John F. Sweeney
Presentation
A 44-year-old female presented to her primary care doctor several months ago complaining of a recent onset of easy bruising and gum bleeding. A CBC demonstrated a platelet count <10 × 109/L. She was diagnosed with immune thrombocytopenic purpura (ITP) and admitted to the hospital for treatment. She was started on high-dose intravenous immunoglobulin and high-dose corticosteroids with an excellent response in her platelet count. She was discharged home on a gradual prednisone taper. As her prednisone doses were weaned below 20 mg per day, the patient experienced a recurrence in her thrombocytopenia with associated recurrence of easy bruising.
Differential Diagnosis
Excluding trauma, benign hematologic diseases are the most common indication for splenectomy (Table 1). ITP is the most common indication for splenectomy and constitutes >70% of patients undergoing splenectomy for benign disease. ITP is a disorder characterized by antiplatelet antibodies to platelet membrane glycoprotein. This results in opsonization of platelets and their premature removal from the circulation by the spleen. Adult patients typically present with petechiae, purpura, and bruising tendency. Mucosal bleeding, including epistaxis and hematuria, tend to be more frequent when the platelet count decreases to <20 × 109/L. The incidence of severe bleeding (e.g., intracranial hemorrhage) increases with platelet counts below 10 × 109/L.
TABLE 1. Hematologic Indications for Splenectomy
Additional benign hematologic conditions that are indications for splenectomy include patients with congenital hemolytic anemia, metabolism abnormalities, hemoglobinopathies, and erythrocyte structure abnormalities (e.g., hereditary spherocytosis and elliptocytosis). Splenectomy may be indicated as a diagnostic tool or for palliation in patients with malignant hematologic disease. Surgical staging is utilized most often in Hodgkin’s disease, resulting in a change in diagnosis and subsequent impact on therapy and prognosis in up to 30% to 40% of patients. Splenectomy can also provide relief to patients with symptomatic splenomegaly, which may or may not be accompanied by hypersplenism. Patients with malignant hematologic diseases are more likely to have massively enlarged spleens (>1,000 g), resulting in significant discomfort and pain as well as early satiety. When splenomegaly is accompanied by cytopenias (hypersplenism), the cytopenia often improved or sometimes cured by removal of the spleen.
Workup
Although the presumptive diagnosis is ITP, the patient undergoes a bone marrow aspirate that demonstrates normal marrow cellularity with specific mention of adequate megakaryocytes. Review of the peripheral blood smear does not demonstrate platelet clumping.
Discussion
First-line therapy for ITP includes oral corticosteroids and IV immunoglobulin. The majority of patients will initially respond to medical management of ITP, but recurrent thrombocytopenia is common. The indication and timing of splenectomy is often individualized according to response to treatment and patient and physician preferences. Splenectomy is indicated for ITP in patients with episodes of severe bleeding related to thrombocytopenia, patients who fail to respond to 4 to 6 weeks of medical therapy, patients who require toxic doses of immunosuppressive mediations to achieve remission, or patients who relapse following an initial response to steroids. Patients with ITP are ideal candidates for a minimally invasive approach because they are frequently young, otherwise healthy patients with normal to only slightly enlarged spleens.
Technique for Laparoscopic Splenectomy
Removal of the spleen laparoscopically is facilitated by the fact that the anatomic landmarks are relatively consistent, the operation is extirpative and does not require reconstruction, and in most cases the spleen does not need to be preserved for pathology so it can be morcellated in the abdominal cavity prior to removal (Table 2). Laparoscopic splenectomy (LS) has been shown in several retrospective studies to have equivalent or superior short- and long-term outcomes when compared to open splenectomy.
TABLE 2. Key Steps for Laparoscopic Splenectomy
Preoperative Preparation
The patient’s preoperative preparation includes administration of polyvalent pneumococcal vaccine at least 2 weeks before surgery. The evening before surgery, patients commence a clear liquid diet and take a mild laxative several hours before bedtime to decompress the colon and facilitate laparoscopic visualization of the left upper quadrant and spleen. Several units of packed red blood cells are cross-matched, and in patients with idiopathic thrombocytopenic purpura, platelets are crossmatched for administration after the splenic artery has been ligated intraoperatively if there is failure of clot formation.
