Incidence of symptomatic tethered spinal cord in pediatric patients presenting with neurofibromatosis types 1 and 2

Journal of Neurosurgery. Pediatrics
Carolyn S QuinseyNathan R Selden

Abstract

OBJECTIVE The relationship between a tethered cord (TC) and neurofibromatosis type 1 (NF1) and NF2 is not known. The purpose of this study was to define the incidence of TC in pediatric neurosurgical patients who present with NF. METHODS The authors performed a single-institution (tertiary care pediatric hospital) 10-year retrospective analysis of patients who were diagnosed with or who underwent surgery for a TC and/or NF. Clinical and radiological characteristics were analyzed, as was histopathology. RESULTS A total of 424 patients underwent surgery for a TC during the study period, and 67 patients with NF were seen in the pediatric neurosurgery clinic. Of these 67 patients, 9 (13%) were diagnosed with a TC, and filum lysis surgery was recommended. Among the 9 patients with NF recommended for TC-release surgery, 4 (44%) were female, the mean age was 8 years (range 4-14 years), the conus position ranged from L1-2 to L-3, and 3 (33%) had a filum lipoma, defined as high signal intensity on T1-weighted MR images. All 9 of these patients presented with neuromotor, skeletal, voiding, and/or pain-related symptoms. Histopathological examination consistently revealed dense fibroconnective tissue and blood vessels. CONCLUSIONS Despite ...Continue Reading

References

Apr 15, 2003·Journal of Neurosurgery·Rebecca M AllenNathan R W Selden
Feb 16, 2005·European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society·Athanasios I TsirikosM Hilali Noordeen
Sep 15, 2006·Journal of Neurosurgery·Nathan R SeldenDavid B Lashley
Jan 11, 2014·Journal of Neurosurgery. Pediatrics·Eric M ThompsonNathan R Selden
Mar 19, 2014·Journal of Neurosurgery. Pediatrics·Michael J CoolsCormac O Maher
Sep 13, 2016·Journal of Pediatric Genetics·Jesse Lee Kresak, Meggen Walsh
Dec 3, 2016·Journal of Neurosurgery. Pediatrics·Brent R O'NeillMichael H Handler
Feb 24, 2017·Nature Reviews. Disease Primers·David H GutmannKimberly J Johnson

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