gms | German Medical Science

Objective: Vestibular schwannomas (VS) in children are rare and mostly associated with NF II. Surgical resection seems the most important treatment option in NF II since radiation is less effective and in the young age problematic. We reviewed our results in children who underwent VS resection in the last 6 years.

Methods: There were 14 children with 19 VS amongst almost 300 patients who underwent VS resection from 2005–2009. 12 children, aged 9–16 years, median 13 y, had NF II. In 7 children (Group A) an early, neurophysiologically guided intervention on 9 VS (stage T1) was performed at a time hearing was still good to excellent, aiming at bony decompression of the internal auditory canal, partial tumor removal and hearing preservation. 3 children (Group B) underwent surgery because of progressive VS size (T2 or T3a) with deteriorating but still useful hearing. 5 children with tumor size T1 (n=1), T2 (n=3), and T3 (n=1) had little or no useful hearing pre-op (Group C), in 2 a facial nerve palsy existed. Here the goal was total tumor removal and facial nerve preservation/reconstruction. The two non-NF II children belonged to this group.

Results: In 1 of 9 VS of Group A there was hearing loss, most likely due to injury of the labyrinthine artery, in the others functional hearing was preserved. In Group B hearing could only be preserved in 1 of 3 patients, without facial nerve impairment in any. In group C, residual hearing was lost in all, the VS was totally removed in 4 of 5. In one patient an additional facial nerve neurinoma was found and partially removed. 2 children had a new facial impairment post-operatively, which recovered over time.

Conclusions: Surgical strategy in NF II is strongly dependent on tumor size, tumor dynamics and hearing function. Our "early decompressive strategy" in T1 tumors with good to excellent hearing at age ≤16y showed good results, but is not without risks and long-term follow-up >10 years is required to compare results versus natural course. However, Group B shows that the chance of hearing preservation is lower at a time of tumor growth and already deteriorated hearing. In Group C, facial nerve function is the most important issue as well as radical tumor removal to avoid later brain stem compression and associated problems.