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Developmental CSF cysts of the posterior fossa

Analysis of 20 Operated Cases

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Summary

20 cases of operated developmental CSF cysts are surveyed: 8 Dandy-Walker syndromes and 12 extraaxial “arachnoid” cysts. A CSF shunt is the only possible operation for Dandy-Walker syndrome and a single shunt from the cyst is preferable to a double shunt; the success rate is 50%. Congenital extraaxial “arachnoid” cysts, which arise from a developmental anomaly of the roof of the hindbrain or primitive leptomeningeal spaces, must be differentiated from acquired arachnoid cysts. Those of this series fall into two groups: midline and lateral. Supravermian midline cysts resemble the Dandy-Walker syndrome in several respects, including the frequent recourse to a shunt operation and the uncertain distant prognosis, whilst the other extra-axial cysts usually simulate a cerebellar tumor. Lateral cysts may, however, present peculiar symptoms such as a homolateral motor deficit that does not always respond to surgery, and an oscillating symptomcomplex. Surgical removal is the treatment of choice both for subvermian midline and lateral cysts but subsequent shunting may be necessary to control persisting hydrocephalus. The long term prognosis is generally excellent for subvermian midline cysts but may be only fair for lateral cysts.

Zusammenfassung

Übersicht über 20 operierte Fälle von Mißbildungszysten:

8 mit Dandy-Walker-Syndrom und 12 extraaxiale “Arachnoidal”-Zysten.

Eine Liquor-Drainage erweist sich als die einzig mögliche Operation beim Dandy-Walker-Syndrom, und dabei ist eine einfache Ableitung der Cyste einem Doppel-Shunt vorzuziehen. Die Erfolgsrate beträgt 50%.

Extra-axiale “arachnoidale” Mißbildungszysten, die von einer Entwicklungsstörung des Daches des Rautenhirns oder den primitiven leptomeningealen Räumen ausgehen, müssen von erworbenen Arachnoidalzysten unterschieden werden. Die letzten zerfallen in zwei Gruppen: in der Mittellinie gelegene und laterale.

Supravermiale Mittellinien-Zysten ähneln in mancher Beziehung dem Dandy-Walker-Syndrom, einschließlich der häufigen Notwendigkeit einer Shunt-Operation und der unsicheren Spät-Prognose, während die anderen extra-axialen Zysten gewöhnlich einen Kleinhirn(-hemisphären)tumor imitieren.

Laterale Zysten können indessen besondere Symptome aufweisen, wie eine homolaterale motorische Schwäche, die sich nicht immer durch Operation bessert, und einen schwankenden Symptomenkomplex.

Operative Entfernung der Zyste ist die Behandlung der Wahl, sowohl bei oberhalb des Vermis gelegenen Mittellinien — als auch bei lateralen Zysten. Aber eine Shunt-Operation kann danach außerdem erforderlich sein, um einen persistierenden Hydrocephalus zu beherrschen. Die Spätprognose ist im allgemeinen für die supravermialen Zysten sehr gut, aber nur mäßig gut für die lateralen Zysten.

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  1. Institute of Neurosurgery of Rome Medical School, Viale dell' Università 30, 00185, Rome, Italy

    L. Palma, N. Di Lorenzo & S. Nicole

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Palma, L., Di Lorenzo, N. & Nicole, S. Developmental CSF cysts of the posterior fossa. Neurosurg. Rev. 2, 159–169 (1979). https://doi.org/10.1007/BF01650646

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