Outcome from surgery for vestibular schwannomas in children

Br J Neurosurg. 2009 Jun;23(3):226-31. doi: 10.1080/02688690902968844.

Abstract

Object: A review of sporadic and NF2-related vestibular schwannoma surgery in children (under 18 years of age) with a specific interest in resection rates, recurrence, facial nerve outcomes, hearing preservation, hearing rehabilitation and genetic analysis.

Methods: A retrospective analysis of prospectively collected data of 35 consecutively operated vestibular schwannomas in 29 paediatric patients that underwent 38 operations between 1992 and 2007. Pre- and post-operative radiology, facial nerve function, pure tone audiogram and speech discrimination tests were performed with a mean follow-up of 4.5 years. Tumour and blood mutations were analysed in 86% of patients.

Results: Total resection was achieved in all sporadic cases and 68% of NF2 cases. Near total resection led to tumour recurrence in 5 out of 10 cases. The facial nerve was anatomically preserved in 92%. Facial nerve function was excellent to good (Grades 1-3) in 88% with outcome related to tumour size. Hearing preservation was successful in 3 of 11 cases.

Conclusions: Surgery with complete resection results in excellent tumour control, but it is more difficult to attain total resection in NF2 with a relatively high recurrence rate of persistently growing tumours. A better facial outcome is associated with smaller tumours, near-total resection and first time surgery. Hearing preservation is possible in a minority. Hearing rehabilitation can be successful by utilising cochlear implants and auditory brain stem implants (ABI) as appropriate. Overall there is a low complication rate and results are comparable with adult series.

MeSH terms

  • Adolescent
  • Auditory Brain Stem Implants
  • Auditory Threshold / physiology
  • Child
  • Cochlear Implants
  • Codon, Nonsense / genetics
  • Cranial Nerve Neoplasms / surgery
  • Facial Nerve Diseases / surgery
  • Facial Nerve* / physiology
  • Female
  • Hearing Disorders / prevention & control
  • Humans
  • Male
  • Neoplasm Recurrence, Local / pathology
  • Neurofibromatosis 2 / genetics
  • Neurofibromatosis 2 / pathology
  • Neurofibromatosis 2 / surgery*
  • Neuroma, Acoustic / genetics
  • Neuroma, Acoustic / pathology
  • Neuroma, Acoustic / surgery*
  • Outcome Assessment, Health Care
  • Postoperative Complications / prevention & control
  • Postoperative Complications / rehabilitation
  • Prognosis
  • Retrospective Studies
  • Speech Discrimination Tests

Substances

  • Codon, Nonsense