Editorial Spontaneous Rupture of the Spleen in Patients With Infectious Mononucleosis Splenomegaly is a well-recognized finding in patients with infectious mononucleosis (1M). Because of the enlargement of the spleen and the intrinsic changes in the splenic parenchyma during the clinical course of 1M, the spleen is susceptible to rupture with minimal trauma. Even without a notable traumatic insult, patients with 1M may experience spontaneous splenic rupture. In this issue of the Mayo Clinic Proceedings (pages 846 to 853), Farley and associates analyze five cases of straightforward spontaneous rupture of the spleen in patients with 1M and describe four other patients in whom this entity was strongly suggested but not fully confirmed. In 1967, Sakulsky and colleagues' described one case of ruptured spleen in a patient with 1M. That report and the current article by Farley and colleagues (which includes the case reported by Sakulsky and co-workers' in its overall tally) are the only two publications on this subject from the Mayo Clinic, where 8,116 patients had the diagnosis ofIM during a 40-year period. The identification of only five fully verified and four suggestive cases in this large patient population indicates the very low incidence of spontaneous splenic rupture in patients with 1M. When I searched for cases of spontaneous splenic rupture in patients with 1M in the medical records at my institution, I found only one such case. In addition to describing the nine aforementioned cases in their article, Farley and associates skillfully and thoroughly review the available literature and their own experience to ascertain the most important aspects of spontaneous rupture of the spleen in patients with 1M. The validity of their analysis is enhanced by the fact that they used previously well-established and strict criteria'> to ensure that only patients with fully substantiated 1M were included in their study. Frequency of Occurrence-s-The first confirmed case of 1M complicated by splenic rupture was reported by King" in 1941. Smith and Custer,' who found 44 cases of spontaneous rupture of the spleen in the literature and their own experience, stated that 1M was second only to malaria as a Address reprint requests to Dr, J. S. Aldrete, Department of Surgery, Room 406, Kracke Building, University of Alabama Medical Center, 1922 7th Avenue South, Birmingham, AL 35294-0007. Mayo Clin Proc 67:910-912, 1992
cause of splenic rupture. Currently, 1M may be the most common cause of spontaneous splenic rupture. In 1987, McLean and colleagues" reported that the most frequent cause of death in patients with 1M is sudden splenic rupture, an apparently accurate conclusion because of the self-limiting clinical course observed in most patients with 1M. Because many of these cases may never be reported, however, the exact incidence of spontaneous splenic rupture in 1M and any subsequent morbidity and mortality remain unknown. Early Recognition.-The current article by Farley and co-workers achieves two important objectives: (1) dissemination of the information that this complication, although rare, does occur and necessitates prompt recognition for successful immediate treatment and (2) analysis of whether nonoperative treatment should be attempted in selected cases. When this rare complication of 1M occurs, it poses a substantial risk to the life of patients who otherwise would fully recover from this insidious, uncommon, but seldom fatal disease that affects primarily young adults. Therefore, early recognition is important. Diagnostic-Considerations.-The current availability of computed tomography allows confirmation of the presumptive diagnosis in those patients who are hemodynamically stable and can safely undergo the diagnostic imaging procedure. Once the diagnosis of splenic rupture has been established by computed tomography, a difficult decision must be made about whether to perform prompt celiotomy or to observe the patient closely in the hope of avoiding celiotomy and splenectomy. Any patient with confirmed, or even suspected, 1M in whom abdominal pain develops (present in approximately 90% of the cases described by Farley and associates) and extends to the left supraclavicular region (a more accurate location than the left shoulder) should be highly suspected of having splenic rupture. This impression will be strongly reinforced by the following findings on physical examination: splenomegaly, tender spleen, and, particularly, signs of peritoneal irritation. Even without evidence of peritoneal irritation, the diagnosis of splenic rupture should be seriously considered. Operative Versus Nonoperative Management.-In patients who have hemodynamic instability suggestive of hypovolemic shock, in combination with the presence of the previously noted symptoms and signs, immediate treatment for sudden blood loss should be initiated, and urgent celiotomy to arrest the bleeding from the ruptured spleen should be done without delay. More than 60% of patients 910
Mayo elin Proc, September 1992, Vol 67
