Severe thrombocytopenia due to hypersplenism successfully treated with partial splenic embolization in preoperative management.

Abstract: Hypersplenism is a known complication of portal hypertension secondary to cirrhosis of the liver. Although thrombocytopenia secondary to hypersplenism does not cause clinically significant hemostatic defect, it may need to be addressed in selective circumstances, such as preoperative preparation for a surgery. This report describes a 30-year-old male with a history of cirrhosis of the liver and hypersplenism who had a recurrence of craniopharyngioma. A platelet count of 40 X [10.sup.9]/L limited his treatment options. A stereotactic injection of radioactive P32 into the tumor was planned but was thought not to be feasible because of the thrombocytopenia. The thrombocytopenia responded favorably to partial splenic embolization, and the patient underwent successful stereotactic injection of radioactive P32 into the tumor.

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Hypersplenism is a known complication of portal hypertension secondary to cirrhosis of the liver. The thrombocytopenia associated with hypersplenism does not usually induce a clinically significant hemostatic defect. However, thrombocytopenia may increase the chance of a potentially devastating bleeding complication in a patient who is scheduled to undergo neurosurgery. Currently, no universally accepted therapy has been established for hypersplenism. Surgical splenectomy is not an optimal option in patients with Child's class B and C cirrhosis because of increased perioperative mortality rates associated with major surgical procedures. (1) In addition, surgical splenectomy has been associated with an increased long-term risk of septic events. (2) Partial splenic embolization (PSE) is a feasible but often overlooked alternative in selected patients in whom thrombocytopenia secondary to hypersplenism needs to be addressed.

Case Report

In 2001, a 30-year-old male presented with seizures. His medical history was significant for craniopharyngioma, which was surgically removed in 1987. After the surgery, the patient had hypopituitarism, requiring hormone replacement in the form of levothyroxine, desmopressin, and cortisone. He subsequently had cirrhosis of the liver secondary to chronic alcohol use and potential concomitant nonalcoholic steatohepatitis. Evaluation of his new seizure disorder by magnetic resonance imaging of the brain showed a very large cystic mass arising in the suprasellar area and extending eccentrically into the left frontal lobe. A stereotactic injection of radioactive P32 into the tumor cyst was planned. However, this was not feasible secondary to thrombocytopenia (platelet count, 40 X [10.sup.9]/L). He also had mild coagulopathy, with prothrombin time of 15.8 seconds and international normalization ratio of 1.3. The patient was seen in consultation for thrombocytopenia and hypersplenism in the hematology and hepatology clinics. The diagnosis of hypersplenism was confirmed by the presence of splenomegaly, bone marrow biopsy showing hypercellular bone marrow, and normal [B.sub.12], folate levels. A computed tomography scan of the abdomen showed moderate splenomegaly. The platelet count did not improve after 20 U of platelet transfusion, further substantiating hypersplenism as the underlying cause of thrombocytopenia.

We recommended PSE in an attempt to increase the platelet count before the planned neurosurgery. The catheterization of the splenic artery was achieved through the use of a femoral artery approach. A splenic arteriogram was performed, which showed patent splenic artery, supplying markedly enlarged spleen. Using 250 to 355 [micro]m polyvinyl alcohol particles (Cordis, Miami, FL), splenic parenchyma was embolized. The postembolization arteriogram showed embolization of nearly 60% of the splenic parenchyma. Periprocedural antibiotic was administered for 3 days. The postprocedural management consisted primarily of pain control with morphine sulfate administered through patient-controlled analgesia pump (days 0-2), propoxyphene (65 mg every 4 hours, days 1-4), and oxycodone (5 mg every 4 hours, days 3-4). The patient had a mild postembolization syndrome (PES), consisting of fever up to 38.2[degrees]C on post-PSE days 2 and 3. A computed tomography scan of the spleen after the procedure showed partial splenic infarction (Fig. 1). The patient was discharged on day 4 after PSE; however, he required readmission to the hospital for fever and chills, secondary to lung infiltrates. His pneumonia was appropriately treated and responded to antibiotics. Within 1 week, an improvement in platelet count was observed (Fig. 2). The increase in platelet count was sustained for the period of follow-up (85 days). The patient successfully underwent stereotactic injection of radioactive P32 later, without bleeding complications.

