Accessory splenic tissue is removed when discovered at the time of splenectomy for immunologic disorders. There is no observed benefit from accessory splenectomy during abdominal surgery for nonsplenic disease (Table 59-3).
Splenic Disorders and Treatments
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Table 59-3 Splenic Disorders and Treatments
| ||Disorder ||Treatment |
|Anomalies ||Wandering spleen ||Splenopexy ± Gastropexy |
| || ||Laparoscopic vs. open |
| ||Accessory spleens ||Removal at time of splenectomy for hematologic disorder |
| ||Splenogonadal fusion ||Division of obstructing band |
| || ||Removal of accessory splenic tissue |
|Hereditary anemia ||Hereditary spherocytosis ||Splenectomy (after age 5) if patient symptomatic |
| || ||Consider laparoscopic or open partial splenectomy |
|Membrane defects ||Hereditary elliptocytosis || |
No treatment if asymptomatic
Splenectomy for rare severe anemia
| ||Thalassemia || |
Transfusion: for Hgb <6 g/dL
Reduce transfusion requirements
Relieve compressive symptoms
Relieve abdominal pain caused by splenic infarcts
|Hemoglobinopathies ||Sickle cell disease || |
Transfusion for Hgb <6 g/dL and splenic sequestration
Sequestration more than 2 to 3 episodes per year or 1 life-threatening episode
|Enzyme defects ||G-6-PD deficiency ||No treatment |
| ||Pyruvate kinase deficiency ||Splenectomy for severe anemia |
|Autoimmune disorders ||Idiopathic thrombocytopenia purpura (ITP) || |
No treatment if mild
Steroids or IV immunoglobulin
Surgery for thrombocytopenia lasting 6 months in patients with low platelet counts interfering with quality of life
Immunosuppressive agents for surgical failure if no retained accessory spleen is identified
| || |
Splenectomy for steroid failure if radioactive RBCs are sequestered in the spleen.
| ||Cold antibody ||Immunosuppresison and plasmapheresis |
|Malignancies ||Primary ||Total splenectomy with chemotherapy |
| ||Secondary ||Chemotherapy |
|Cysts ||Congenital || |
Partial splenectomy or cystectomy
Total splenectomy if massive
Aspiration and sclerosis: high recurrence rate
Observe if small (<4 cm) and asymptomatic
| ||Acquired ||Partial or total splenectomy |
|Benign masses || ||Partial or total splenectomy |
|Abscess || || |
|Hypersplenism and splenomegaly || || |
Splenectomy for significant clinical illness with WBCs <4000. Platelets <100,000 or Hgb decrease >0.5 g/dL/wk
Portosystemic shunt for hypersplenism caused by portal hypertension
|Trauma ||Penetrating ||Laparotomy with splenic salvage if possible |
| ||Blunt || |
Supportive care with blood transfusion if necessary
Laparotomy for failure of supportive care
Suturing of spleen
Application of hemostatic agent
Role of laparoscopy evolving: diagnosis and possible clearance of hemoperitoneum to alter inflammatory response.
The wandering spleen is at risk for trauma and torsion. This operation may be done via laparotomy or with a minimally invasive approach. Placement of the spleen in an extra peritoneal pocket, fixation with a polyglycolic acid mesh snood or by gastropexy and splenopexy have all been described as successful surgical options.
Hereditary Hemolytic Disorders
Hereditary Hemolytic Spherocytosis
Splenectomy has proven to be a safe and effective treatment for HHS. The timing of the surgery depends on multiple factors, including the child's age and the severity of symptoms. Risks of aplastic crisis and cholelithiasis seem to increase with time. Laparoscopic splenectomy is a safe and effective technique. Modifications in the technique continue to evolve, including application of single-site and partial splenectomy options. The presence of gallstones should be investigated prior to operation so that cholecystectomy can be done concomitantly if stones are present. Presence of gallstones, in the absence of other complications of HHS is not a compelling indication for splenectomy.
Hereditary Hemolytic Elliptocytosis
Hereditary elliptocytosis rarely causes hemolysis and anemia severe enough to warrant splenectomy. Splenectomy is effective treatment, if indicated by severe and persistent symptoms.
Both thalassemia major and sickle cell disease are treated symptomatically, focusing on prevention of infection, provision of appropriate transfusion therapy, and measures to minimize physiologic stress.
Most children with thalassemia major accommodate appropriately to their anemia. Splenectomy may be useful to reduce excessive erythrocyte transfusion requirements. Splenectomy may also be indicated to alleviate compressive symptoms of splenomegaly.
The major indications for splenectomy in sickle cell disease are acute splenic sequestration, hypersplenism, and splenic abscess. In the event of multiple yearly sequestration crises or following one life-threatening splenic sequestration crisis, splenectomy is indicated following transfusion and stabilization. Preemptive vaccination against encapsulated organisms is essential and a surveillance ultrasound exam for cholelithiasis should be obtained. Splenectomy may be performed with either celiotomy or with minimal access approach, depending on the clinical scenario. Splenectomy is effective in treating splenic abscesses in patients with sickle cell disease.