with this complication, however, have no signs or symptoms of acute blood loss; in this group of patients, the diagnosis can be confirmed by computed tomography. If such a patient remains hemodynamically stable, nonoperative treatment can be considered as an alternative. Currently, some traumatic lesions of the otherwise normal spleen can be managed safely by close observation and bed rest; therefore, this therapeutic option might logically be considered for patients with 1M who have spontaneous rupture of the spleen. Farley and colleagues discuss in detail the pertinent information available in the literature. A few reports of isolated cases document the feasibility of this therapeutic approach; however, several investigators suggest or conclude that nonoperative management of the ruptured spleen in patients with 1M is too risky and perhaps ineffective. Many patients initially treated nonoperatively eventually must undergo celiotomy after several days or weeks of hospitalization and transfusion of excessive amounts of blood. This outcome suggests that the selection of cases of spontaneous splenic rupture is too imprecise to avoid celiotomy. The number of patients described in the literature who were selected for nonoperative treatment and subsequently required massive blood transfusions and splenectomy strongly detract from selecting nonoperative treatment. Limiting the number of blood transfusions to be given within a specific period (40 ml of blood/kg of body weight during a period of 24 hours is a frequently used maximum) should help determine when a patient should be transferred from the nonoperative to the operative therapy group. Use of Computed Tomography.-In patients with 1M, perhaps because of the congestive splenomegaly, an abnormal splenic parenchyma, and a weakened splenic capsule, intraparenchymal or subcapsular splenic hematomas are susceptible to rupture into the peritoneal cavity, and the concomitant sudden blood loss can become a life-threatening situation. Some patients with 1M have symptoms and signs of spontaneous splenic rupture but are completely stable hemodynamically; in this circumstance, the physician might elect to confirm the diagnosis by computed tomography. A blood sample should be obtained from such patients for typing and crossmatching of at least three units of blood to be available if needed. After insertion of a large-diameter intravenous line, the patient should be taken to the radiology suite for computed tomographic scanning. Ideally, a member of the surgical team should accompany the patient at all times to monitor the hemodynamics and to implement a preestablished plan of therapy (for example, prompt transfusion and transfer to the operating room) if hemodynamic instability suddenly ensues when the patient is in the radiology suite or subsequently in the surgical intensive-care unit. If the patient's condition remains stable during and
EDITORIAL
911
after computed tomography and if the images show a contained subcapsular hematoma of modest size, only in such selected patients can the nonoperative observation plan be implemented, after the patient and relatives have been fully advised of the limitations and risks of this therapeutic option. If the computed tomographic images show a deep laceration in the parenchyma and a large subcapsular hematoma, the probability of rupture of such a lesion in an enlarged spleen is extremely high. Therefore, such patients should undergo immediate celiotomy. Preservation ofthe Spleen.-Farley and co-workers also discuss the type of operative management that should be used. Specifically, the issue of attempting splenic salvage rather than performing complete splenectomy is addressed. Splenic function can be preserved by surgically repairing the laceration (splenorrhaphy) or by removing only the part of the spleen involved with the rupture (partial splenectomy). These surgical techniques of splenic preservation have been feasible and effective, particularly in children with traumatically ruptured but otherwise normal spleens. In my opinion, however, in patients with 1M who have splenomegaly and increased fragility of the splenic parenchyma and capsule, any type of splenorrhaphy or partial splenectomy would have minimal probability of success and therefore would only increase the morbidity and mortality associated with this complication of 1M. Furthermore, the main reason for using such spleen-salvaging techniques would be to avoid the postsplenectomy sepsis syndrome, which occurs much more frequently in children than in adults. Because most patients with 1M are beyond the age of substantial risk for developing postsplenectomy sepsis, I agree with Farley and associates that spleen-salvaging techniques or autotransplantation of splenic tissue is not indicated and that total splenectomy is the procedure of choice in a patient with 1M and a ruptured spleen. Conclusion.-All physicians should be aware of the rare but possible occurrence of splenic rupture in patients with 1M. This self-limiting disease that occurs predominantly in young adults can be associated with this potentially fatal complication. Familiarity with the specific signs and symptoms is important for early detection, and computed tomography can be used to confirm the diagnosis. With prompt implementation of a well-defined plan of therapy, the associated morbidity and mortality may be minimized or eliminated. Joaquin S. Aldrete, M.D. Department of Surgery University of Alabama School of Medicine Birmingham, Alabama
