Splenectomy can improve chronic hemolytic anemias, such as pyruvate kinase deficiency (PK) and hereditary spherocytosis (HS). However, total splenectomy (T.S) results in partial immune defect and
increases risk of overwhelming post-splenectomy infection (OPSI) especially in young children. Partial splenectomy (P.S) has emerged as an alternative to T.S and successfully used in HS. It can be done at
younger age, and is not associated with OPSI. Reports on P.S in pediatric population are still sparse, and possible benefit in other types of hemolytic anemia unclear. Aim. Evaluate our local experience
with P.S.
Method: Retrospective chart review between 1995-2005. Need to perform 2ary T.S used as an outcome measure. Patients requiring T.S within 1 yr of P.S (early T.S group) were compared with those having a
T.S performed at a later time or not (late or no T.S group). T-test used for statistical analysis.
Results: 7 patients (2 PK and 5 HS) had a P.S done at a mean age of 4.4 yrs (27m.-9 yrs). 2/5 HS patients also had congenital heart disease (CHD). Surgical procedure was laparoscopy in all but one. All patients were followed by ultrasound 2x year. There were no surgical or infectious complications. In 1/2 PK patients, no decrease of transfusion requirements was observed and T.S performed within 12 months; the 2nd patient showed transient improvement but eventually had T.S 2½ yrs later. In 3/5 HS patients, T.S was performed within 12 months because of persistent hemolytic anemia. 2 HS patients had clear improvement: one with complex CHD needed no transfusions for 4 yrs, but hemolytic anemia worsened during the 5th
year, and eventually had T.S. The 5th HS patient is still doing well almost 5yrs post P.S. Overall, 6/7 patients required T.S of which 4 were done <1 year after P.S. All 3 patients <3 yrs belonged to the
"early T.S" group. While all patients had spleen reduction by 50-70% by surgery, spleen size in the "early T.S" group returned more rapidly to pre-surgery size than those of "late or no T.S" group (spleen
size within 1st year of P.S: 92% compared to 54% respectively; P = 0.001).
Conclusions: P.S is a safe procedure. It can permit a gain of several months with reduced PRBC requirement, but rapid spleen regrowth seems to predict the need for T.S. While P.S benefited the most in H.S patients, its role in PK and in patients operated at a very young age remains questionable. Indication and benefit of P.S. need to be determined in a larger prospective study.