Immediately preoperatively, pneumatic compression boots are applied and a preoperative antibiotic (1 gs cephazolin) is given. Patients who have been receiving corticosteroids within 6 months of surgery are given stress doses of intravenous corticosteroids. Before transport to the operating room, a beanbag-stabilizing device is placed on the operating table to enable subsequent patient positioning and stabilization. After endotracheal induction of general anesthesia, a Foley catheter and an orogastric tube are placed.
The patient is positioned in the incomplete right lateral decubitus position at an angle of 45°. This allows the patient’s position to be changed from nearly supine to nearly lateral by tilting the operating table. In this way, a combined supine and lateral approach can be realized. It is important to position the patient with the iliac crest immediately over the table’s kidney rest and mid-break point. The kidney rest is elevated and the table flexed, allowing more distance between the iliac crest and the left lower costal margin in the midaxillary line. The beanbag-stabilizing device is activated, and the patient’s hip is secured to the table with loosely applied tape. Legs are padded with pillows, and an axillary roll is placed. The left arm is hung over the chest on a sling. The arm must be far enough cephalad to clear the operative field and allow obstruction-free use of the laparoscopic instruments. All pressure points are adequately padded.
The skin is prepared and draped so that either laparoscopy or open surgery can be performed. The table is tilted 30° to the left to place the patient in the near-supine position. Before incisions are made, the area is anesthetized with long-lasting local anesthetic.
Laparoscopic Splenectomy
We prefer to obtain intra-abdominal access via an open technique with placement of a 12-mm Hasson trocar approximately 3 to 4 cm below the costal margin in the left midclavicular line (Figure 1A). The abdomen is then insufflated to a pressure of 15 mm Hg with carbon dioxide and a 10-mm, 30° laparoscope is introduced into the abdomen. Two 5-mm trocars are then placed in the upper midline or to the left of the midline along the costal margin. The first 5-mm trocar is placed 3 to 4 cm below the xiphoid process and the second trocar is placed in between the subxiphoid 5-mm trocar and the Hasson trocar. The abdomen is inspected with special attention paid to the greater omentum and splenocolic regions that are common locations for accessory splenic tissue. Accessory spleens are found in 10% to 15% of patients with hematologic disease and have been associated disease recurrence in patients with ITP when they are not removed.
FIGURE 1A • Laparoscopic Splenectomy Port Placement.
Following division of the splenocolic ligament and mobilization of the splenic flexure, an additional 12-mm trocar is placed in the left anterior axillary line, below the costal margin. The patient is then placed in steep reverse Trendelenburg position and the table rolled to the patient’s right giving a true left lateral decubitus position. Ultrasonic shears are used to divide the gastrosplenic ligament and short gastric blood vessels, allowing the stomach to fall to the patient’s right and providing excellent exposure to the splenic hilum. The splenic artery can then be easily identified and ligated with hemoclips if desired at this point of the case. Attention is then turned toward mobilization of the lower pole of the spleen. The splenophrenic and the splenorenal ligaments are divided using ultrasonic shears. If a lower pole vessel is encountered at this point, it is divided using an endoscopic stapling device with a vascular cartridge. This approach allows for visualization of the splenic hilum and the tail of the pancreas by retracting the spleen toward the abdominal wall. The superior splenophrenic attachments to the upper pole of the spleen are left intact to prevent torsion of the spleen during division of the hilum. The endoscopic stapling device with a vascular cartridge is then used to divide the well-exposed splenic hilum. Several fires of the stapler may be necessary. Following division of the remaining upper pole attachments, the spleen is placed into a specimen retrieval bag. The mouth of the bag is brought through the 12-mm Hasson trocar site, and the spleen is then morcellated with sponge forceps and removed in pieces. Special care must be taken to avoid ripping the endoscopic bag during this process in order to prevent spillage of splenic tissue in the abdomen. The left upper quadrant is irrigated and inspected for hemostasis. A second search for accessory splenic tissue is undertaken before the 12-mm fascial openings are securely closed with absorbable suture and the skin incisions are closed. The orogastric tube is removed in the operating room, and the patient is taken to the recovery room.
