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Partial Splenic Embolization for the Treatment of Hereditary Spherocytosis

¹ All authors: Department of General Surgery, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670 Japan.

Received March 17, 2003; accepted after revision April 30, 2003.

 
Address correspondence to M. Miyazaki (masaru{at}med.m.chiba-u.ac.jp).

Abstract

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OBJECTIVE. Splenectomy is the standard surgical treatment for hereditary spherocytosis, but partial splenic embolization is another potential option. We retrospectively studied the therapeutic effects of partial splenic embolization as a treatment for hereditary spherocytosis.

CONCLUSION. Partial splenic embolization is a safe and effective alternative to splenectomy or partial splenectomy in the treatment of hereditary spherocytosis.

Introduction

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It has been reported that partial splenic embolization is a safe and effective alternative to splenectomy in the treatment of chronic idiopathic thrombocytopenic purpura [1, 2] and hypersplenism associated with portal hypertension [3, 4]. These reports suggest that partial splenic embolization is also a potential alternative to splenectomy in the treatment of hereditary spherocytosis. We explored this procedure in patients with hereditary spherocytosis.

Materials and Methods

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Between February 1995 and March 2002, five patients with hereditary spherocytosis, three men and two women, underwent partial splenic embolization in our institution (Table 1). Although hematologists recommended that the patients undergo surgery, they refused, and it was proposed that they undergo partial splenic embolization instead. Surgeons informed the patients about the details of partial splenic embolization. None had contraindications for surgery. Their median age was 33 years (range, 18–55 years). Patient 1 had undergone cholecystectomy for cholecystolithiasis 33 years previously. Patient 5 had silent cholecystolithiasis and abdominal pain from splenomegaly. Patients 2, 3, 4, and 5 had jaundice (total bilirubin level, > 60 µmol/L). None had sequestration crisis or needed blood transfusions before the procedure. All were discharged after approximately 1 week and were followed up at our outpatient clinic. Follow-up periods ranged from 12 to 96 months (median, 24 months). Serial changes in hemoglobin levels, hematocrit values, reticulocyte counts, and total bilirubin levels were measured on days 1, 3, 5, 7, 10, 14, and 28 after embolization, and subsequently every 3 months during follow-up. The study and publication of the results were approved by our institutional review board.

Partial Splenic Embolization
Partial splenic embolization was performed as has been previously described [1, 2]. Embolization of intrasplenic arterial branches was carried out by injection of 2 x 2 mm fragments of embolic material (Gelform, Upjohn, Kalamazoo, MI) and an antibiotic solution (0.2 g of ampicillin). Intended infarction volume of the spleen was approximately 80% in all patients. A single branch artery was preserved to achieve the intended infarction volume. Two sheets of Gelform (2 x 6 cm) were generally required for the embolization. After embolization, the patients received antibiotics IV (2.0 g of ampicillin per day) for 3–5 days.

All patients underwent CT of the abdomen before partial splenic embolization. Four of the five patients underwent unenhanced CT of the abdomen 4–8 weeks after embolization, and volumetric analyses were performed. Splenic volume was measured on a computer (Power Macintosh G4, Apple, Cupertino, CA) using a threshold function of NIH Image (rsb.info.nih.gov/nih-image, National Institutes of Health, Bethesda, MD). Source images were obtained by film scanning of abdominal CT. The spleen was selected by the computer on the basis of specified density of the splenic tissue. Unwanted contiguous areas were excluded using an erase function. Three-dimensional volumes were computed by summation of two-dimensional section areas multiplied by the slice thickness. The percentage of splenic infarction was calculated as infarcted volume / total splenic volume x 100. The infarcted area was defined by relatively low splenic parenchymal attenuation. Optimal window level and width settings of abdominal CT were 30 H and 250 H, respectively.

Statistical Analysis
A Student's t test was used to analyze paired samples. Median and range results are presented, with the ranges in parentheses. A p value less than 0.05 was considered statistically significant.