912
EDITORIAL
REFERENCES 1. Sakulsky SB, Wallace RB, Silverstein MN, Dockerty MB: Ruptured spleen in infectious mononucleosis. Arch Surg 94:349-352, 1967 2. Orloff MJ, Peskin GW: Spontaneous rupture of the normal spleen: a surgical enigma. Int Abstr Surg 106:1-11,1958 3. Rutkow 1M: Rupture of the spleen in infectious mononucleosis: a critical review. Arch Surg 113:718-720, 1978
Mayo Clin Proc, September 1992, Vol 67
4. King RB: Spontaneous rupture of the spleen in infectious mononucleosis: report of a case. N Engl J Med 224:10581060, 1941 5. Smith EB, Custer RP: Rupture of the spleen in infectious mononucleosis: a clinicopathologic report of seven cases. Blood 1:317-333,1946 6. McLean ER Jr, Diehl W, Edoga JK, Widmann WD: Failure of conservative management of splenic rupture in a patient with mononucleosis. J Pediatr Surg 22: 1034-1035, 1987
cause of splenic rupture. Currently, 1M may be the most common cause of spontaneous splenic rupture. In 1987, McLean and colleagues" reported that the most frequent cause of death in patients with 1M is sudden splenic rupture, an apparently accurate conclusion because of the self-limiting clinical course observed in most patients with 1M. Because many of these cases may never be reported, however, the exact incidence of spontaneous splenic rupture in 1M and any subsequent morbidity and mortality remain unknown. Early Recognition.-The current article by Farley and co-workers achieves two important objectives: (1) dissemination of the information that this complication, although rare, does occur and necessitates prompt recognition for successful immediate treatment and (2) analysis of whether nonoperative treatment should be attempted in selected cases. When this rare complication of 1M occurs, it poses a substantial risk to the life of patients who otherwise would fully recover from this insidious, uncommon, but seldom fatal disease that affects primarily young adults. Therefore, early recognition is important. Diagnostic-Considerations.-The current availability of computed tomography allows confirmation of the presumptive diagnosis in those patients who are hemodynamically stable and can safely undergo the diagnostic imaging procedure. Once the diagnosis of splenic rupture has been established by computed tomography, a difficult decision must be made about whether to perform prompt celiotomy or to observe the patient closely in the hope of avoiding celiotomy and splenectomy. Any patient with confirmed, or even suspected, 1M in whom abdominal pain develops (present in approximately 90% of the cases described by Farley and associates) and extends to the left supraclavicular region (a more accurate location than the left shoulder) should be highly suspected of having splenic rupture. This impression will be strongly reinforced by the following findings on physical examination: splenomegaly, tender spleen, and, particularly, signs of peritoneal irritation. Even without evidence of peritoneal irritation, the diagnosis of splenic rupture should be seriously considered. Operative Versus Nonoperative Management.-In patients who have hemodynamic instability suggestive of hypovolemic shock, in combination with the presence of the previously noted symptoms and signs, immediate treatment for sudden blood loss should be initiated, and urgent celiotomy to arrest the bleeding from the ruptured spleen should be done without delay. More than 60% of patients 910
Mayo elin Proc, September 1992, Vol 67
with this complication, however, have no signs or symptoms of acute blood loss; in this group of patients, the diagnosis can be confirmed by computed tomography. If such a patient remains hemodynamically stable, nonoperative treatment can be considered as an alternative. Currently, some traumatic lesions of the otherwise normal spleen can be managed safely by close observation and bed rest; therefore, this therapeutic option might logically be considered for patients with 1M who have spontaneous rupture of the spleen. Farley and colleagues discuss in detail the pertinent information available in the literature. A few reports of isolated cases document the feasibility of this therapeutic approach; however, several investigators suggest or conclude that nonoperative management of the ruptured spleen in patients with 1M is too risky and perhaps ineffective. Many patients initially treated nonoperatively eventually must undergo celiotomy after several days or weeks of hospitalization and transfusion of excessive amounts of blood. This outcome suggests that the selection of cases of spontaneous splenic rupture is too imprecise to avoid celiotomy. The number of patients described in the literature who were selected for nonoperative treatment and subsequently required massive blood transfusions and splenectomy strongly detract from selecting nonoperative treatment. Limiting the number of blood transfusions to be given within a specific period (40 ml of blood/kg of body weight during a period of 24 hours is a frequently used maximum) should help determine when a patient should be transferred from the nonoperative to the operative therapy group. Use of Computed Tomography.