Heredity spherocystois is caused by a defect in the red blood cell membrane that causes an abnormal shape to the RBC. Destruction of these deformed cells occurs in the spleen and causes among other things severe anemia, increased bilirubin, and the formation of pigment gallstones.
Traditionally, the mainstay of treatment has been total splenectomy, but this is associated with the risk of potentially fatal overwhelming post-splenectomy infection (OPSI).
Partial splenectomy has been advocated as a means of improving the anemia and incidence of liver and gallbladder sequela while still retaining some splenic immune function against infection.
However, partial splenectomy can be problematic. Studies have shown that partial splenectomy does result in increased hemoglobin values and decreased reticulocyte and bilirubin levels.
Despite this, it appears that gallstone formation does still occur. In addition, in one study out of Canada partial splenectomy resulted in longer hospital stay, more postoperative pain, and a longer time to normal oral intake.