Hereditary spherocytosis surgery

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Generally, the treatment of hereditary spherocytosis involves presplenectomy care, splenectomy and management of postsplenectomy complications. Splenectomy is very effective in reducing hemolysis, leading to significant prolongation of red blood cell lifespan. Partial splenectomies can be used in pediatric patients as it controls hemolysis and preserves splenic function. Patients having concomitant gallstones are likely to benefit from combined splenectomy and cholecystectomy in terms of life expectancy. Post splenectomy complications may include; infections & sepsis caused by encapsulated organisms (streptococcus pneumoniae, neisseria meningitidis, haemophilus influenza), deep venous thrombosis (DVT), pulmonary emboli and portal vein thrombosis.

• Splenectomy
  • Splenectomy is very effective in reducing hemolysis, leading to significant prolongation of the red blood cell lifespan.^[1][2]
  • Patients should be selected for splenectomy on the basis of their clinical symptoms and presence of complications such as gallstones, not simply on the basis of diagnosis alone.
  • Generally, the treatment of hereditary spherocytosis involves presplenectomy care, splenectomy, and management of postsplenectomy complications.
  • In pediatric cases, splenectomy ideally should not be performed until a child is older than 6 years because of the increased incidence of postsplenectomy infections with encapsulated organisms such as streptococcus pneumoniae and haemophilus influenzae in young children.
  • Partial splenectomies are increasingly used in pediatric patients, as this approach appears to both control hemolysis and preserve splenic function.
  • European guidelines on splenectomy for hereditary spherocytosis note that a laparoscopic approach is currently considered the gold standard for removal of a normal-sized or slightly enlarged spleen and is preferred to open splenectomy, but it should be performed only by experienced surgeons.
  • Children or young adults with mild hereditary spherocytosis who also has gallstones are likely to benefit from combined splenectomy and cholecystectomy in terms of life expectancy.^[3]
  • Following splenectomy, the clinical manifestations and complications (anemia & gallstones) are much reduced in severe hereditary spherocytosis and abolished in milder cases, but at the risk of increased life threatening sepsis from encapsulated organisms, particularly streptococcus pneumoniae.^[3][4]

• Complications of splenectomy
  • Splenectomy has a number of known risks of which patients (or parents) should be aware^[5]
  • Operative risks (eg, infection, bleeding, or injury to adjacent organs such as the stomach or tail of the pancreas); these are relatively infrequent.
  • Infections, including overwhelming sepsis, from encapsulated organisms (streptococcus pneumoniae, neisseria meningitidis, hemophilus influenzae) that can no longer be removed by normal splenic clearance mechanisms, as well as certain other microorganisms including plasmodia, Babesia, Bordetella and Capnocytophaga species (from animal bites).^[6]
  • These risks are thought to be highest in the first year following splenectomy and in individuals undergoing splenectomy before five to six years of age.
  • However, risks of sepsis are likely to have declined with improved options for preoperative vaccinations and postoperative prophylactic penicillin. This was illustrated in a 1991 study from the Danish National Patient Registry that demonstrated a dramatic reduction in serious streptococcus pneumoniae infections following pneumococcal vaccination.^[7]
  • Individuals who did not receive appropriate pre-splenectomy vaccinations should have a thorough review of their immunization history and should receive vaccinations.
  • Venous thromboembolic (VTE) complications including thromboses of the deep veins, pulmonary emboli, splenic or portal vein thrombosis, as well as thrombosis in other unusual sites.^[8][9]
  • Venous thromboembolism events appear to be more common in individuals with hereditary spherocytosis who undergo splenectomy than in those who do not, but the individuals who undergo splenectomy may have had more severe underlying disease, making direct comparisons difficult.
  • Thromboprophylaxis at the time of surgery should be used based on standard practices; there is no indication for extended thromboprophylaxis beyond the usual duration.
  • Arterial thrombotic events may also be increased relative to individuals with hereditary spherocytosis who do not undergo splenectomy, with the same caveat that applies to venous thromboembolism (patients who undergo splenectomy may have more severe underlying disease).^[10][11]
  • It is not clear whether pulmonary artery hypertension (PAH) is a complication of splenectomy in hereditary spherocytosis.^[12]

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