Published online August 1, 2007
PEDIATRICS Vol. 120 No. 2 August 2007, pp. e432-e435 (doi:10.1542/10.1542/peds.2006-3071)

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EXPERIENCE & REASON

Nonoperative Management of Spontaneous Splenic Rupture in Infectious Mononucleosis: A Case Report and Review of the Literature

Jacob T. Stephenson, MD^a and Jeffrey J. DuBois, MD, FACS, FAAP^b

^a General Surgery, David Grant Medical Center, Travis Air Force Base, California
^b Pediatric Surgery, Kaiser Permanente Health System, Sacramento, California

ABSTRACT

Spontaneous rupture of the spleen is a rare complication of infectious mononucleosis with no clear consensus on appropriate management. Although management of traumatic splenic rupture has largely moved to nonoperative treatment, splenectomy is still frequently used in dealing with rupture of the diseased spleen. Here we report the case of a 16-year-old boy with splenic rupture secondary to laboratory-confirmed infectious mononucleosis in the absence of trauma. Nonoperative management including ICU admission, serial computed tomography scans, and activity limitation was used successfully. Our experience, along with a review of the literature, leads us to conclude that splenic preservation can be a safe alternative to splenectomy in hemodynamically stable patients with spontaneous splenic rupture. This is of particular importance in the pediatric population, which is at higher risk for postsplenectomy sepsis.

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Key Words: mononucleosis • splenic rupture • nonoperative • spleen

Abbreviations: IM, infectious mononucleosis • CT, computed tomography

Spontaneous splenic rupture is a rare complication of infectious mononucleosis (IM), estimated at 0.1% to 0.5% of clinically apparent IM.¹ Typical presentation in the pediatric population includes a prodrome of fever, pharyngitis, and lymphadenopathy for 1 to 3 weeks with an acute onset of abdominal pain with signs of hypovolemia. Kehr sign (pain referred to the left shoulder) may also be present in 50% of patients.¹ Radiologic confirmation is made with ultrasound or an abdominal computed tomography (CT) scan.

The management of spontaneous rupture of the diseased spleen has traditionally been splenectomy, but reports of successful nonoperative management have suggested that the diseased spleen can heal and retain immune function.^2–4

CASE REPORT

A previously healthy 16-year-old boy presented with acute onset of left upper-abdominal pain, nausea, and vomiting. Symptoms followed a 3-week history of fevers to 102°F, sore throat, malaise, and migratory extremity swelling. His abdominal pain began while at rest at home, and the patient denied history of any trauma. On initial examination, he was tachycardic with a heart rate of 120 beats per minute and normotensive with a blood pressure of 130/90 mmHg. Bilateral nontender cervical lymphadenopathy was appreciated. The abdomen was soft but generally tender, with guarding in the left upper quadrant and a palpable spleen 6 cm below the costal margin. Admission laboratory data included an initial and repeat hematocrit level of 24% and 22%, respectively, with a white blood cell count of 21000/µL with 18% atypical lymphocytes. Monospot test results were positive, as were titers for Epstein-Barr virus immunoglobulins G and M. A mild elevation of liver enzyme levels was noted along with an aspartate aminotransferase level of 104 U/L, an alanine aminotransferase level of 133 U/L, total bilirubin level of 0.4 mg/dL, and a lactate dehydrogenase level of 1729 U/L.

An abdominal CT scan (Fig 1) showed splenomegaly with heterogenous appearance resulting from a grade 3 subcapsular splenic hematoma with hemoperitoneum. The splenic hilum was intact, although the parenchyma was fractured in multiple planes. There was no other notable pathology in the abdomen, but a small left pleural effusion was seen.

View larger version (74K): [in this window] [in a new window]   FIGURE 1 Abdominal CT on initial presentation revealed a grade 3 splenic rupture with hemoperitoneum.

 
The patient was admitted to the PICU, where he was monitored with serial examinations and laboratory tests. He remained hemodynamically stable but was noted on admission to be mildly tachypneic and short of breath, which prompted transfusion of 2 units of packed red blood cells for symptomatic anemia. His hematocrit level remained at >30% for the remainder of his hospital stay. A repeat CT scan at 1 week showed resolution of the hemoperitoneum, continued subcapsular fluid, and a markedly increased left pleural effusion. The effusion was drained by a pigtail catheter placed under ultrasound guidance, and subsequent chest radiographs documented resolution.