-In patients with 1M, perhaps because of the congestive splenomegaly, an abnormal splenic parenchyma, and a weakened splenic capsule, intraparenchymal or subcapsular splenic hematomas are susceptible to rupture into the peritoneal cavity, and the concomitant sudden blood loss can become a life-threatening situation. Some patients with 1M have symptoms and signs of spontaneous splenic rupture but are completely stable hemodynamically; in this circumstance, the physician might elect to confirm the diagnosis by computed tomography. A blood sample should be obtained from such patients for typing and crossmatching of at least three units of blood to be available if needed. After insertion of a large-diameter intravenous line, the patient should be taken to the radiology suite for computed tomographic scanning. Ideally, a member of the surgical team should accompany the patient at all times to monitor the hemodynamics and to implement a preestablished plan of therapy (for example, prompt transfusion and transfer to the operating room) if hemodynamic instability suddenly ensues when the patient is in the radiology suite or subsequently in the surgical intensive-care unit. If the patient's condition remains stable during and
EDITORIAL
911
after computed tomography and if the images show a contained subcapsular hematoma of modest size, only in such selected patients can the nonoperative observation plan be implemented, after the patient and relatives have been fully advised of the limitations and risks of this therapeutic option. If the computed tomographic images show a deep laceration in the parenchyma and a large subcapsular hematoma, the probability of rupture of such a lesion in an enlarged spleen is extremely high. Therefore, such patients should undergo immediate celiotomy. Preservation ofthe Spleen.-Farley and co-workers also discuss the type of operative management that should be used. Specifically, the issue of attempting splenic salvage rather than performing complete splenectomy is addressed. Splenic function can be preserved by surgically repairing the laceration (splenorrhaphy) or by removing only the part of the spleen involved with the rupture (partial splenectomy). These surgical techniques of splenic preservation have been feasible and effective, particularly in children with traumatically ruptured but otherwise normal spleens. In my opinion, however, in patients with 1M who have splenomegaly and increased fragility of the splenic parenchyma and capsule, any type of splenorrhaphy or partial splenectomy would have minimal probability of success and therefore would only increase the morbidity and mortality associated with this complication of 1M. Furthermore, the main reason for using such spleen-salvaging techniques would be to avoid the postsplenectomy sepsis syndrome, which occurs much more frequently in children than in adults. Because most patients with 1M are beyond the age of substantial risk for developing postsplenectomy sepsis, I agree with Farley and associates that spleen-salvaging techniques or autotransplantation of splenic tissue is not indicated and that total splenectomy is the procedure of choice in a patient with 1M and a ruptured spleen. Conclusion.-All physicians should be aware of the rare but possible occurrence of splenic rupture in patients with 1M. This self-limiting disease that occurs predominantly in young adults can be associated with this potentially fatal complication. Familiarity with the specific signs and symptoms is important for early detection, and computed tomography can be used to confirm the diagnosis. With prompt implementation of a well-defined plan of therapy, the associated morbidity and mortality may be minimized or eliminated. Joaquin S. Aldrete, M.D. Department of Surgery University of Alabama School of Medicine Birmingham, Alabama
912
EDITORIAL
REFERENCES 1. Sakulsky SB, Wallace RB, Silverstein MN, Dockerty MB: Ruptured spleen in infectious mononucleosis. Arch Surg 94:349-352, 1967 2. Orloff MJ, Peskin GW: Spontaneous rupture of the normal spleen: a surgical enigma. Int Abstr Surg 106:1-11,1958 3. Rutkow 1M: Rupture of the spleen in infectious mononucleosis: a critical review. Arch Surg 113:718-720, 1978
Mayo Clin Proc, September 1992, Vol 67
4. King RB: Spontaneous rupture of the spleen in infectious mononucleosis: report of a case. N Engl J Med 224:10581060, 1941 5. Smith EB, Custer RP: Rupture of the spleen in infectious mononucleosis: a clinicopathologic report of seven cases. Blood 1:317-333,1946 6. McLean ER Jr, Diehl W, Edoga JK, Widmann WD: Failure of conservative management of splenic rupture in a patient with mononucleosis. J Pediatr Surg 22: 1034-1035, 1987
