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Published in: Current Urology Reports 11/2015

01-11-2015 | Pediatric Urology (M Castellan and R Gosalbez, Section Editors)

Systematic Review of Urologic Outcomes from Tethered Cord Release in Occult Spinal Dysraphism in Children

Authors: Jeffrey T. White, Derek C. Samples, Juan C. Prieto, Izabela Tarasiewicz

Published in: Current Urology Reports | Issue 11/2015

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Abstract

Tethered cord syndrome describes a condition of multisystem end organ dysfunction due to fixation of the spinal cord. This systematic review focuses on the closed skin variant of this condition, occult spinal dysraphism. The embryology, pathophysiology, presentation, and classification of occult spinal dysraphism are explained to develop a simple framework for discussions regarding this often confusing condition. Following Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines, we synthesized urologic outcome data after tethered cord release in children from 17 studies performed over the past 25 years. These results prompted several conclusions. First, the different subgroups and different nomenclature of tethered cord syndrome are often confused, making interpretation of results difficult. Second, untethering has a positive effect on urologic symptoms and urodynamics parameters. Third, timing of untethering is important: early intervention prevents significant long-term traction aiming to avoid irreversible neurologic damage. Fourth, pediatric urologists and neurosurgeons have an important role in diagnosing and treating this condition and should work closely as part of a multidisciplinary team.
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Literature
1.
go back to reference Yamada S, Zinke DE, Sanders D. Pathophysiology of “tethered cord syndrome”. J Neurosurg. 1981;54(4):494–503.CrossRefPubMed Yamada S, Zinke DE, Sanders D. Pathophysiology of “tethered cord syndrome”. J Neurosurg. 1981;54(4):494–503.CrossRefPubMed
2.
go back to reference Blount JP, Elton S. Spinal lipomas. Neurosurg Focus. 2001;10(1), e3.PubMed Blount JP, Elton S. Spinal lipomas. Neurosurg Focus. 2001;10(1), e3.PubMed
3.
4.
go back to reference Pourquie O et al. Lateral and axial signals involved in avian somite patterning: a role for BMP4. Cell. 1996;84(3):461–71.CrossRefPubMed Pourquie O et al. Lateral and axial signals involved in avian somite patterning: a role for BMP4. Cell. 1996;84(3):461–71.CrossRefPubMed
5.
go back to reference Slack JMW. Essential developmental biology. 3rd ed. Chichester: Wiley; 2015. p. xi. 479 p. Slack JMW. Essential developmental biology. 3rd ed. Chichester: Wiley; 2015. p. xi. 479 p.
6.
go back to reference Shapiro E et al. Altered smooth muscle development and innervation in the lower genitourinary and gastrointestinal tract of the male human fetus with myelomeningocele. J Urol. 1998;160(3 Pt 2):1047–53. discussion 1079.CrossRefPubMed Shapiro E et al. Altered smooth muscle development and innervation in the lower genitourinary and gastrointestinal tract of the male human fetus with myelomeningocele. J Urol. 1998;160(3 Pt 2):1047–53. discussion 1079.CrossRefPubMed
7.•
go back to reference Shen J et al. Morphology of nervous lesion in the spinal cord and bladder of fetal rats with myelomeningocele at different gestational age. J Pediatr Surg. 2013;48(12):2446–52. Authors showed that fetal rats with myelomeningocele have altered innervation of the bladder.CrossRefPubMed Shen J et al. Morphology of nervous lesion in the spinal cord and bladder of fetal rats with myelomeningocele at different gestational age. J Pediatr Surg. 2013;48(12):2446–52. Authors showed that fetal rats with myelomeningocele have altered innervation of the bladder.CrossRefPubMed
8.
go back to reference Nielsen LA et al. Neural tube defects and associated anomalies in a fetal and perinatal autopsy series. APMIS. 2006;114(4):239–46.CrossRefPubMed Nielsen LA et al. Neural tube defects and associated anomalies in a fetal and perinatal autopsy series. APMIS. 2006;114(4):239–46.CrossRefPubMed
9.
go back to reference Amari F et al. Prenatal course and outcome in 103 cases of fetal spina bifida: a single center experience. Acta Obstet Gynecol Scand. 2010;89(10):1276–83.CrossRefPubMed Amari F et al. Prenatal course and outcome in 103 cases of fetal spina bifida: a single center experience. Acta Obstet Gynecol Scand. 2010;89(10):1276–83.CrossRefPubMed
10.
11.