He was transferred to the pediatric ward at 1 week and then discharged from the hospital after a 12-day hospital stay. Strict activity limitations were given, and immunizations against Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis were administered. At clinic follow-up on postadmission day 20, the patient was recovering well, with continued splenomegaly at 4 cm below the costal margin but no tenderness. A chest radiograph confirmed complete resolution of the pleural effusion. A CT scan at 3 months (Fig 2) revealed resolving subcapsular hematoma with some slight residual architectural distortion of the spleen. Splenomegaly was resolved on examination, so he was allowed to resume mild activity but was cautioned against contact sports. A follow-up CT scan at 8 months (Fig 3) revealed small calcification in the location of previous hematoma but normal splenic architecture. He was cleared at this point for full activities.

View larger version (81K): [in this window] [in a new window]   FIGURE 2 Three-month follow-up CT: continued subcapsular hematoma but normalizing architecture of the splenic pulp.

 

View larger version (84K): [in this window] [in a new window]   FIGURE 3 Eight-month follow-up: calcification of previous hematoma but otherwise structurally normal spleen.

 
At the 32-month follow-up, the patient had no delayed rupture and was living an active life, which included contact sports. He was treated for 1 episode of pneumonia 2 years after his initial presentation but has otherwise had no infectious complications up to now. Ultrasound of his spleen showed a stable calcification with otherwise normal architecture and normal size.

DISCUSSION

Since the first report of postsplenectomy sepsis by King and Shumacker in 1952, there has been a growing trend toward splenic conservation.⁵ The spleen is vital in protecting against infection by encapsulated organisms,⁶ with an estimated risk of overwhelming sepsis in the asplenic individual at 1% to 3% but a mortality rate of 50% to 80%.³ Nonoperative management avoids the risk of overwhelming postsplenectomy sepsis, as well as the inherent risks of a laparotomy. Other complications associated with splenectomy include pancreatitis, pneumonia, pulmonary embolism, and a higher rate of wound infections.^{1, 3, 4}

For these reasons, nonoperative management of splenic rupture in a stable trauma patient is now considered the standard of care.³ Other forms of splenic conservation including splenorrhaphy, partial splenectomy, and splenic artery embolization have also been used with good success in trauma patients.⁴

Because of the rarity of spontaneous splenic rupture, no clear consensus of treatment has been reached. In the setting of hemodynamic instability, splenectomy is the procedure of choice. However, in a stable patient, splenectomy or nonoperative treatment is a topic of debate.⁷ Successful conservative management has been reported, but there is a valid concern that the diseased spleen may not heal reliably, which raises the risk of a delayed or recurrent rupture.⁸

Epstein-Barr virus is known to distort the splenic architecture by infiltrating the parenchyma with lymphocytes and atypical lymphoid cells.⁹ This invasion compromises the fibrous support system of the spleen and thins the splenic capsule, which facilitates rupture.¹⁰ The rupture then occurs after minor trauma or spontaneously. The etiology behind spontaneous rupture is hypothesized to be a result of an acute increase in portal venous pressure caused by Valsalva maneuver, causing vascular engorgement.^{8, 10} Sudden compression of the enlarged spleen then occurs from contraction of the diaphragm or the abdominal wall, which causes the thinned capsule to rupture.¹¹ Thus, the cause of spontaneous rupture is essentially trauma from within the abdomen, suggesting that it could be dealt with in the same manner as traumatic injury of the diseased spleen.

In the English literature to date, there are at least 23 reports of successful nonoperative management of spontaneous splenic rupture resulting from IM.^{1, 2, 8, 12–15} The majority of these patients were adolescents, with ages ranging from 14 to 57 (average: 19 years). In contrast, 8 patients (average age: 24 years) in whom initial conservative management failed are reported; they required splenectomy.^{8, 16, 17} In reviewing these cases, nonoperative therapy failed in these patients because of persistent hemodynamic instability requiring >4 units of packed red blood cells. Multiple cases have been reported throughout the last 60 years of initial surgical therapy; there have been 5 reports of sudden death secondary to rupture and exsanguination.^{9, 12}

