Abstract
Introduction
Recurrent tethered cord (RTC) is almost the rule after the repair of myelomeningocele and quite frequent after the repair of lipomyelomeningocele, resulting from the adhesions of the placode within a too narrow spinal canal. About one-third of patients with myelomeningocele and 10 % of those with spinal lipoma develop symptomatic RTC, mainly caused by the ischemic–metabolic injuries due to the cord stretching. The goal of this review is to provide information about the pathophysiology, the radiological picture, and the management of RTV according to the pertinent literature and the authors’ experience.
Radiological investigation
The magnetic resonance imaging (MRI) picture is characterized by a low position of the conus and by tethering of the spinal cord to the subcutaneous scar or to the inner surface of the spinal canal. The radiological work-up always includes brain MRI, to rule out other possible causes of late neurological deterioration (as shunt malfunction), and MRI of the whole spinal cord, to detect possible associated lesions (syringomyelia, dermoids, etc.). X-rays and/or computed tomography scan of the spine is required for the assessment of scoliosis or other bony malformations.
Management
The surgical treatment is planned after a multidisciplinary neurological, urological, orthopedic, physiatric, and radiological evaluation. The surgical detethering is carried out cautiously, possibly with electrophysiological intraoperative monitoring. Surgery ensures improvement or stability of the clinical picture in 70–80 % of cases, the remaining 20–30 % of patients needing multiple operations for their recovery. Complications may affect up to one-third of operated patients, being mainly represented by CSF leak, pseudomeningocele, and shunt malfunction.
Similar content being viewed by others
References
Albright AL, Pollack IF, Adelson PD, Solot JJ (1999) Outcome data and analysis in pediatric neurosurgery. Neurosurgery 45:101–106
Bamba Y, Nonaka M, Nakajima S, Yamasaki M (2011) Three-dimensional reconstructed computed tomography-magnetic resonance fusion image-based preoperative planning for surgical procedures for spinal lipoma or tethered spinal cord after myelomeningocele repair. Neurol Med Chir (Tokyo) 51:397–402
Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA (2001) Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 34:114–120
Bowman RM, McLone DG (2008) Tethered cord in children with spina bifida. In: Özek MM, Cinalli G, Maixner W (eds) Spina bifida: management and outcome. Springer, Milan, pp 267–274
Bowman RM, Mohan A, Ito J, Seibly JM, McLone DG (2009) Tethered cord release: a long-term study in 114 patients. J Neurosurg Pediatr 3:181–187
Brezner A, Kay B (1999) Spinal cord ultrasonography in children with myelomeningocele. Dev Med Child Neurol 41:450–455
Browd SR, Zauberman J, Karandikar M, Ojemann JG, Avellino AM, Ellenbogen RG (2009) A new fiber-mediated carbon dioxide laser facilitates pediatric spinal cord detethering. Technical note. J Neurosurg Pediatr 4:280–284
Caldarelli M, Di Rocco C, Colosimo C Jr, Fariello G, Di Gennaro M (1995) Surgical treatment of late neurological deterioration in children with myelodysplasia. Acta Neurochir 137:199–206
Carstens C, Koch H, Brocai DR, Nietard FU (1996) Development of pathological lumbar kyphosis in myelomeningocele. J Bone Joint Surg 78B:945–950
Cochrane DD, Rassehk SR, Thiessen PN (1998) Functional deterioration after placode untethering in myelomeningocele. Pediatr Neurosurg 28:57–62
Deletis V, Sala F (2008) Intraoperative neurophysiological monitoring of the spinal cord during spinal cord and spine surgery: a review focus on the corticospinal tracts. Clin Neurophysiol 119:248–264
Filler AG, Briton JA, Uttley D, Marsh HT (1995) Adult postrepair myelomeningocele and tethered cord syndrome: good surgical outcome after abrupt neurological decline. Br J Neurosurg 9:659–666
Filippi CG, Andrews T, Gonyea JV, Linnel G, Cauley KA (2010) Magnetic resonance diffusion tensor imaging and tractography of the lower spinal cord: application to diastematomyelia and tethered cord. Eur Radiol 20:2194–2199
