Asplenia in childhood may be congenital (e.g. Ivemark-syndrome) or acquired
(functional hyposplenism in sickle cell disease; after splenectomy or bone
marrow transplantation). Hereditary spherocytosis is the most common indic
ation for splenectomy in childhood. Virtually every patient without spleen
has a significantly increased risk of severe postsplenectomy infection (mos
tly caused by Streptococcus pneumoniae). Therefore, vaccinations against pn
eumococci, haemophilus influenzae and, under certain circumstances, meningo
cocci are recommended. In addition a continuous prophlyaxis with antibiotic
s should be performed for at least three years (or even longer depending on
the disease) after splenectomy followed by lifelong interventional applica
tion of broad spectrum antibiotics in case of any unclear infection or high
fever. This prophylaxis must be started as early as four months of age in
sickle cell disease. in future the use of penicillin may be hampered by the
growing resistance of pneumococci. Due to this fact the indication for spl
enectomy in childhood should be restricted to patients with hematologic dis
ease (spherocytosis and other hemolytic anemias, chronic ITP etc.) and mode
rate to severe symptoms. It is unclear whether partial splenectomy for sphe
rocytosis (and other hemolytic anemias) is an alternative regarding both lo
nglasting reduction of hemolysis and prevention of severe infection. After
trauma every effort should be undertaken to preserve a splenic remnant.