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Management strategies in neurofibromatosis type 2
- Source :
- European Archives of Oto-Rhino-Laryngology. 260:12-18
- Publication Year :
- 2003
- Publisher :
- Springer Science and Business Media LLC, 2003.
-
Abstract
- The objective of this study was to review the experience of the Neurotology and Skull Base Surgery Unit of Addenbrooke's Hospital in Cambridge, England, in the management of patients with neurofibromatosis type 2 (NF2). This was a retrospective review of the institution's series conducted at a neurotological tertiary referral centre. Over a 17-year period (1984-2001), 35 patients with NF2 were managed. These patients presented with multiple cranio-spinal neoplasms, including 62 cerebellopontine angle tumours of which 59 were vestibular schwannomas (nine patients with unilateral tumours and 25 patients with bilateral tumours). Clinical presentation, diagnosis and patient management were reviewed. The outcome parameters measured were tumour progression, the incidence of complications (hearing deterioration and facial nerve palsy) and the need for secondary intervention. Five vestibular schwannomas were treated with stereotactic radiosurgery (gamma-knife), all of which showed evidence of progression in size and/or deterioration in hearing. Thirty-one VS were conservatively treated with annual surveillance. In nine cases, the tumours had a follow-up shorter than 6 months and were therefore excluded from the results. In 13 cases the VS did not progress in size, and the hearing remained stable, while in the remaining nine cases, tumour progression was evident. In fifteen cases, surgery was performed at the authors' institution. In 11 cases a translabyrinthine approach was adopted, and in the remaining four cases a retrosigmoid approach was preferred. In all these cases, tumour removal was total and facial nerve function was House-Brackmann grade I-III in 55%. Successful hearing preservation was elusive in those patients in whom a hearing preservation approach was attempted. NF2 remains an extremely challenging disorder for the neurotologist and the patient alike. Early diagnosis offers distinct advantages to the patients, their families and the community at large. Of the treatment modalities, surgery unequivocally offers the most superior tumour control. Hearing preservation remains a challenge in these patients, but is optimised by the early detection of tumours in the NF2 patient.
- Subjects :
- Adult
Male
Neurofibromatosis 2
medicine.medical_specialty
Adolescent
Radiosurgery
Severity of Illness Index
Neurotology
otorhinolaryngologic diseases
medicine
Humans
Neurofibromatosis type 2
Neurofibromatosis
Child
Retrospective Studies
Translabyrinthine approach
business.industry
Cranial Nerves
Retrospective cohort study
General Medicine
Middle Aged
Cerebellopontine angle
medicine.disease
Surgery
Otorhinolaryngology
Female
Neurosurgery
business
Subjects
Details
- ISSN :
- 14344726 and 09374477
- Volume :
- 260
- Database :
- OpenAIRE
- Journal :
- European Archives of Oto-Rhino-Laryngology
- Accession number :
- edsair.doi.dedup.....4af181cdbf2826d1b0b425421b7ae14d
- Full Text :
- https://doi.org/10.1007/s00405-002-0503-9