Journal article
Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium
Journal of neurosurgery. Pediatrics, Vol.25(6), pp.629-639
06/01/2020
DOI: 10.3171/2020.1.PEDS19493
PMID: 32114543
Abstract
OBJECTIVE Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established.
METHODS Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of <= 2 mm or <= 3 mm or a reduction in AP diameter of >= 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (>= 1 mm), or 2) change in syrinx length (craniocaudal, >= 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length.
RESULTS The authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a <= 2-mm or <= 3-mm AP diameter or >= 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.160.91], p = 0.03) and smaller syrinx diameter (5.82 +/- 3.38 vs 7.86 +/- 3.05 mm; HR 0.60 [95% CI 0.34-1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 +/- 4.01 vs 9.65 +/- 4.31 levels; HR 0.21 [95% CI 0.12-0.38], p = 0.0001) and smaller pB-C2 distance (6.86 +/- 1.27 vs 7.18 +/- 1.38 mm; HR 1.44 [95% CI 1.02-2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 +/- 5.02 vs 10.29 +/- 4.25 years; HR 1.89 [95% CI 1.31-3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 +/- 3.64 vs 3.97 +/- 3.07 mm; HR 3.10 [95% CI 1.67- 5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate.
CONCLUSIONS These data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials. gov) will best answer this question.
Details
- Title: Subtitle
- Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium
- Creators
- Andrew T. Hale - Vanderbilt UniversityP. David Adelson - Barrow Neurological InstituteGregory W. Albert - Arkansas Children's HospitalPhilipp R. Aldana - Florida CollegeTord D. Alden - Lurie Children's HospitalRichard C. E. Anderson - Morgan Stanley Children's HospitalDavid F. Bauer - Dartmouth–Hitchcock Medical CenterChristopher M. Bonfield - Monroe Carell Jr. Children's Hospital at VanderbiltDouglas L. Brockmeyer - Primary Children's HospitalJoshua J. Chern - Children's Healthcare of AtlantaDaniel E. Couture - Wake Forest UniversityDavid J. Daniels - 13Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota;Susan R. Durham - University of VermontRichard G. Ellenbogen - Seattle Children'sRamin Eskandari - Medical University of South CarolinaTimothy M. George - Dell Children's Medical Center of Central TexasGerald A. Grant - Lucile Packard Children's HospitalPatrick C. Graupman - 19Division of Pediatric Neurosurgery, Gillette Children’s Hospital, St. Paul, Minnesota;Stephanie Greene - University of Pittsburgh Medical CenterJeffrey P. Greenfield - NewYork–Presbyterian HospitalNaina L. Gross - University of Oklahoma Health Sciences CenterDaniel J. Guillaume - University of MinnesotaGregory G. Heuer - Children's Hospital of PhiladelphiaMark Iantosca - Penn State Milton S. Hershey Medical CenterBermans J. Iskandar - University of Wisconsin–MadisonEric M. Jackson - Johns Hopkins MedicineJames M. Johnston - University of Alabama at BirminghamRobert F. Keating - Children’s National Health SystemJeffrey R. Leonard - Nationwide Children's HospitalCormac O. Maher - University of Michigan–Ann ArborFrancesco T. Mangano - Cincinnati Children's Hospital Medical CenterJ. Gordon McComb - Children's Hospital of Los AngelesThanda Meehan - Washington University in St. LouisArnold H. Menezes - University of IowaBrent O'Neill - Children's Hospital ColoradoGreg Olavarria - Arnold Palmer Hospital for ChildrenTae Sung Park - Washington University in St. LouisJohn Ragheb - University of MiamiNathan R. Selden - Doernbecher Children's HospitalManish N. Shah - 40Division of Pediatric Neurosurgery, McGovern Medical School, Houston, Texas.Matthew D. Smyth - Washington University in St. LouisScellig S. D. Stone - Boston Children's HospitalJennifer M. Strahle - Washington University in St. LouisScott D. Wait - Carolina Neurosurgery and Spine AssociatesJohn C. Wellons - Monroe Carell Jr. Children's HospitalWilliam E. Whitehead - Texas Children's HospitalChevis N. Shannon - Monroe Carell Jr. Children's HospitalDavid D. Limbrick - Washington University in St. LouisPark-Reeves Syringomyelia Research Consortium Investigators
- Resource Type
- Journal article
- Publication Details
- Journal of neurosurgery. Pediatrics, Vol.25(6), pp.629-639
- Publisher
- Amer Assoc Neurological Surgeons
- DOI
- 10.3171/2020.1.PEDS19493
- PMID
- 32114543
- ISSN
- 1933-0707
- eISSN
- 1933-0715
- Number of pages
- 11
- Grant note
- F30HL143826 / National Institutes of Health; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA 5T32GM007347 / Vanderbilt University Medical Scientist Training Program
- Language
- English
- Date published
- 06/01/2020
- Academic Unit
- Stead Family Department of Pediatrics; Neurosurgery; Otolaryngology
- Record Identifier
- 9984303905102771
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