Abstract
OBJECTIVE The relationship between a tethered cord (TC) and neurofbromatosis type 1 (NF1) and NF2 is not known. The purpose of this study was to defne 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 flum 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 flum lipoma, defned 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 fbroconnective tissue and blood vessels. CONCLUSIONS Despite the lack of any known pathophysiological relationship between NF and TC, the incidence of a symptomatic TC in patients with NF1 and NF2 who presented for any reason to this tertiary care pediatric neurosurgery clinic was 13%. Counseling patients and families regarding TC symptomatology might be indicated in this patient population.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 456-459 |
| Number of pages | 4 |
| Journal | Journal of Neurosurgery: Pediatrics |
| Volume | 21 |
| Issue number | 5 |
| DOIs | |
| State | Published - May 1 2018 |
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Keywords
- Filum lipoma
- Neurofibromatosis
- Pediatric
- Spine
- Tethered cord
ASJC Scopus subject areas
- Surgery
- Pediatrics, Perinatology, and Child Health
- Clinical Neurology
Cite this
Incidence of symptomatic tethered spinal cord in pediatric patients presenting with neurofbromatosis types 1 and 2. / Quinsey, Carolyn S.; Krause, Katie; Baird, Lissa; Sayama, Christina; Selden, Nathan.
In: Journal of Neurosurgery: Pediatrics, Vol. 21, No. 5, 01.05.2018, p. 456-459.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Incidence of symptomatic tethered spinal cord in pediatric patients presenting with neurofbromatosis types 1 and 2
AU - Quinsey, Carolyn S.
AU - Krause, Katie
AU - Baird, Lissa
AU - Sayama, Christina
AU - Selden, Nathan
PY - 2018/5/1
Y1 - 2018/5/1
N2 - OBJECTIVE The relationship between a tethered cord (TC) and neurofbromatosis type 1 (NF1) and NF2 is not known. The purpose of this study was to defne 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 flum 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 flum lipoma, defned 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 fbroconnective tissue and blood vessels. CONCLUSIONS Despite the lack of any known pathophysiological relationship between NF and TC, the incidence of a symptomatic TC in patients with NF1 and NF2 who presented for any reason to this tertiary care pediatric neurosurgery clinic was 13%. Counseling patients and families regarding TC symptomatology might be indicated in this patient population.
AB - OBJECTIVE The relationship between a tethered cord (TC) and neurofbromatosis type 1 (NF1) and NF2 is not known. The purpose of this study was to defne 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 flum 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 flum lipoma, defned 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 fbroconnective tissue and blood vessels. CONCLUSIONS Despite the lack of any known pathophysiological relationship between NF and TC, the incidence of a symptomatic TC in patients with NF1 and NF2 who presented for any reason to this tertiary care pediatric neurosurgery clinic was 13%. Counseling patients and families regarding TC symptomatology might be indicated in this patient population.
KW - Filum lipoma
KW - Neurofibromatosis
KW - Pediatric
KW - Spine
KW - Tethered cord
UR - http://www.scopus.com/inward/record.url?scp=85048246351&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85048246351&partnerID=8YFLogxK
U2 - 10.3171/2017.12.PEDS17306
DO - 10.3171/2017.12.PEDS17306
M3 - Article
C2 - 29473815
AN - SCOPUS:85048246351
VL - 21
SP - 456
EP - 459
JO - Journal of neurosurgery. Pediatrics
JF - Journal of neurosurgery. Pediatrics
SN - 1933-0707
IS - 5
ER -