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Laparoscopic Splenectomy
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Laparoscopic splenectomy was first reported in the 1990s and quickly became the preferred method for splenectomy as well as for the management of other splenic disorders such as splenic cysts and wandering spleen. Laparoscopic partial splenectomy (LPS) has also been utilized in children with heredity spherocytosis (HS) and sickle cell disease (SCD). The main advantages of laparoscopic splenectomy include less pain, shorter length of hospitalization, faster return to regular activities, and smaller scars. In the early comparative series from the 1990s and 2000s, laparoscopic splenectomy was associated with a longer operative time compared with open splenectomy. However, in the current era, laparoscopic splenectomies can often be performed faster than the historic open times. Alternatively, the laparoscopic technique can be associated with difficulty in patients with marked splenomegaly. The primary advances in splenectomy have been related to smaller instrumentation and the development of advanced energy devices. Although concern has been raised about the detection of accessory spleens, comparison studies have demonstrated similar rates of detection between the laparoscopic and open approaches.
Indications for Workup and Operation
Splenectomy in children is most frequently required for HS, idiopathic thrombocytopenic purpura (ITP), and SCD with splenomegaly and splenic sequestration.
Hereditary Spherocytosis
Hereditary spherocytosis affects approximately 1 in 3000 Caucasian individuals. Total splenectomy alleviates hemolysis in this condition. However, potential postoperative complications include an increased incidence of postsplenectomy sepsis and future thrombotic events. To decrease these complications, some hematologists have advocated for partial splenectomy in children with HS, particularly in younger children with HS. The role of partial splenectomy is to remove approximately 85% to 90% of the splenic tissue. A large multicenter trial evaluating partial splenectomy demonstrated that this procedure is effective in improving the hemoglobin level. However, the postoperative rise in hemoglobin was not as great as with total splenectomy. In addition, regrowth of the residual tissue occurs in most children, and for some, this requires subsequent total splenectomy.
In general, children with HS should undergo an ultrasound to evaluate for associated gallstones. If gallstones are present, a laparoscopic cholecystectomy is typically performed at the same time. In our center, we have found that children younger than 10 years of age with HS have a 27% incidence of gallstones, compared to 56% in the group older than 10 years of age.
Idiopathic Thrombocytopenic Purpura
The management of ITP includes steroids as a typical first-line agent, but numerous other therapeutic strategies are now available including intravenous immunoglobulin, rituximab (monoclonal antibody against CD20-positive B cells), and thrombopoietic agents such as romiplostim. Other therapeutic agents include azathioprine, cyclophosphamide, danazol, and mycophenolate mofetil. In ITP, it is essential to evaluate for accessory spleens thoroughly as this can be a cause of recurrent ITP.
Sickle Cell Disease
Sickle cell disease results from an amino acid substitution of the β chain of normal hemoglobin AA, resulting in hemoglobin S. Children may be homozygous (sickle cell disease) or have less severe heterozygous types (such as sickle C or sickle thalassemia). These red blood cells are rigid and lead to splenic sequestration when they pass through the spleen. Severe sequestration can lead to anemia, splenomegaly, and thrombocytopenia, known as an acute splenic sequestration crisis. In general, the development of one crisis at a young age usually leads to splenectomy. Preoperative preparation with a hemoglobin of at least 10 g/dL is generally recommended.
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