The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism, to determine the ongoing longitudinal risk for tethered cord release (TCR) among adults with myelomeningocele who had not previously undergone TCR during childhood. The authors also sought to identify the impact of lesion level, ambulation status, and prior treatments for hydrocephalus or Chiari malformations on TCR rates.
Adults in the registry who had not previously undergone TCR during childhood were studied. This group was compared with the remaining adults in the registry. The frequency of first-time TCR and time to TCR (using Kaplan-Meier analysis) were determined independently for males and females. Cox proportional hazards analysis identified correlations between sex, best lesion level and ambulation status prior to TCR, and previous treatments for hydrocephalus and Chiari decompression.
Among 967 adults in the NSBPR (422 [43.6%] males and 545 [56.4%] females) who had not undergone TCR during childhood, the authors identified 47 people (4.9%) who underwent their first TCR during adulthood. This study cohort had significantly better mean functional motor levels and ambulation compared with the remaining adult cohort (both p < 0.001). The study group included 35 females (74.5%) and 12 males (25.5%); this sex distribution was significantly different in comparison with the remaining adult cohort (p = 0.016). The Kaplan-Meier curves for first TCR for females and males were significantly different (p = 0.01, log-rank test). TCR rates were correlated with sex (males had decreased risk; OR 0.31, 95% CI 0.16–0.62, p < 0.001), prior treatment for hydrocephalus (those who underwent prior treatment had decreased risk; OR 0.21, 95% CI 0.20–0.42, p < 0.001), and prior treatment for Chiari malformation (those who underwent prior treatment had greater risk; OR 3.84, 95% CI 1.50–9.88, p = 0.005).
Adults with myelomeningocele who escape childhood without undergoing TCR have an ongoing, albeit decreased, risk for spinal cord tethering requiring TCR. This risk is obviously not due to spinal column growth and therefore must reflect other factors such as dynamic changes in spinal cord health over time. Among people with MMC who underwent their first TCR as adults, females seemed to be overrepresented. Similar to the authors’ prior childhood study, people who underwent previous Chiari decompression seemed to be overrepresented, whereas those who underwent previous treatment for hydrocephalus seem to be underrepresented. These novel findings deserve further study.
INCLUDE WHEN CITINGPublished online June 16, 2023; DOI: 10.3171/2023.5.SPINE2328.
DisclosuresThe authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
BamerAM,ConnellFA,DudgeonBJ,JohnsonKL.Frequency of purchase and associated costs of assistive technology for Washington State Medicaid program enrollees with spina bifida by age.Disabil Health J.2010;3(3):155–161.
OuyangL,GrosseSD,ArmourBS,WaitzmanNJ.Health care expenditures of children and adults with spina bifida in a privately insured U.S. population.Birth Defects Res A Clin Mol Teratol.2007;79(7):552–558.
DiasMS,WangM,RizkEB,et al.Tethered spinal cord among people with myelomeningocele: an analysis of the National Spina Bifida Patient Registry.J Neurosurg Pediatr.2021;28(1):21–27.
BowmanRM,McLoneDG,GrantJA,TomitaT,ItoJA.Spina bifida outcome: a 25-year prospective.Pediatr Neurosurg.2001;34(3):114–120.
BowmanRM,MohanA,ItoJ,SeiblyJM,McLoneDG.Tethered cord release: a long-term study in 114 patients.J Neurosurg Pediatr.2009;3(3):181–187.
BegeerJH,Meihuizen de RegtMJ,HogenEschI,Ter WeemeCA,MooijJJ,VenckenLM.Progressive neurological deficit in children with spina bifida aperta.Z Kinderchir.1986;41(suppl 1):13-15.
BowmanRM,McLoneDM.Tethered cord in children with spina bifida. In:OzekMM,CinalliG,MaixnerWJ, eds.Spina Bifida Management and Outcome.Springer-Verlag;2008:267-274.
