Pediatric Clinics of North America - CIPERJ

PARTIAL SPLENECTOMY FOR HEREDITARY SPHEROCYTOSIS
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Similarly, Soyer and colleagues [47] demonstrated an elevated rate of thrombosis
in a cohort of children who had hematologic conditions and were
undergoing splenectomy. At this time, it is unclear whether or not there is
a decreased risk for thrombosis after partial splenectomy compared with
the risk noted after total splenectomy. A demonstrable risk reduction for
thrombosis would further support the use of partial splenectomy not only
for children who have HS but also in other common conditions requiring
splenic resection.
Clinical studies of partial splenectomy in hereditary spherocytosis
For children who have HS, the role of partial splenectomy has been
evaluated in several nonrandomized clinical studies in Europe and North
America [7,13,48]. The following section summarizes the experience gained
to date [7,13,14,16,48–51].
French experience
The resurgence of and recent interest in using partial splenectomy was
stimulated by Tchernia and colleagues from the Hoˆ pital Bice` tre in Paris,
France [2,7,13]. This group advocates an 80% to 90% partial splenectomy
in children who have HS. In their most recent summary, 40 children who
had HS and underwent partial splenectomy were monitored for 1 to 14
years. In this nonrandomized trial, partial splenectomy resulted in a sustained
decrease in hemolytic anemia with a significantly increased hemoglobin
concentration, which improved from 9.2 2.6 g/dL at baseline to 12.3
1.9 g/dL at 3 to 4 years after surgery (P!.01). The increased hemoglobin
concentration was maintained for up to 10 years after surgery.
This group also demonstrated preserved phagocytic function of the
splenic remnant using radionuclide liver-spleen imaging in most children.
Furthermore, they demonstrated in a subset of children an increased red
cell lifespan averaging 6.5 days (range: 5 to 14.5 days). Tchernia and colleagues
[2,7,13] have shown that partial splenectomy does not abolish the
risk for aplastic crisis or the formation of gallstones, documenting that
a moderate degree of hemolysis persists in some children. Furthermore,
they have shown that the remnant spleen grew in some patients, although
there was no clear correlation between splenic regrowth and recurrent hemolytic
anemia. The authors have made this same fascinating observation.
In addition to the French group, a group in the Czech Republic recently
published their initial experience with a cohort of 14 children who had HS
and were undergoing partial splenectomy by a similar technique to that used
by Tchernia. In this nonrandomized study, the mean hemoglobin concentration
increased from 9.9 to 13.8 g/dL [52]. After 5 years of follow-up, no child
required late conversion to total splenectomy.