Fone PD, Vapnek JM, Litwiller SE, Couillard DR, McDonald CM, Boggan JE, Stone AR (1997) Urodynamic findings in the tethered spinal cord syndrome: does surgical release improve bladder function? J Urol 157:604–609
George TM, Fagan LH (2005) Adult tethered cord syndrome in patients with postrepair myelomeningocele: an evidence-based outcome study. J Neurosurg Pediatr 102:150–156
Herman JM, McLone DG, Storrs BB, Dauser RC (1993) Analysis of 153 patients with myelomeningocele or spinal lipoma reoperated upon for a tethered cord. Presentation, management and outcome. Pediatr Neurosurg 19:243–249
Hoffman HJ, Taecholarn C, Hendrick EB, Humphreys RP (1985) Management of lipomyelomeningoceles. Experience at the Hospital for Sick Children, Toronto. J Neurosurg 62:1–8
Hoving EW, Haitsma E, Oude Ophuis CM, Journée HL (2011) The value of intraoperative neurophysiological monitoring in tethered cord surgery. Childs Nerv Syst 27:1445–1452
Hsieh PC, Stapleton CJ, Moldavskiy P, Koski TR, Ondra SL, Gokaslan ZL, Kuntz C (2010) Posterior vertebral column subtraction osteotomy for the treatment of tethered cord syndrome: review of the literature and clinical outcomes of all cases reported to date. Neurosurg Focus 29:E6
Hudgins RJ, Gilreath CL (2004) Tethered spinal cord following repair of myelomeningocele. Neurosurg Focus 16:E7
Husain AM, Shah D (2009) Prognostic value of neurophysiologic intraoperative monitoring in tethered cord syndrome surgery. J Clin Neurophysiol 26:244–247
Huttmann S, Krauss J, Collmann H, Roosen K (2001) Surgical management of tethered spinal cord in adults: report of 54 cases. J Neurosurg Spine 95:173–178
Iskandar BJ, Fulmer BB, Hadley MN, Oakes WJ (1998) Congenital tethered spinal cord syndrome in adults. J Neurosurg 88:958–961
Iskandar BJ, McLaughlin C, Oakes WJ (2000) Split cord malformations in myelomeningocele patients. Br J Neurosurg 14:200–203
Johnson DL, Levy LM (1995) Predicting outcome in the tethered cord syndrome: a study of cord motion. Pediatr Neurosurg 22:115–119
Kale SS, Mahapatra AK (1998) The role of somatosensory evoked potentials in spinal dysraphism—do they have a prognostic significance? Childs Nerv Syst 14:328–331
Kanev PN, Lemire RJ, Loeser JD, Berger MS (1990) Management and long-term follow-up review of children with lipomyelomeningocele, 1952–1987. J Neurosurg 73:48–52
Khealani B, Husain AM (2009) Neurophysiologic intraoperative monitoring during surgery for tethered cord syndrome. J Clin Neurophysiol 26:76–81
Klekamp J, Raimondi AJ, Samii M (1994) Occult dysraphism in adulthood: clinical course and management. Childs Nerv Syst 10:312–320
Kokubun S, Ozawa H, Aizawa T, Ly NM, Tanaka Y (2011) Spine-shortening osteotomy for patients with tethered cord syndrome caused by lipomyelomeningocele. J Neurosurg Spine 15:21–27
Lew SM, Kothbauer KF (2007) Tethered cord syndrome: an updated review. Pediatr Neurosurg 43:236–248
Mahapatra A, Gupta D (2005) The split cord malformations: a clinical study of 254 patients and a proposal for a new clinical-imaging classification. J Neurosurg 103(6 Suppl Pediatrics):531–536
Maher CO, Goumnerova L, Madsen JR, Proctor M, Scott RM (2007) Outcome following multiple repeated spinal cord untethering operations. J Neurosurg 106:434–438
Martínez-Lage JF, Ruiz-Espejo Villar A, Almagro MJ, Sánchez del Rincón I, Ros de San Pedro J, Felipe-Murcia M, Murcia-García FJ (2007) Spinal cord tethering in myelomeningocele and lipomeningocele patients: the second operation. Neurocir 18:312–319 [Spanish]
Massimi L, Peraio S, Peppucci E, Tamburrini G, Di Rocco C (2011) Section of the filum terminale: is it worthwhile in Chiari type I malformation? Neurol Sci 32(suppl 3):S349–S351
McLone DG, Naidich TP (1986) Laser resection of fifty spinal lipomas. Neurosurgery 18:611–615
Mooney JF, Glazier SS, Barfield WR (2012) Concurrent orthopedic and neurosurgical procedures in pediatric patients with spinal deformity. J Pediatr Orthop B 21:602–605