KelloggR,LeeP,DeibertCP,et al.Twenty years’ experience with myelomeningocele management at a single institution: lessons learned.J Neurosurg Pediatr.2018;22(4):439–443.
PangD,WilbergerJEJr.Tethered cord syndrome in adults.J Neurosurg.1982;57(1):32–47.
YamadaS,IaconoRP,YamadaBS.Pathophysiology of the tethered spinal cord. In:YamadaS, ed.Tethered Cord Syndrome.American Association of Neurological Surgeons;1996:29-48.
YamadaS,WonDJ,YamadaSM.Pathophysiology of tethered cord syndrome: correlation with symptomatology.Neurosurg Focus.2004;16(2):E6.
YamadaS,ZinkeDE,SandersD.Pathophysiology of "tethered cord syndrome.".J Neurosurg.1981;54(4):494–503.
FillerAG,BrittonJA,UttleyD,MarshHT.Adult postrepair myelomeningocoele and tethered cord syndrome: good surgical outcome after abrupt neurological decline.Br J Neurosurg.1995;9(5):659–666.
AkayKM,ErşahinY,CakirY.Tethered cord syndrome in adults.Acta Neurochir (Wien).2000;142(10):1111–1115.
PiattJHJr.Treatment of myelomeningocele: a review of outcomes and continuing neurosurgical considerations among adults.J Neurosurg Pediatr.2010;6(6):515–525.
GeorgeTM,FaganLH.Adult tethered cord syndrome in patients with postrepair myelomeningocele: an evidence-based outcome study.J Neurosurg.2005;102(2 suppl):150–156.
SawinKJ,LiuT,WardE,et al.The National Spina Bifida Patient Registry: profile of a large cohort of participants from the first 10 clinics.J Pediatr.2015;166(2):444–50.e1.
ThibadeauJK,WardEA,SoeMM,et al.Testing the feasibility of a National Spina Bifida Patient Registry.Birth Defects Res A Clin Mol Teratol.2013;97(1):36–41.
AlabiNB,ThibadeauJ,WienerJS,et al.Surgeries and health outcomes among patients with spina bifida.Pediatrics.2018;142(3):e20173730.
EnglishWJ,MaltbyGL.Diastematomyelia in adults.J Neurosurg.1967;27(3):260–264.
GuptaSK,KhoslaVK,SharmaBS,MathuriyaSN,PathakA,TewariMK.Tethered cord syndrome in adults.Surg Neurol.1999;52(4):362–370.
HüttmannS,KraussJ,CollmannH,SörensenN,RoosenK.Surgical management of tethered spinal cord in adults: report of 54 cases.J Neurosurg.2001;95(2 suppl):173–178.
De KeyserJ,EbingerG,CalliauwL.The role of childbirth on the natural history of occult spinal dysraphism.J Neurol Neurosurg Psychiatry.1985;48(7):721–722.
PoolJL.Spinal cord and local signs secondary to occult sacral meningoceles in adults.Bull N Y Acad Med.1952;28(10):655–663.
ShurtleffDB.Myelodysplasia: management and treatment.Curr Probl Pediatr.1980;10(3):1–98.
BriggsVG.Population estimates of people with spina bifida in the United States in 2020.medRxiv.Preprint posted online February 3, 2022. doi:
PiattJHJr.Adults with myelomeningocele and other forms of spinal dysraphism: hospital care in the United States since the turn of the millennium.J Neurosurg Spine.2016;25(1):69–77.
BendtM,GabrielssonH,RiedelD,et al.Adults with spina bifida: a cross-sectional study of health issues and living conditions.大脑Behav.2020;10(8):e01736.
DominoJS,LundyP,GlynnEF,PartingtonM.Estimating the prevalence of neurosurgical interventions in adults with spina bifida using the Health Facts data set: implications for transition planning and the development of adult clinics.J Neurosurg Pediatr.2021;29(4):371–378.
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