Most patients with G-6-PD or pyruvate kinase deficiency do not need treatment if they maintain a hemoglobin level above 8 g/dL and are asymptomatic. These entities are rarely severe enough to require splenectomy. G-6-PD deficiency will not respond to splenectomy because the spleen is not the site of hemolysis. Splenectomy may decrease the need for transfusion in patients with pyruvate kinase deficiency.
ITP in childhood is often self-limiting. Eighty percent of children with ITP recover spontaneously. Splenectomy is rarely considered in acute ITP unless potentially life-threatening hemorrhage intervenes. In chronic forms of the disease, splenectomy is an option when medical management fails or no longer is effective; or in the child who is symptomatic and whose lifestyle is severely limited by thrombocytopenia. The success rate of splenectomy seems to parallel the clinical response to steroids and intravenous immunoglobulin therapy. Unsuccessful splenectomy should prompt surveillance and elimination of occult or residual splenic tissue, such as accessory spleen, followed by additional immunosuppressive therapy.
Autoimmune Hemolytic Anemia
Patients with Coombs-positive warm antibody normally respond to steroid therapy. In the event of steroid failure, splenectomy may be considered. Coombs-positive cold antibodies do not benefit from splenectomy, as the site of hemolysis is extrasplenic. The treatment is plasmapheresis and immunosuppression.
Tumors, Cysts, and Abscesses
Primary malignant tumors of the spleen are treated with splenectomy and chemotherapy. Splenectomy is no longer part of the staging of Hodgkin lymphoma. Noninvasive imaging studies have supplanted splenectomy in this situation.
Benign tumors and acquired cysts are treated with either partial or total splenectomy, depending on the size of the mass. Subtotal or partial splenectomy is optimal if it can be done safely. If there is a suspicion of echinococcal disease, appropriate screening measures and intraoperative precautions should be exercised. Congenital splenic cysts smaller than 4 cm may be observed and are effectively followed with yearly ultrasound examination to document stability of the cyst. Laparoscopic unroofing and subtotal resection of epidermal cysts of the spleen results in an unacceptable rate of recurrence unless the entire cyst wall is excised or complete excision with partial splenectomy is performed. Failure to do so often results in recurrence of a cyst that remarkably resembles the original lesion (Fig. 59-1). Every effort should be made to resect the entire cyst wall. If the epidermal cyst is large, centrally located, or complicated, total splenectomy—either open or via minimal access approach—may be the only option. Guided aspiration and successful sclerosis of a splenic cyst has been documented but is not universally successful and may require multiple treatments.
Recurrent epidermoid cyst of spleen after laparoscopic unroofing and partial cystectomy.
In children, splenic abscesses may arise as a manifestation of serious infection in other parts of the body. Multiple, small abscesses may present without specific clinical signs or symptoms. These children are treated with high-dose antibiotics and usually do not need splenectomy unless medical management fails. Image-guided drainage in combination with antibiotics is successful in two-thirds of patients and does not impact the success of subsequent splenectomy.
Hypersplenism and Splenomegaly
Numerous disorders, both benign and malignant, may cause hypersplenism in children. Hypersplenism due to portal hypertension is best approached by treatment of the underlying cause or with decompression of the portal system. Laparoscopic splenectomy may alleviate symptomatic neutropenia, thrombocytopenia, or anemia in selected cases. Compressive symptoms of splenomegaly can limit the quality of life that may improve with splenectomy. Partial splenectomy in Type I Gaucher disease, unresponsive to enzyme replacement therapy, may improve symptoms even in the face of remnant regrowth.
In the vast majority of children with splenic injury from blunt trauma, hemorrhage is self-limited and non life-threatening. These injuries are nicely imaged with CT scanning. Guidelines for nonoperative management of isolated splenic injury in childhood are published from the American Pediatric Surgical Association (Table 59-4).
APSA Trauma Committee Guidelines for Isolated Splenic Injury
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Table 59-4 APSA Trauma Committee Guidelines for Isolated Splenic Injury
| ||CT Grade |
| ||I ||II ||III ||IV |
|ICU days ||None ||None ||None ||1 day |
|Hospital days ||2 days ||3 days ||4days ||5 days |
|Predischarge imaging ||None ||None ||None ||None |
|Postdischarge imaging ||None ||None ||None ||None |
|Activity restrictiona ||3 weeks ||4 weeks ||5 weeks ||6 weeks |
The foundation of nonoperative management of splenic injury is hemodynamic stability. All other aspects of care depend on maintenance of adequate tissue perfusion and avoidance of shock. The absolute indication for operation is unrelenting and uncorrectable shock. In children, contrast blush on CT is less significant compared to this finding in adults and is not a harbinger of nonoperative management failure. If there are severe, coexisting injuries that make splenic salvage unsafe or if the child has required transfusion of greater than 40/mL/kg of blood, operative intervention should be considered. Control of hemorrhage and splenic salvage, if possible, are goals.
Routine postinjury imaging of the spleen is unnecessary. The true risk of radiation exposure from CT imaging remains to be defined, but it seems prudent to limit CT exposure unless the information obtained is essential to care. Routine follow-up CT imaging is not part of current evidence-based guidelines. Additional imaging may be important if post injury symptoms of pain, left upper quadrant tenderness or fullness are noted to persist long after the injury.