Br J Neurosurg. 2005 Feb;19(1):5-12.
Management of the patient and family with neurofibromatosis 2: a consensus conference statement.
Evans DG, Baser ME, O'Reilly B, Rowe J, Gleeson M, Saeed S, King A, Huson SM, Kerr R, Thomas N, Irving R, MacFarlane R, Ferner R, McLeod R, Moffat D, Ramsden R.
Department of Medical Genetics, St Mary's Hospital, Manchester, UK.
A consensus conference on neurofibromatosis 2 (NF2) was held in 2002 at
the request of the United Kingdom (UK) Neurofibromatosis Association,
with particular emphasis on vestibular schwannoma (VS) surgery. NF2
patients should be managed at specialty treatment centres, whose staff
has extensive experience with the disease. All NF2 patients and their
families should have access to genetic testing because presymptomatic
diagnosis improves the clinical management of the disease.
Some clinical manifestations of NF2, such as ocular abnormalities, can be
detected in infancy; therefore, clinical screening for at-risk members
of NF2 families can start at birth, with the first magnetic resonance
(MRI) scan at 10-12 years of age. Minimal interference, maintenance of
quality of life, and conservation of function or auditory
rehabilitation are the cornerstones of NF2 management, and the decision
points to achieve these goals for patients with different clinical
presentations are discussed.