title: A Retrospective Analysis of Vision-Impairing Tumors Among 467 Patients with Neurofibromatosis Type 2.
abstract: BACKGROUND
  Vision is important for patients with hearing loss caused by neurofibromatosis type 2 (NF2). Tumors adjacent to the anterior visual pathway can potentially impair the vision. Only a few case reports and small-series studies have been reported.
  
  
  OBJECTIVE
  To evaluate the clinical features of tumors adjacent to the anterior visual pathway in a large series of patients with NF2.
  
  
  METHODS
  Seventy-three patients with potentially vision-impairing tumors were carefully screened from among 467 patients with NF2.
  
  
  RESULTS
  Among the 73 patients, 31 had intraorbital tumors, 21 had suprasellar meningiomas, and 21 had medial sphenoid ridge meningiomas. Of the 31 patients with intraorbital tumors, 17 had optic nerve sheath meningiomas, 9 had intraorbital schwannomas, 3 had spheno-orbital meningiomas, 1 had an anterior cranial fossa-orbital meningioma, and 1 had a cranio-orbital schwannoma. To the date of the last follow-up, 43 patients (58.9%) experienced visual loss. In most cases, hearing loss tended to occur earlier than visual loss. Six patients underwent early operations, and they recovered well without any further vision damage. Six other patients underwent operations after having no functional visual ability in the affected eyes, and their visual ability was not saved.
  
  
  CONCLUSIONS
  Tumors adjacent to the anterior visual pathway, although uncommon in patients with NF2, can cause progressive visual loss. Early surgical intervention seems to be the primary treatment strategy, except for in patients' optic nerve sheath meningiomas. If patients adopt a wait and see policy, regular visual examination seems to be mandatory.
authors: Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, Beijing, China.
authors: Peng
authors: P
authors: Li
authors: Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, Beijing, China.
authors: Zhenmin
authors: Z
authors: Wang
authors: Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, Beijing, China.
authors: Qiangyi
authors: Q
authors: Zhou
authors: Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, Beijing, China.
authors: Shiwei
authors: S
authors: Li
authors: Neural Reconstructional Department, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China.
authors: Jing
authors: J
authors: Zhang
authors: Neural Reconstructional Department, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China.
authors: Ying
authors: Y
authors: Wang
authors: Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, Beijing, China.
authors: Xingchao
authors: X
authors: Wang
authors: Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, Beijing, China.
authors: Bo
authors: B
authors: Wang
authors: Neural Reconstructional Department, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China.
authors: Fu
authors: F
authors: Zhao
authors: Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, Beijing, China.
authors: Pinan
authors: P
authors: Liu
authors: Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, Beijing, China. Electronic address: yangzhijunttyy@163.com.
authors: Zhijun
authors: Z
authors: Yang
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D001932:Brain Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D015984:Causality; D002648:Child; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D015897:Comorbidity; D005260:Female; D006311:Hearing Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000517:prevention & control; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D017678:Sex Distribution; D016896:Treatment Outcome; D014786:Vision Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000517:prevention & control*; D055815:Young Adult
keywords: Anterior visual pathway; Neurofibromatosis type 2; Retrospective analysis; Vision-impairing tumors