Results

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Effects of Partial Splenic Embolization
Splenic volume before partial splenic embolization ranged from 264 to 1850 mL (median, 965 mL). Extent of splenic embolization ranged from 83% to 97% (median, 95%) (Table 1 and Fig. 1). The median hemoglobin level rose significantly from 11.2 (range, 7.6–15.1) to 13.6 (range, 9.3–16.8) g/dL at 3 months after partial splenic embolization (p = 0.010) and stabilized at 13.4 (10.9–15.9) g/dL by 6 months (p = 0.016). In all patients, hemoglobin levels were maintained at 12 g/dL or more during the follow-up period (Fig. 2A). Median (range) hematocrit value also rose significantly from 30% (26–33%) to 33% (30–41%) at 1 month after partial splenic embolization (p = 0.039) and stabilized at 38% (31–46%) by 3 months (p = 0.0047). In all patients, hematocrit values were maintained at 33% or more during the follow-up period (Fig. 2B). Median (range) reticulocyte count decreased from 163 (80–210) to 44 (24–88) x 10⁹/L at 1 month after partial splenic embolization (p = 0.013) and stabilized at 40 (22–66) x 10⁹/L by 6 months (p = 0.010). In four of the five patients, reticulocyte counts were maintained at less than 55 x 10⁹/L during the follow-up period. In one other patient, reticulocyte count partially relapsed at 12 months after embolization and stabilized at about 100 x 10⁹/L for 7 years (Fig. 2C). Median (range) total bilirubin level decreased from 73.5 (37.6–133) to 18.8 (13.7–44.5) µmol/L at 1 month after partial splenic embolization (p = 0.031) and stabilized at 39.3 (23.9–61.6) µmol/L by after 6 months (p = 0.046). In all patients, total bilirubin levels were maintained at less than 60 µmol/L for the follow-up period (Fig. 2D). None of the patients had jaundice, sequestration crisis, need of blood transfusion, or newly detected cholecystolithiasis.

View larger version (111K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1. —33-year-old woman with hereditary spherocytosis who responded to partial splenic embolization. CT scan (window level, 30 H; width, 250 H) 5 days after embolization shows spleen 96% infarcted and line of demarcation between infarcted (arrows) and normal regions.

View larger version (16K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A. —Changes in blood values after partial splenic embolization. Black square, black triangle, circle, white triangle, and white square correspond to patient numbers 1, 2, 3, 4, and 5, respectively. Graph shows changes in hemoglobin levels. In all patients, hemoglobin levels were maintained at 12 g/dL or more during follow-up periods.

View larger version (17K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B. —Changes in blood values after partial splenic embolization. Black square, black triangle, circle, white triangle, and white square correspond to patient numbers 1, 2, 3, 4, and 5, respectively. Graph shows changes in hematocrit values. In all patients, hematocrit values were maintained at 33% or more during follow-up periods.

View larger version (18K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2C. —Changes in blood values after partial splenic embolization. Black square, black triangle, circle, white triangle, and white square correspond to patient numbers 1, 2, 3, 4, and 5, respectively. Graph shows changes in reticulocyte counts. In four of five patients, reticulocyte counts were maintained at less than 55 x 109/L during follow-up periods. In another patient, reticulocyte count partially relapsed at 12 months after embolization and stabilized at approximately 100 x 109/L for 7 years.

View larger version (17K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2D. —Changes in blood values after partial splenic embolization. Black square, black triangle, circle, white triangle, and white square correspond to patient numbers 1, 2, 3, 4, and 5, respectively. Graph shows changes in total serum bilirubin levels. Levels were maintained at less than 60 µmol/L during follow-up periods in all patients.

Adverse Effects of Partial Splenic Embolization
All patients had fever and upper abdominal pain after partial splenic embolization. Duration of fever (> 38.0°C) ranged from 2 to 6 days. In four of five patients, abdominal pain was alleviated in 1 week. The other patient (patient 5), with remarkable splenomegaly— estimated splenic volume of 1850 mL—had prolonged abdominal pain for 23 days and nausea for 8 days after the procedure. Patients 3 and 4 had left pleural effusion and ascites that were apparent at day 4 and day 5, respectively, and disappeared by day 10 without any drainage. None of the patients experienced splenic abscess or rupture. In all patients, WBC increased. Peak values occurred between day 1 and day 3 after the procedure and ranged from 16,400 to 31,500/µL (median, 21,500/µL). Platelet counts also increased after the procedure. A peak value occurred between day 7 and day 10 after embolization and ranged from 421 to 1320 x 10³/µL (median, 671 x 10³/µL) (Table 1).

Discussion

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Total splenectomy is the usual treatment for hereditary spherocytosis, and laparoscopic splenectomy seems to be the current standard among surgical treatments [5, 6]. Laparoscopic splenectomy can provide long-term hematologic response without late failure [5]. The effects of partial splenic embolization on splenic functions may be equivalent to those of partial splenectomy [7]. Although subtotal splenectomy is less effective than total splenectomy in limiting disease expression [7], partial splenectomy is sufficient to control hemolysis in patients with hereditary spherocytosis [8]. In our study, partial splenic embolization also successfully increased hemoglobin levels and hematocrit values and decreased reticulocyte counts and bilirubin levels. None of the patients had jaundice, sequestration crisis, need of blood transfusion, or newly detected cholecystolithiasis after the procedure. Furthermore, partial splenic embolization potentially has a long-term effect on hematologic parameters [3, 9]. Therefore, partial splenic embolization appears to be a reasonable treatment option for patients with hereditary spherocytosis.