Hand-assisted Laparoscopic Splenectomy
The LS can be converted to hand-assisted laparoscopic splenectomy (HALS) if difficult anatomy, dense adhesions due to a previous upper abdominal surgery or excessive splenomegaly, is encountered. Preoperatively, the decision to proceed with HALS is made for patients with very large spleens or if the spleen must be removed intact for pathologic examination. When HALS is indicated preoperatively, we still place all trocars as described for a LS and proceed with division of the gastrosplenic ligament and short gastric blood vessels (Table 3). This provides excellent exposure to the splenic hilum and allows for early ligation of the splenic artery, which we feel is an essential step in patients with significant splenomegaly. We then create an incision connecting the two 5-mm trocars about 7 cm in size in the left paramedian position (Figure 1B). The left hand is then placed into the abdomen, which is then reinsufflated. There are several commercially available hand-port devices that can be used for HALS. The spleen is then mobilized as described for a total LS with the left hand providing gentle traction while at the same time preventing injury to the splenic capsule by the ultrasonic shears or a laparoscopic grasper. After the splenic hilum is divided and the spleen completely mobilized, it is placed in a specimen retrieval bag. A sterile radiograph cassette bag can be placed in the abdomen through the hand incision to retrieve those spleens that do not fit in the large specimen retrieval bags. The fascia is closed with appropriate strength suture and the abdomen then reinsuflated and inspected for hemostasis as described above.
TABLE 3. Key Steps for Hand-assisted Laparoscopic Splenectomy
FIGURE 1B • Hand-assisted Laparoscopic Splenectomy (HALS) Port Placement.
Postoperative Care
Postoperatively, the patient is allowed clear liquids orally and ambulates the night of surgery. The Foley catheter is removed the following morning. Pain is controlled with intermittent parenteral narcotics until the patient is able to take oral pain medication. Diet is advanced on postoperative day 1, and the patient is discharged when oral intake is tolerated and pain is controlled with oral analgesics usually on postoperative day 2.
Case Conclusion
The patient does very well after LS. Her platelet count returns to the normal range before discharge from the hospital. At 6- and 12-month follow-up, the patient has no clinical evidence of thrombocytopenia and has normal platelet counts.
LS has become the “gold standard” for removal of the spleen in the setting of ITP Although the increase in platelet number that defines a complete response to splenectomy varies between studies, numerous retrospective reviews and prospective nonrandomized trials have determined that the response rates (80% to 89%) and long-term remission rates (50% to 70%) to LS are comparable to those following open splenectomy, despite initial concern about the accuracy of accessory spleen identification using laparoscopy. LS also provides patients with improved short-term morbidity. Reductions in postoperative morbidity characteristic of minimally invasive procedures such as reduced length of hospital stay and reduced postoperative ileus have been consistently demonstrated in patients with ITP who undergo LS.
TAKE HOME POINTS
· Immune thrombocytopenic purpura (ITP) is the most common indication for splenectomy excluding trauma.
· Splenectomy is indicated for treatment of ITP in patients with episodes of severe bleeding related to thrombocytopenia, patients who fail to respond to 4 to 6 weeks of medical therapy, patients who require toxic doses of immunosuppressive medications to achieve remission, or patients who relapse following an initial response to steroids.
· Laparoscopic splenectomy (LS) is the optimal approach for removal of the spleen in the setting of ITP. It is associated with a shorter hospital stay, decreased postoperative pain, and earlier return to regular activities.
· Accessory spleens are found in 10% to 15% of patients and if not removed at the time of splenectomy will lead to recurrence of ITP.
· LS for ITP is associated with short-term response rates of 80% to 89% and complete long-term remission rates of 50% to 70% that are compatible with outcomes for open splenectomy.
SUGGESTED READINGS
Bresler L, Guerci A, Brunaud L, et al. Laparoscopic splenectomy for idiopathic thrombocytopenic purpura: outcome and long-term results. World J Surg. 2002;26:111–114.
Brunt LM, Langer JC, Quasebarth MA, et al. Comparative analysis of laparoscopic versus open splenectomy. Am J Surg. 1996;172:596–599;discussion 599–601.
Friedman RL, Fallas MJ, Carroll BJ, et al. Laparoscopic splenectomy for ITP. The gold standard. Surg Endosc. 1996;10:991–995.
Mikhael J, Northridge K, Lindquist K, et al. Short-term and long-term failure of laparoscopic splenectomy in adult immune thrombocytopenic purpura patients: a systematic review. Am J Hematol. 2009;84(11):743–748.
Rescorla FJ, Engum SA, West KW, et al. Laparoscopic splenectomy has become the gold standard in children. Am Surg. 2002;68:297–301;discussion 301–302.
Targarona EM, Espert JJ, Cerdan G, et al. Effect of spleen size on splenectomy outcome. A comparison of open and laparoscopic surgery. Surg Endosc. 1999;13:559–562.