go back to reference Stetler WR, Park P, Sullivan S. Pathophysiology of adult tethered cord syndrome: review of the literature. Neurosurg Focus. 2010;29(1), E2.CrossRefPubMed Stetler WR, Park P, Sullivan S. Pathophysiology of adult tethered cord syndrome: review of the literature. Neurosurg Focus. 2010;29(1), E2.CrossRefPubMed
12.
go back to reference Bui CJ, Tubbs RS, Oakes WJ. Tethered cord syndrome in children: a review. Neurosurg Focus. 2007;23(2), E2.PubMed Bui CJ, Tubbs RS, Oakes WJ. Tethered cord syndrome in children: a review. Neurosurg Focus. 2007;23(2), E2.PubMed
13.
go back to reference Powell KR et al. A prospective search for congenital dermal abnormalities of the craniospinal axis. J Pediatr. 1975;87(5):744–50.CrossRefPubMed Powell KR et al. A prospective search for congenital dermal abnormalities of the craniospinal axis. J Pediatr. 1975;87(5):744–50.CrossRefPubMed
14.
go back to reference James CCM, Lassman LP. Spina bifida occulta: orthopaedic, radiological and neurosurgical aspects. London: Academic Press, Grune & Stratton; 1981. p. x. 230 p. James CCM, Lassman LP. Spina bifida occulta: orthopaedic, radiological and neurosurgical aspects. London: Academic Press, Grune & Stratton; 1981. p. x. 230 p.
15.
go back to reference Hertzler DA et al. Tethered cord syndrome: a review of the literature from embryology to adult presentation. Neurosurg Focus. 2010;29(1), E1.CrossRefPubMed Hertzler DA et al. Tethered cord syndrome: a review of the literature from embryology to adult presentation. Neurosurg Focus. 2010;29(1), E1.CrossRefPubMed
16.
go back to reference Kuo MF et al. Tethered spinal cord and VACTERL association. J Neurosurg. 2007;106(3 Suppl):201–4.PubMed Kuo MF et al. Tethered spinal cord and VACTERL association. J Neurosurg. 2007;106(3 Suppl):201–4.PubMed
17.•
go back to reference Kucera JN et al. The simple sacral dimple: diagnostic yield of ultrasound in neonates. Pediatr Radiol. 2015;45(2):211–6. Retrospective review of the utility of ultrasound for a simple sacral dimple. Ultrasound was helpful in patients less than 6 months of age. Abnormal findings were not predictive of surgery at centers with a conservative management strategy.CrossRefPubMed Kucera JN et al. The simple sacral dimple: diagnostic yield of ultrasound in neonates. Pediatr Radiol. 2015;45(2):211–6. Retrospective review of the utility of ultrasound for a simple sacral dimple. Ultrasound was helpful in patients less than 6 months of age. Abnormal findings were not predictive of surgery at centers with a conservative management strategy.CrossRefPubMed
18.
go back to reference Wilson DA, Prince JR. John Caffey award. MR imaging determination of the location of the normal conus medullaris throughout childhood. AJR Am J Roentgenol. 1989;152(5):1029–32.CrossRefPubMed Wilson DA, Prince JR. John Caffey award. MR imaging determination of the location of the normal conus medullaris throughout childhood. AJR Am J Roentgenol. 1989;152(5):1029–32.CrossRefPubMed
19.
go back to reference Uchino A, Mori T, Ohno M. Thickened fatty filum terminale: MR imaging. Neuroradiology. 1991;33(4):331–3.CrossRefPubMed Uchino A, Mori T, Ohno M. Thickened fatty filum terminale: MR imaging. Neuroradiology. 1991;33(4):331–3.CrossRefPubMed
20.
go back to reference Wolf S, Schneble F, Tröger J. The conus medullaris: time of ascendence to normal level. Pediatr Radiol. 1992;22(8):590–2.CrossRefPubMed Wolf S, Schneble F, Tröger J. The conus medullaris: time of ascendence to normal level. Pediatr Radiol. 1992;22(8):590–2.CrossRefPubMed
21.
go back to reference Drake JM. Surgical management of the tethered spinal cord—walking the fine line. Neurosurg Focus. 2007;23(2), E4.CrossRefPubMed Drake JM. Surgical management of the tethered spinal cord—walking the fine line. Neurosurg Focus. 2007;23(2), E4.CrossRefPubMed
22.
23.
go back to reference Saifuddin A, Burnett SJ, White J. The variation of position of the conus medullaris in an adult population. A magnetic resonance imaging study. Spine. 1998;23(13):1452–6.CrossRefPubMed Saifuddin A, Burnett SJ, White J. The variation of position of the conus medullaris in an adult population. A magnetic resonance imaging study. Spine. 1998;23(13):1452–6.CrossRefPubMed
24.
go back to reference van Leeuwen R, Notermans NC, Vandertop WP. Surgery in adults with tethered cord syndrome: outcome study with independent clinical review. J Neurosurg. 2001;94(2 Suppl):205–9.PubMed van Leeuwen R, Notermans NC, Vandertop WP. Surgery in adults with tethered cord syndrome: outcome study with independent clinical review. J Neurosurg. 2001;94(2 Suppl):205–9.PubMed
25.
go back to reference Finn MA, Walker ML. Spinal lipomas: clinical spectrum, embryology, and treatment. Neurosurg Focus. 2007;23(2), E10.PubMed Finn MA, Walker ML. