Zusammenfassung.
Die Asplenie im Kindesalter kann angeboren oder erworben sein. Bei
jeder Form von Asplenie ist das Risiko für eine fulminant verlaufende
Sepsis und/oder Meningitis (meist durch Pneumokokken) erhöht. Jeder
Patient mit Milzverlust muss daher gegen Pneumokokken, Haemophilus influenzae
und ggf. Meningokokken geimpft sein. Darüber hinaus wird eine mindestens
dreijährige koninuierliche Antibiotika-Prophylaxe (oder in
Abhängigkeit von der Erkrankung auch länger bis lebenslang)
empfohlen. Im Anschluss ist ein lebenslanger interventioneller Einsatz eines
Breitbandantibiotikums bei allen hoch fieberhaften Infektionen erforderlich.
Dabei könnte die zunehmende Penicillinresistenz der Pneumokokken eine
wirksame Dauerprophylaxe nach Splenektomie in Zukunft erschweren. Die
Indikation zur Splenektomie bei hämatologischen Erkrankungen wird im
Kindesalter daher zunehmend zurückhaltender gestellt; möglicherweise
kann bei Patienten mit hereditärer Sphärozytose ein kleiner Milzrest
erhalten bleiben.
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
indication for splenectomy in childhood. Virtually every patient without spleen
has a significantly increased risk of severe postsplenectomy infection (mostly
caused by Streptococcus pneumoniae). Therefore, vaccinations against
pneumococci, haemophilus influenzae and, under certain circumstances,
meningococci are recommended. In addition a continuous prophlyaxis with
antibiotics should be performed for at least three years (or even longer
depending on the disease) after splenectomy followed by lifelong interventional
application 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
splenectomy in childhood should be restricted to patients with hematologic
disease (spherocytosis and other hemolytic anemias, chronic ITP etc.) and
moderate to severe symptoms. It is unclear whether partial splenectomy for
spherocytosis (and other hemolytic anemias) is an alternative regarding both
longlasting reduction of hemolysis and prevention of severe infection. After
trauma every effort should be undertaken to preserve a splenic remnant.
Schlüsselwörter
Asplenie - Postsplenektomie-Infektionen - Infektionsprophylaxe - Pneumokokken
Key words
Asplenia - antiinfectious prophylaxis - overwhelming postsplenectomy infections - pneumococci
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1917-1924
Prof. Dr. Stefan Eber
Universitäts-Kinderklinik
Steinwiesstr. 75
8032 Zürich
Telefon: 00 41-1-2 66-71 11
Fax: 00 41-1-2 66-71 71
eMail: stefan.eber@kispi.unizh.ch