ANSt. N . Z . J . SlW,?. (1995) 65, 247-250 ORIGINAL ARTICLE

THE MANAGEMENT OF SPLENIC RUPTURE IN INFECTIOUS MONONUCLEOSIS

M. K. GORDON,* J. A. RIETVELD~ AND F. A. FRIZELLE~ Department of Surgery, *Christchurch Hospital and 'Dunedin Hospital, Otago Medical School. Otago, New Zealand

Non-operative management of splenic trauma is now well established; however, the role of conservative management in spontaneous splenic rupture is undetermined. The leading cause of spontaneous splenic rupture is infectious mononucleosis. We report on the management of four patients with spontaneous rupture, in association with infectious mononucleosis. Three patients eventually required splenectomy, and one was successfully managed non-operatively. The comparative risks of operative and non-operative management are discussed. We believe that when splenic rupture complicates infectious mononucleosis, early splenectomy is the most appropriate management.

Key words: infectious mononucleosis, splenectomy, splenic rupture.

INTRODUCTION Infectious mononucleosis (IM) is a common disorder with an annual incidence of 25-50/1OO OOO. ' The predominant age range is through the second and third decades and the sex incidence is equal.* Finch emphasizes the generally benign and limited course.' Splenic rupture associated with IM, either spontaneous or following minor trauma, is poten- tially but uncommon, occurring in an estimated 0.5% of cases6

Splenic involvement is common, perhaps universal in IM' with splenomegaly being clinically evident in 25-5096' and detectable on ultrasound in 100% in the Dommerby series.x Histologically there is intense congestion of the red pulp, with infiltration of the capsule, supporting trabeculae, blood vessel walls and red pulp by lymphocytes and large atypical lymphoid c e h Y There is believed to be extensive dissolu- tion and fragmentation of the capsule and the fibrous support elements.'"

The weakened spleen is prone to both deep parenchymal and subcapsular haematoma, and to rupture. l o Splenic ven- ous congestion accompanying the Valsalva manoeuvre of straining is thought to precipitate splenic rupture. I I Gener- ally stated to occur during the second or third weeks after the onset of symptoms of IM (when splenomegaly is maxi- mal)," rupture has been reported from 3 days' to 10 weeks" after symptoms begin and also in the absence of symptoms'.' and after full serological and clinical recovery. 'I

Prompt splenectomy has generally been regarded as man- datory when splenic rupture complicates IM.s," However. many patients are haemodynamically stable on presentation, with a subcapsular haematoma and minimal free peritoneal haemorrhage,'3-'8 and because of the important role of the spleen in the immunological defence system, conservative management has recently been advocated.Ih We report our experiences with the management of four patients, two from Christchurch Hospital, and two from Dunedin Hospital, New Zealand.

Correspondence: Dr F. A. Frizelle. Surgical Skills Unit. Department of Surgery. Ninewells Medical School and Hospital. Dundee, Scotland.

Accepted for publication 2 I July 1994.

Case 1 A 22 year old male presented with an acute onset of pain in his chest and abdomen, radiating to his left shoulder. There was no history of any significant abdominal trauma. Nine months earlier he had sustained a splenic contusion while skiing which was treated conservatively and had completely resolved. On admission he was haemodynamically stable, his temperature was 38.5"C and he had a palpable cervical lymphadenopathy. Abdominal examination revealed local- ized left upper abdominal tenderness and guarding.

Laboratory data revealed a white cell count of 8.1 X 1O-'L with atypical lymphocytes and serology confirming IM infection. He remained haemodynamically stable despite his haemoglobin dropping from 12.2 gm/dL to 9.4 gm/dL. A computerized tomography (CT) scan revealed free intraperi- toneal blood and splenic rupture. He had an unremarkable hospital course and was discharged 10 days after admission. At 3 months ultrasound examination of the spleen showed resolution of the haematoma and normal splenic architecture.

Case 2 A 20 year old male presented with a 2 day history of intermittent abdominal pain radiating to the left shoulder. There was no history of recent trauma; however, he had symptoms of a systemic viral illness for several weeks. On admission he was haemodynamically stable, his temperature was 38.7"C and he had palpable cervical lymphadenopathy. Examination of the abdomen revealed localized left upper quadrant tenderness and guarding.