Nakanishi K, Tanaka N, Kamei N, Nakamae T, Izumi B, Ohta R, Fujioka Y, Ochi M (2013) Use of prone position magnetic resonance imaging for detecting the terminal filum in patients with occult tethered cord syndrome. J Neurosurg Spine 18:76–84
Oi S, Yamada H, Matsumoto S (1990) Tethered cord syndrome versus low-placed conus medullaris in an over-distended spinal cord following initial repair for myelodysplasia. Childs Nerv Syst 6:264–269
Özek MM, Belirgen M (2008) Vertebral anomalies and spinal malformations in myelomeningocele. In: Özek MM, Cinalli G, Maixner W (eds) Spina bifida: management and outcome. Springer, Milan, pp 185–196
Pang D, Zovickian J, Oviedo A (2010) Long-term outcome of total and near-total resection of spinal cord lipomas and radical reconstruction of the neural placode, part II: outcome analysis and preoperative profiling. Neurosurgery 66:253–272
Pierre-Kahn A, Zerah M, Renier D, Cinalli G, Sainte-Rose C, Lellouch-Tubiana A, Brunelle F, Le Merrer M, Giudicelli Y, Pichon J, Kleinknecht B, Nataf F (1997) Congenital lumbosacral lipomas. Childs Nerv Syst 13:298–334
Pouratian N, Elias WJ, Jane JA Jr, Phillips LH 2nd, Jane JA Sr (2010) Electrophysiologically guided untethering of secondary tethered spinal cord syndrome. Neurosurg Focus 29:E3
Rajasekaran S, Kanna RM, Shetty AP (2012) Diffusion tensor imaging of the spinal cord and its clinical applications. J Bone Joint Surg Br 94:1024–1031
Reigel DH, Tchernoukha K, Bazmi B, Kortyna R, Rotenstein D (1994) Change in spinal curvature following release of tethered spinal cord associated with spina bifida. Pediatr Neurosurg 20:30–42
Rendeli C, Ausili E, Tabacco F, Focarelli B, Massimi L, Caldarelli M, Tamburrini G, Di Rocco C (2007) Urodynamic evaluation in children with lipomeningocele: timing for neurosurgery, spinal cord tethering and followup. J Urol 177:2319–2324
Sakamoto H, Hakuba A, Fujitani K, Nishimura S (1991) Surgical treatment of the tethered spinal cord after repair of lipomyelomeningocele. J Neurosurg 74:709–714
Sala F, Krzan MJ, Deletis V (2002) Intraoperative neurophysiological monitoring in pediatric neurosurgery: why, when, how? Childs Nerv Syst 18:264–287
Sharma U, Pal K, Pratap A, Gupta DK, Jagannathan NR (2006) Potential of proton magnetic resonance spectroscopy in the evaluation of patients with tethered cord syndrome following surgery. J Neurosurg 105(5 Suppl Pediatrics):396–402
Shih P, Halpin RJ, Ganju A, Liu JC, Koski TR (2010) Management of recurrent adult tethered cord syndrome. Neurosurg Focus 29:E5
Tani S, Yamada S, Knighton RS (1987) Extensibility of the lumbar and sacral cord. Pathophysiology of the tethered spinal cord in cats. J Neurosurg 66:116–123
Trivedi J, Thomson JD, Slakey JB, Banta JV, Jones PW (2002) Clinical and radiographic predictors of scoliosis in patients with myelomeningocele. J Bone Joint Surg 84A:1389–1394
van Leeuwen R, Notermans NC, Vandertop WP (2001) Surgery in adults with tethered cord syndrome: outcome study with independent clinical review. J Neurosurg Spine 94:205–209
Vernet O, O’Gorman AM, Farmer JP, McPhillips M, Montes JL (1996) Use of the prone position in the MRI evaluation of spinal cord rethetering. Pediatr Neurosurg 25:286–294
Yamada S, Iacono RP, Andrade T, Mandybur G, Yamada BS (1995) Pathophysiology of tethered cord syndrome. Neurosurg Clin N Am 6:311–323
Yamada S, Won DJ, Yamada SM (2004) Pathophysiology of tethered cord syndrome: correlation with symptomatology. Neurosurg Focus 16:E6
Zerah M, Roujeau T, Catala M, Pierre-Kahn A (2008) Spinal lipomas. In: Özek MM, Cinalli G, Maixner W (eds) Spina bifida: management and outcome. Springer, Milan, pp 445–474
Zide B, Constantini S, Epstein FJ (1995) Prevention of recurrent tethered spinal cord. Pediatr Neurosurg 22:111–114
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Caldarelli, M., Boscarelli, A. & Massimi, L. Recurrent tethered cord: radiological investigation and management. Childs Nerv Syst 29, 1601–1609 (2013). https://doi.org/10.1007/s00381-013-2150-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-013-2150-4