Discussion

Thrombocytopenia secondary to hypersplenism can complicate the treatment of patients with cirrhosis and portal hypertension. The risk of surgical bleeding varies inversely with the platelet count. Platelet counts higher than 100 X [10.sup.9]/L are preferable for high-risk surgeries such as neurosurgery, (3) in which a small amount of bleeding could have catastrophic effects. Hence, preoperative correction of thrombocytopenia is of utmost importance in these patients. The minimally invasive PSE has an advantage of allowing avoidance of complications associated with laparotomy and total splenectomy. After its initial description by Maddison, (4) PSE was temporarily abandoned because of high incidence of complications such as splenic rupture and splenic abscess associated with an unacceptable mortality rate. (5) Even so, just a few years later, Mozes et al (6) performed this procedure to improve blood counts in azathioprine-intolerant renal transplant recipients and later demonstrated its safety in a randomized, prospective study. (7) More recently, PSE has been shown to be safe and effective in pediatric patients with hypersplenism. (8) PSE has been used in the management of hypersplenism secondary to [beta]-thalassemia major, (9) hypersplenism secondary to cirrhosis with hepatocellular carcinoma, (10) hypersplenism in renal transplantation, (11) thrombocytopenia after liver transplantation, (12) after Kasai procedure for biliary atresia, (13) hereditary spherocytosis, (14) hypersplenism caused by Gaucher disease, (15) and to prevent dose limitation of cancer chemotherapy. (16) To our knowledge, this is the first time PSE has been used in a preoperative setting for neurosurgery. When performed correctly, PSE offers an alternative to surgical splenectomy in selected high-risk patients in whom splenic ablation is indicated. Complications of splenic parenchymal embolization include but are not limited to pain, nontarget embolization, abscess formation, atelectasis, pleural effusion, pneumonia, and arteriography-related problems. PES is a constellation of symptoms seen in excess of 50% of patients undergoing solid organ embolization procedures. It consists of fever, malaise, discomfort, and leukocytosis. It generally lasts no more than 3 to 5 days and may represent a reaction to the presence of necrotic tissue within the body. The measurement of C-reactive protein (CRP) helps to distinguish PES from an infection, as CRP typically peaks in 4 to 5 days after the procedure and then falls to normal by day 10. An underlying infection is suggested by CRP failure to fall or its continued rise. (17) The ablation of 60 to 70% of the spleen would be expected to result in significant improvement in terms of hypersplenism, whereas the residual 30 to 40% of the functional spleen would be expected to offer the necessary immunologic protection against sepsis by encapsulated organisms.

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Conclusion

Partial splenic embolization is a useful and safe therapeutic approach in the treatment of persistent thrombocytopenia secondary to hypersplenism in patients who require intervention.

Who looks outside, dreams; who looks inside, awakes.
--Carl Jung

Accepted October 19, 2004.

References

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17. Kadir S. Current Practice of Interventional Radiology. Hamilton, Ontario, B.C. Decker, 1991.

RELATED ARTICLE: Key Points

* Partial splenic embolization is safe and effective in treating thrombocytopenia secondary to hypersplenism in the setting of advanced liver disease.

* Postembolization syndrome and pneumonia are complications of partial splenic embolization.

Amar G. Pinto, MD, Jan Namyslowski, MD, and Prashant Pandya, DO

From the Division of Gastroenterology/Hepatology and Department of Radiology, Indiana University School of Medicine, Indianapolis, IN.

Reprint requests to Dr. Prashant Pandya, D2005, OPW, 1001 West 10th Street, Indianapolis, IN 46202. Email: [e-mail]