Laboratory data revealed a white count of 7.2 X IO-'L with atypical lymphocytes and serology confirmed IM infec- tion. Ultrasound examination revealed splenic rupture. He was initially treated with fluid replacement (including blood); however, he suddenly became haemodynamically unstable and was submitted to laparotomy and splenectomy 5 days after admission. A large amount of free blood was found in the peritoneum and splenectomy was performed with a total of 7 units of blood required to be transfused. His postopera- tive course was unremarkable and he was given pneumovax prior to discharge. At follow up 3 months later he had made a full recovery.

248 GORDON ET AL.

Case 3 A 20 year old male presented acutely with a 6 h history of abdominal pain, radiating to the left shoulder. There was no history of recent abdominal trauma; however, 2 weeks prior to presentation he had a severe upper respiratory tract infection. On admission he was haemodynamically stable, his temperature was 38.0”C and laboratory data revealed a white cell count of 12.4 X IO-‘L with atypical lymphocytes.

Examination revealed palpable cervical lymphadenopathy and his abdomen was tender and guarded. On the basis of his clinical presentation the diagnosis of ruptured viscus was made and the patient underwent laparotomy 4 h after admis- sion. Free blood was found in the peritoneum and the spleen was found to be ruptured. Splenectomy was performed and a total of 5 units of blood was required to be transfused. His postoperative course was unremarkable and the patient was given pneumovax prior to discharge. Follow up at 1 year revealed a complete recovery from his surgery. Histological examination of the spleen revealed changes consistent with IM infection and subsequent serology confirmed IM infection.

Case 4 A 57 year old male presented with an acute myocardial infarction which was treated by thrombolysis. Three hours post-thrombolysis he developed sudden pain in his left upper abdomen without any precipitating trauma. He be- came tachycardic and hypotensive. He was initially treated conservatively with fluid replacement including blood.

Laboratory data revealed a white cell count of 1 1.2 X IO-’L with atypical lymphocytes. A CT scan was performed and this revealed a large splenic haematoma and free intraperi- toneal blood. Due to his continuing haemodynamic instabil- ity laparotomy was undertaken 36 h after the onset of his symptoms. At operation the CT findings were confirmed and splenectomy was carried out. He had a complicated post- operative course, requiring a total of 18 units of blood to be transfused. Histological examination of the spleen revealed changes consistent with IM infection. He was given pneu- movax prior to discharge and was discharged home some 15 days later. When seen I year later he had made a full recovery.

DISCUSSION In 1844 Berthet de Gray presented a case of a patient whose spleen had been removed 8 days after it protruded through a stab wound. He reported that the patient maintained normal digestion and concluded that ‘the spleen is not an organ indispensable for living’, but noted that the patient died of pneumonia after I3 years. Numerous reports of splenectomy without apparent detriment since the 17th century” led the prevalent view summarized by Hamilton Baily in 1927 that ‘in no instance is there the slightest indication that the splenectomized person is more susceptible to infection than the rest of humanity’.” The role of the spleen remained obscure and its value to life controversial until the middle of this century.

Recognition of increased susceptibility of the asplenic individual to severe sepsis followed King and Shumacker’s 1952 report of overwhelming sepsis in five splenectomized infants.*I Subsequent reports of overwhelming post-

splenectomy infections (OWPSI) in both children” and adults.?’ reports of increased postoperative septic complica- tions following splenectomy2’.24 and experimental evidence of defects in both cell-mediated’5.2h and humeral immu- nity,”,” provide evidence of an ongoing role of the spleen in immune surveillance. The idea that splenectomy may be performed with impunity has been laid to rest.

The actual risk of major post-splenectomy sepsis is uncer- tain. Singer determined an incidence of fulminant sepsis of 4.3% in a review of 2795 patients with a 2.5% mortality.” The series dealt predominantly with infants and children with haematological disease. It is now clear that infants are at a higher risk,’” probably reflecting the importance of the spleen to the primary response on first exposure to an infective agent.” The disease process prompting splenec- tomy also heavily influences risks of both postoperative and subsequent sepsis. Schwartz et al. provides data for an unselected population stratified by indication for splenec- t ~ m y . ~ ~ Only two cases of fulminant sepsis occurred in 193 Rochester residents splenectomized for various indications; during 1090 patient years of follow up, there was an inci- dence of 0.18 cases/100 persons per year. The relative risk of any infection when splenectomy was performed for trauma was one-half that when performed for haematological disease, and one-fifth that when performed incidental to abdominal malignancy. Luna and Dellinger reviewing all reports strati- tied by indication for splenectomy. determined the mortality from OWPSI to be 0.43% for 253 I combined adults having undergone splenectomy for trauma, with a death rate of 0.00077% per patient year.’” The risk of serious post- splenectomy sepsis for the otherwise fit individual in the age range where IM predominates, while very real, is small.

