title: Diagnosis and outcome of childhood perineurioma.
abstract: BACKGROUND
  Intraneural perineurioma is a rare peripheral nerve tumor of childhood and early adulthood. Patients demonstrate progressive muscle weakness and atrophy largely without sensory complaints.
  
  
  METHODS
  We report two children with perineurioma affecting the radial and femoral nerves. Electromyography (EMG), ultrasound, and 3-T MR imaging were important tools for localizing perineurioma and permitting its differentiation from other nerve lesions. The first patient underwent surgical excision of the perineurioma and a traditional nerve graft. At 10 months post-operative follow-up, she demonstrated no meaningful recovery of muscle strength compared to her pre-operative assessment. EMG did confirm axonal continuity indicating that reinnervation had occurred via the nerve graft. The second patient underwent a two-staged surgical procedure that included an end-to-side nerve transfer. At 18 months post-operative follow-up, she demonstrated mild improvement in muscle strength and EMG evidence of ongoing reinnervation.
  
  
  CONCLUSIONS
  The surgical management of perineurioma remains controversial, and reports of clinical recovery after nerve grafts and nerve transfers vary. Nerve transfers have been reported to provide superior results to traditional nerve grafting in adults with post-traumatic plexus injuries. The modest gain in strength of our patient who underwent a nerve transfer raises the question if this may also apply to patients with perineurioma. Additional studies will be required, which must also take into consideration that features of long-standing neuropathy (i.e., limb length discrepancy) have the potential to reduce the likelihood of reinnervation and clinical recovery.
authors.affiliation: Children's Hospital of Eastern Ontario Research Institute, University of Ottawa, 401 Smyth Rd, Ottawa, ON, K1H 8L1, Canada. hmcmillan@cheo.on.ca.
authors.forename: Hugh J
authors.initials: HJ
authors.lastname: McMillan
authors.affiliation: The Ottawa Hospital, Civic Campus, University of Ottawa, 1053 Carling Ave, Ottawa, ON, K1Y 4E9, Canada.
authors.forename: C
authors.initials: C
authors.lastname: Torres
authors.affiliation: Children's Hospital of Eastern Ontario Research Institute, University of Ottawa, 401 Smyth Rd, Ottawa, ON, K1H 8L1, Canada.
authors.forename: J
authors.initials: J
authors.lastname: Michaud
authors.affiliation: Children's Hospital of Eastern Ontario Research Institute, University of Ottawa, 401 Smyth Rd, Ottawa, ON, K1H 8L1, Canada.
authors.forename: Y
authors.initials: Y
authors.lastname: Ying
authors.affiliation: The Ottawa Hospital, Civic Campus, University of Ottawa, 1053 Carling Ave, Ottawa, ON, K1Y 4E9, Canada.
authors.forename: K U
authors.initials: KU
authors.lastname: Boyd
authors.affiliation: The Ottawa Hospital, Civic Campus, University of Ottawa, 1053 Carling Ave, Ottawa, ON, K1Y 4E9, Canada.
authors.forename: P R
authors.initials: PR
authors.lastname: Bourque
mesh_terms: D000293:Adolescent; D019468:Disease Management; D004576:Electromyography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D018396:Mucin-1 / Q000378:metabolism; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging / Q000503:physiopathology; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000601:surgery*; D017253:Neurofibromatoses / Q000378:metabolism; D010524:Peripheral Nervous System Neoplasms / Q000175:diagnosis* / Q000601:surgery*
chemical_list: D018396:Mucin-1
keywords: Magnetic resonance imaging; Nerve sheath neoplasm; Nerve transfer; Peripheral nervous system neoplasms