Hereditary spherocytosis surgery
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
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Overview
Surgery
Splenectomy:
- Splenectomy is the definitive treatment for HS. [3] Except in the unusual autosomal recessive variant of HS, splenectomy usually eliminates hemolysis and the associated signs and symptoms. [4] Interestinagly, splenectomy does little to correct the cytoskeletal membrane defects of HS; an atomic force microscopy study by Li et al found that after splenectomy, erythrocytes were larger but still spheroidal-shaped, with a disorganized membrane ultrastructure and reduced surface particle size.
- Generally, the treatment of HS 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 S pneumoniae and H 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 HS 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. In children undergoing splenectomy, the gallbladder should be removed concomitantly if the patient has symptomatic gallstones . The study group could not come to consensus on the use of partial splenectomy. [21] In a study of 79 patients who underwent subtotal splenectomy (85-95% removal), with mean follow-up of approximately 11 years (range, 3-23 years), Pincez et al reported that the benefits varied according to disease severity. In children younger than 6 years with severe disease, the procedure reduces the transfusion rate and increases the hemoglobin to a level compatible with normal growth and activity. Half of those patients will not require total splenectomy; the other half will require it at an age when it will be much safer. [22] In a study that included 12 patients who underwent subtotal splenectomy at a mean age of 6.5 years, Rosman et al reported that in three children, the procedure was unsuccessful because no functional splenic remnant remained after 6 months; four children required secondary splenectomy for hematologic recurrence after a median of 5 years; and in the remaining five patients, a functional splenic remnant was present for at least 5.5 years. [23] In patients with intermediate HS, subtotal splenectomy avoids the long-term risk of infections and vascular events associated with total splenectomy. However, it comes with the cost of persistence of a lower but persistent hemolytic state. [22] A retrospective review by Abdullah et al of splenectomy for HS in over 1650 children found that the morbidity and mortality are low, and that performance of concurrent cholecystectomy and/or appendectomy is safe. In addition, Abdullah et al found that of 13 potentially avoidable complications identified as pediatric quality indicators by the Agency for Healthcare Research and Quality (AHRQ), none occurred in more than 1% of the splenectomized children. [4]