The ability of a normal spleen to heal was noted by Billroth as early as 1876. Considerable clinical experience has confirmed safe healing of the child’s traumatized ~pleen.’’.’~ Pearl et al. reported on 75 consecutive splenic injuries in children, and documented successful non-operative management in 87%, with only 10% of those with isolated splenic trauma requiring tran~fusion.~’ There were no haemorrhage-related fatalities and no cases of delayed rup- ture. Conservative management of splenic injury in the adult remains controversial.

Mahon and Sutton have questioned the reliability of healing in the adult spleen.’’ Noting a relatively thinner capsule, and less functional smooth muscle and elastic tissue in the capsule and splenic vasculature, he suggests that the haemostatic mechanisms may be impaired. Mucha reported no difference in healing between child and adult, detailing successful non-operative management in 24% of his series of 221 adults.34 The higher intervention rate compared with Pearl’s series of children may be explained by the severity of associated injuries, and a relatively lower transfusion threshold for intervention. Morbidity from associated inju- ries was 23% in the former series, 4% in the latter. Mucha did report four delayed ruptures occurring between the second and eleventh day of observation.

Blood transfusion is not without risk. Hepatitis is currently the primary cause of morbidity and mortality related to transfusion. The frequency of contracting hepatitis varies with the donor pool. US data from prospective studies demonstrate a frequency of 7-50% (20-50% of those af- fected progress to chronic hepatitis, 5- 10% of these may

SPLENIC RUPTURE MANAGEMENT 249

die).30 Current New Zealand data of transfusion risks esti- mate this as significantly less with hepatitis frequency of 0.005% and cytomegalovirus at 1% (although this is of negligible clinical ~ignificance).~~

Advocating a selective approach to conservative manage- ment, Mucha’s group have adopted a 2 unit transfusion threshold to intervention, which is approximately one-half the 40mL/kg limit accepted by Pearl. Luna and Dellinger have applied a statistical model to the situation of splenic rupture in the adult, comparing the risk of fulminant post- splenectomy sepsis with risk of additional transfusion re- quirement accompanying non-operative management.30 They concluded that the benefits of splenic retention are negated by the increased transfusion requirements. The balance of the equation is affected by the purity of the donor pool.

The case for splenic conservation is easily argued in children with traumatized spleens; where the risk of post- splenectomy sepsis is high, safety is well established, and transfusion requirements generally modest. Optimal man- agement of splenic rupture in the adults may be based on the proved safety of the option chosen. The safety of non- operative management is being established in the trauma- tized spleen;36 however, it certainly has not been established when rupture complicates IM.

Ten patients have been successfully managed without operation; 16*37-41 we report the eleventh. Blood transfusion has been avoided in f ~ u r . ’ ~ . ~ ~ Requirements were not reported in three21.38.40 while the other two received 21694’ units and one3’ received 8 units. Gauderer et al. suggest that with the advent of modem imaging and the availability of intensive care monitoring facilities, a non-operative approach is justi- fied in selected patients.I6 However, disturbing features have emerged when non-operative management has failed.

Vitello’s patient required a 6 unit transfusion,’O our pa- tients required 7, 5 and 18 units, after failed non-operative management. Delayed rupture occurred after 5 days, 4 h and 36 h of observation in our patients, respectively, and 69 days after the conservatively managed initial rupture in the 14 year old girl reported by McLean et a1.,I2 which was well past any reasonable period of observation in an intensive monitoring situation.

One of Gauderer’s patients returned to work within 2 weeks and survived a major automobile accident 3 months later without further splenic injury. l6 McLean’s report of delayed rupture after 10 weeks,’* and the persistence of perisplenic and intrasplenic haematomas for several months in two of Johnson’s three patients,38 suggest that the spleen may remain susceptible to delayed rupture for a considerable period of time. It remains to be established how long the spleen remains vulnerable, with a restriction on activity and travel.

Kurchin and Yellin provide the sole report of successful splenorrhaphy when rupture has complicated IM.42 This is difficult for the experienced surgeon when the spleen is healthy34 and it is therefore likely to be extremely difficult in the diseased spleen where rupture is often multifocal, with extensive capsular disruption and softened splenic parenchyma.

Non-operative management is certainly possible in the diseased spleen when rupture complicates IM, but there is little evidence to suggest this option is safe in the short term, or greatly beneficial to the patient in the long-term. The

evidence suggests that prompt splenectomy remains the optimal management in this situation.

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