Chiari Malformation Type I with Concomitant Persistent Trigeminal Artery

Abstract

Background: Chiari malformation type I is a stenosis of the subarachnoid space at the level of the foramen magnum due to a small posterior fossa and herniation of the cerebellar tonsils. We present here the unusual case of a Chiari malformation in conjunction with a persistent trigeminal artery and hypoplasia of the basilar artery, also known as a Saltzman anomaly. Case Report: A 34-year-old female physician presented with recurrent headaches, bilateral weakness of deltoid muscles and numbness of the fingertips 3 to 5. A cranial MRI revealed a descent of the cerebellar tonsils to the level of C2 and a supracerebellar cyst. During surgery, a decompressive suboccipital craniectomy was performed. The supracerebellar cyst was fenestrated and the cerebellar tonsils were resected bilaterally. Two days after surgery the patient developed cranial nerve dysfunction and a right-sided hemiparesis. Cerebral angiography revealed a Saltzman type 1 anomaly with persistent primitive trigeminal artery and hypoplasia of the basilar artery. Discussion: This is the first report in the literature about the coincidence of both unusual anomalies. The latest literature of both rare anatomies and the unusual clinical course will be discussed.

Zusammenfassung

Hintergrund: Die Chiari-Malformation Typ I ist eine Stenose des Subarachnoidalraumes auf der Höhe des Foramen magnums, die durch eine kleine hintere Schädelgrube entsteht und zu einer Herniation der Kleinhirntonsillen führt. Wir beschreiben hier einen ungewöhnlichen Fall einer Chiari-Malformation in Verbindung mit einer persistierenden primitiven Trigeminalarterie und einer Hypoplasie der Arteria basilaris. Die begleitende vaskuläre Fehlbildung wird auch Saltzman-Anomalie genannt. Fallbeschreibung: Eine 34-jährige weibliche Ärztin stellte sich mit Kopfschmerzattacken und einer beidseitigen Schwäche des Musculus deltoideus sowie einem Taubheitsgefühl der Fingerspitzen der Finger 3 bis 5 vor. Ein kraniales Kernspintomogramm zeigte eine Verlagerung der Kleinhirntonsillen bis auf die Höhe C2 sowie eine suprazerebelläre Zyste. Während der Operation wurde eine dekompressive subokzipitale Kraniotomie durchgeführt. Die suprazerebelläre Zyste wurde gefenstert und die Kleinhirntonsillen beidseits reseziert. Zwei Tage nach der Operation entwickelte die Patientin Hirnnervenstörungen und eine rechtsseitige Hemiparese. Eine zerebrale Angiographie zeigte eine Saltzman-1-Anomalie mit persistierender primitiver Trigeminalarterie und Hypoplasie der Arteria basilaris. Diskussion: Dies ist der erste Bericht in der Literatur über die Koinzidenz beider seltenen Anomalien. Die neueste Literatur sowie der ereignisreiche klinische Verlauf werden diskutiert.

Key words

Chiari malformation - persistent primitive trigeminal artery

Schlüsselwörter

Chiari-Malformation - persistierende primitive Trigeminalarterie

References

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