Management von Patienten mit Vestibularisschwannomen Typ IV

Zusammenfassung

Hintergrund Vestibularisschwannome (VS) sind benigne Tumoren, die anhand der Hannover-Klassifikation bzw. der Koos-Klassifizierung eingeteilt werden. Trotz der umfangreichen Literatur sind die Klinik und die Behandlungskonzepte speziell bei großen VS selten beschrieben.

Material und Methoden Zwischen 2003 und 2018 wurden 61 Patienten mit VS Typ IV durch die Arbeitsgruppe Schädelbasischirurgie am Klinikum Dortmund behandelt. Die radiologischen und klinischen Daten wurden retrospektiv ausgewertet. Zudem erfolgte eine Subgruppenanalyse zwischen Patienten mit und ohne Kompression des IV. Ventrikels.

Ergebnisse Neben einer Hörminderung bei 55 Patienten (90 %) hatten die meisten Patienten multiple Symptome wie eine Trigeminusaffektion bei 16 (26 %), eine Fazialisparese bei 7 (12 %), eine Ataxie bei 27 (45 %) und Symptome eines Hirndruckanstiegs durch einen Hydrozephalus bei 4 Patienten (7 %). Bei Patienten mit einem VS Typ IVb wurde signifikant häufiger eine Ataxie, eine tonsilläre Herniation bzw. ein Hydrozephalus festgestellt. Eine komplette Resektion wurde in 48 Patienten (78 %) erreicht und eine weitestgehende Tumorentfernung in 12 Patienten (20 %). In der Langzeituntersuchung zeigten 90 % einen günstigen Outcome bezüglich des Nervus facialis (House-und-Brackman-Grad I–III). 6 Patienten (10 %) benötigten einen dauerhaften ventrikulo-peritonealen Shunt. Mehr als 90 % der Patienten erzielten einen Karnofsky-Index > 70 %.

Diskussion VS Typ IV sind häufig assoziiert mit Hydrozephalus, Ataxie, multiplen Hirnnervenausfällen und gelegentlich Zeichen eines intrakraniellen Druckanstiegs. Die primäre mikrochirurgische Resektion ist weiterhin eine entscheidende Therapieoption.

Abstract

Background Vestibular schwannomas (VSs) are benign, slow-growing tumors that are classified by the Koos and Hannover grading scales. Despite extensive study, the management of large VS remains controversial.

Patients and methods Between 2003 and 2018, 61 patients with VS grade IV were treated in our institution. Patient data and radiological images were retrospectively analyzed. Additionally, we performed a subgroup analysis of patients with and without fourth ventricle distortions.

Results Most patients presented with multiple symptoms, like hearing loss in 55 patients (90 %), trigeminal nerve affection in 16 (26 %), facial nerve affection in 7 (12 %), ataxia in 27 (45 %), and symptoms associated with increased intracranial pressure caused by hydrocephalus in 4 patients (7 %). Patients with type IV b VS presented significantly more often with ataxia, tonsillar herniation, and hydrocephalus. Complete tumor resection was achieved in 48 patients (79 %) and near-total resection was achieved in 12 patients (20 %). During long-term follow-up, 90 % of the patients had favorable outcomes regarding facial nerve function (House and Brackman grade I–III). In six patients (10 %), a ventriculoperitoneal shunt was inserted. More than 90 % of the patients had > 70 % functional impairment based on the Karnofsky Index.

Discussion Large VS is often associated hydrocephalus, ataxia, multiple cranial nerve impairments, and clinical signs of increased intracranial pressure. Primary microsurgical resection, as the primary treatment option, is associated with good outcomes in patients with large VS.

Schlüsselwörter

Vestibularisschwannome - hintere Schädelgrube - Hirnstammkompression - Fazialisparese - Trigeminusaffektion - Hannover-Klassifikation - okklusiver Hydrozephalus

Key words

vestibular schwannoma - posterior fossa - brainstem compression - facial nerve palsy - trigeminal nerve affection - Hannover grading scale

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