Chiari-Malformationen – Diagnostik und Therapie

Zusammenfassung

Es werden meist 5 verschiedene Chiari-Malformationen unterschieden (Grad 0 – IV), in der neueren Literatur wird zusätzlich von einer Chiari-1,5-Malformation und einer Chiari-V-Malformation gesprochen. Die einzelnen Chiari-Malformationen sind als komplett eigenständige Entitäten aufzufassen. Bei der Gruppe der Chiari-I-Malformationen können zusätzlich 5 Untergruppen unterschieden werden.

Alle Chiari-Malformationen (außer Grad IV) bedingen eine Liquorpulsationsbehinderung aus der hinteren Schädelgrube in Richtung Spinalkanal. Ansonsten sind das klinische Erscheinungsbild, die bildgebenden Befunde und insbesondere die Therapie jeder einzelnen Form und Unterform verschieden.

Die Indikation zur Operation muss individuell gestellt werden. Die Art der Operation bei einer Chiari-I-Malformation hängt von der Ursache der Tonsillenherniation ab. Bei Chiari-II-Malformation muss vor einer möglichen Operation die Shuntfunktion oder die Shuntnotwendigkeit intensiv überprüft werden.

• Literatur

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