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Type 2 neurofibromatosis: the need for supraregional care?

Published online by Cambridge University Press:  29 June 2007

D. Gareth R. Evans ,
Richard Ramsden ,
Susan M. Huson ,
Rodney Harris ,
Richard Lye  and
Tom T. King
D. Gareth R. Evans*
Affiliation:
Department of Medical Genetics, St Mary's Hospital, Manchester
Richard Ramsden
Affiliation:
Department of Otolaryngology, Manchester Royal Infirmary, Manchester;
Susan M. Huson
Affiliation:
Department of Medical Genetics, Churchill Hospital, Oxford.
Rodney Harris
Affiliation:
Department of Medical Genetics, St Mary's Hospital, Manchester
Richard Lye
Affiliation:
Department of Neurosurgery, Manchester Royal Infirmary, Manchester;
Tom T. King
Affiliation:
Department of Neurosurgery, London Hospital, Whitechapel, London.
*
Dr D. G. R. Evans, Department of Medical Genetics, St Mary's Hospital, Hathersage Road, Manchester M13 0JH.
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Abstract

The results of a U.K. study of 145 cases of type 2 neurofibromatosis has shown generally very poor operative results in terms of hearing and facial nerve preservation. Only 9 out of 118 vestibular schwannoma (acoustic neuroma) operations resulted in any clinically detectable hearing preservation and only 32 left the patient with good or normal (House grade I or II) ipsilateral facial nerve function. Although operation is still the definitive treatment of vestibular schwannoma (acoustic neuroma) and may be a lifesaving procedure, it appears that the evidence in favour of early operation is only valid when carried out in highly specialized centres. The special problems of NF2 cases who may go on to develop multiple spinal and cranial tumours making them wheelchair bound and blind as well as deaf warrants a careful experienced approach. Timing of operations may be critical for the enhancement of useful years of quality life. We therefore propose the setting up of a national NF2 register, with the management of cases at a few supraregional centres.

Keywords

NeurofibromatosiscentralNF2

Information

Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 107 , Issue 5 , May 1993 , pp. 401 - 406
Copyright
Copyright © JLO (1984) Limited 1993

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