous thrombosis, Am J Gastroenterol 63: 209, 1975. 11. Civetta JM and Kolodny M: Mesenteric venous thrombosis associated with oral contraceptives, Gastroenterology 58: 713, 1970. 12. Rose MB: Superior mesenteric vein thrombosis and oral contraceptives, Postgrad Med J 48: 430, 1972. 13. Miller DR: Unusual focal mesenteric venous thrombosis associated with contraceptive mediation, Ann Surg 173: 135, 1971. 14. Ellis DL and Heifetz CJ: Mesenteric venous thrombosis in two women taking oral contraceptives, Am J Surg 125: 641, 1973. 15. Bussey CD: Primary mesenteric venous thrombosis, Arch Surg 71: 688, 1955. 16. Ottinger LW and Austen WG: Study of 136 patients with mesenteric infarction, Surg Gynec Obst 124: 251, 1967. 17. Berry FB and Bougas JA: Agnogenic venous mesenteric thrombosis, Ann Surg 132: 450, 1950. 18. Hessen I: Roentgen examination in cases of occlusion of the mesenteric vessels, Acta Radiol 44: 293, 1955. 19. Nelson SW and Eggleston W: Findings on plain roentgenograms of the abdomen associated with mesenteric vascular occlusion with a possible new sign of mesenteric venous thrombosis, Am J Roentgenol 83: 886, 1960. 20. Wang CC and Reeves JD: Mesenteric vascular disease, Am J Roentgenol 83: 886, 1960. 21. Inahara T: Acute mesenteric venous thrombosis; treatment by thrombectomy, Ann Surg 174: 956, 1971.