It has been reported that total splenectomy can result in overwhelming postsplenectomy sepsis syndrome. Tchernia et al. [7] recommend subtotal splenectomy, which can maintain the phagocytic function while improving hemolytic rate for patients with hereditary spherocytosis, especially young children. In a similar way, partial splenic embolization could also maintain the phagocytic function and avoid the risk of overwhelming postsplenectomy sepsis syndrome.

Wholey et al. [10] have reported major complications after partial splenic embolization. Spontaneous rupture appears to be a function of both infarction size and underlying infectious complications. In our study, no patients developed splenic abscess or rupture. Preservation of a single branch of the splenic artery and antibiotics might successfully prevent major complications. However, all patients experienced postembolization syndrome (i.e., pain, fever, vomiting) [3] and two of the five patients had pleural effusion and ascites. These conditions were clearly correlated to the extent of splenic embolization. Furthermore, postembolization syndrome seemed to be more severe in patients with hereditary spherocytosis than in patients with idiopathic thrombocytopenic purpura [1, 2]. In patients with splenomegaly, large infarction volume of the spleen might result in severe inflammation and severe postembolization syndrome [3]. We suggest, therefore, that multiple prospective staged splenic embolization may reduce inflammatory response and prevent postembolization syndrome after embolization in patients with splenomegaly. Sequential embolization of separate splenic artery branches potentially decreases the infarction volume of initial embolization. Recently, Palsson et al. [9] have reported that a graded partial splenic embolization is reasonably safe and effective even in severely ill patients with cirrhosis.

Partial splenic embolization caused noteworthy thrombocytosis in all patients. However, it has been reported that in 64 patients undergoing splenectomy, none of the 21 patients who had a platelet count increase greater than 100 x 10³/µL showed evidence of venous thrombosis [11]. Therefore, it has not been substantiated that routine prophylactic anti-thrombotic therapy is needed for patients in whom thrombocytosis develops after partial splenic embolization.

In our study, although the targeted infarction volume was 80%, CT findings confirmed significantly greater infarctions in all patients with complications. In patients with idiopathic thrombocytopenic purpura who did not have splenomegaly, it was possible to embolize about 80% of the splenic parenchyma by preserving a single branch artery [1, 2]. In our study, intended infarction volume was also achieved in one patient without splenomegaly. These results suggest that in patients with splenomegaly, preservation of a few branch arteries may be necessary to achieve the intended infarction volume of 80% and to reduce the complication rate after partial splenic embolization.

Gelfoam seems to be the embolic material most commonly used for partial splenic embolization [1–3, 9]. Instead of Gelfoam, one can also use microspheres [4], coils, polyvinyl alcohol particles [12], and other materials, without any significant difference in embolization effects or complication rates. However, attention should be paid to the size of embolic materials because smaller particles may penetrate the spleen tissue disproportionately in peripheral areas and result in an infarction of unpredictable size [9].

Indications for splenectomy for patients with hereditary spherocytosis are somewhat controversial. Some authorities have advocated splenectomy for virtually every patient with apparent splenomegaly. Unequivocal criteria may include severe symptomatic hemolytic anemia, even a mild hemolytic anemia in association with gallstones, or a history of gallstones in similarly affected siblings [7]. Patients meeting these criteria may be candidates for partial splenic embolization. In our study, one patient without apparent splenomegaly underwent partial splenic embolization for mild hemolytic anemia and jaundice with successful results. Therefore, we suggest that patients without apparent splenomegaly may also be candidates for partial splenic embolization, which has the advantage of being a nonoperative intervention. This treatment should be recommended especially for patients in whom splenectomy may be associated with an increased risk of perioperative complications.

In conclusion, partial splenic embolization is a safe and effective alternative to splenectomy or partial splenectomy in the treatment of hereditary spherocytosis. Further studies are necessary to estimate the long-term results of partial splenic embolization for patients with hereditary spherocytosis.

References

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8. Rice HE, Oldham KT, Hillery CA, Skinner MA, O'Hara SM, Ware RE. Clinical and hematologic benefits of partial splenectomy for congenital hemolytic anemia in children. Ann Surg2003; 237:281 –288[Medline]
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This Article Abstract Figures Only Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kimura, F. Articles by Miyazaki, M. Search for Related Content PubMed PubMed Citation Articles by Kimura, F. Articles by Miyazaki, M. Social Bookmarking                      What's this? Hotlight (NEW!) What's Hotlight?