On the basis of these data, we suggest that nonoperative management is appropriate to undertake in the hemodynamically stable patient who requires <4 units or 40 mL/kg of blood for resuscitation.^{3, 4, 8, 18} An ICU setting with close monitoring by a qualified surgeon is prudent given the possible fatal consequence of an untreated severe bleed and the uncertainty of the structural integrity of the splenic pulp.^{8, 9, 12} Hemoglobin and hematocrit levels should be checked every 4 to 6 hours for the first 24 hours or until stable.¹⁸ Serial imaging by either ultrasound or CT scan to ensure resolution is also advisable.^{19, 20}

The question that still must be answered to ensure that nonoperative management is as safe and effective as splenectomy is whether the diseased spleen can heal and retain immunologic function. Splenomegaly has long been recognized as a complication of IM and other infectious conditions and is known to be a self-limited condition.^{10, 11} Clinical and scientific studies of the injured healthy spleen have documented that the spleen can retain full function while maintaining a low risk for repeat rupture.^{3, 4} A growing body of literature is now suggesting that the pathologic spleen can also heal after parenchymal disruption. Guth et al² published a prospective report of 11 patients with splenomegaly that resulted from HIV, IM, leukemia, and sickle cell disease who experienced isolated splenic rupture, grades 1 to 4. All 11 patients made a full recovery with <2 units of blood transfused per patient. Because of the rarity of spontaneous splenic rupture, there are no current data on immune competence of the spleen after nonoperative management, but it is generally assumed that structural healing correlates with continued immune function.

The observation period in these patients is another topic of controversy. Most reported cases with long-term follow-up have noted full resolution of hematoma by 1 year postevent, with some as quick as 4 to 5 weeks. However, delayed splenic rupture was noted by McLean et al,¹⁷ who reported a patient who was originally treated with nonoperative management but required splenectomy 69 days after original presentation. Conversely, Gauderer et al¹³ reported a patient who suffered significant blunt abdominal trauma without sequelae 3 months after discharge from a conservatively treated rupture.

As with our patient, most authors would recommend very strict activity limitations after discharge, but the time frame is unknown. Lynch et al²⁰ concluded that the time to radiographic healing of injury of the healthy spleen was directly proportional to the severity of the splenic injury, with a mean time of 3, 8, 12, and 21 weeks for grades 1, 2, 3, and 4, respectively. However, the diseased spleen is much less predictable, so radiologic follow-up is essential. CT was primarily used for this patient, but our current practice is to obtain CT at the initial presentation, followed by baseline ultrasound before hospital discharge. Ultrasound is then repeated at 3 and 6 months after discharge or until normal splenic architecture is documented. However, it is still unclear how accurately radiologic healing equates with physiologic healing, necessitating an individual approach with close follow-up for each patient.

CONCLUSIONS

Our experience, along with a review of the literature, leads us to conclude that splenic preservation can be a safe alternative to splenectomy in stable patients with spontaneous splenic rupture resulting from IM. This is especially important to consider in the pediatric and adolescent population, which is at high risk for postsplenectomy sepsis in addition to the risks of nontherapeutic laparotomy. However, this treatment must be approached with caution given the risk of ongoing bleed and the unknown potential of delayed rupture. These patients should be cared for by an appropriately trained surgeon, initially in an ICU setting, with close follow-up and activity limitation after discharge. Specifically, we recommend no activity more than walking until splenomegaly has resolved on examination, followed by a period of no contact sports for a total of 6 months or until normal splenic architecture is seen on radiologic evaluation.

FOOTNOTES

Accepted Jan 26, 2007.

Address correspondence to Jacob T. Stephenson, MD, General Surgery, David Grant Medical Center, 101 Bodin Circle, MSGS, Travis AFB, CA 94535. E-mail: jacob.stephenson{at}travis.af.mil

The authors have indicated they have no financial relationships relevant to this article to disclose.

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PEDIATRICS (ISSN 1098-4275). ©2007 by the American Academy of Pediatrics

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This Article Abstract Full Text (PDF) Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My File Cabinet Download to citation manager Request Permissions Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Stephenson, J. T. Articles by DuBois, J. J. Search for Related Content PubMed PubMed Citation Articles by Stephenson, J. T. Articles by DuBois, J. J. Related Collections Infectious Disease & Immunity Social Bookmarking                         What's this?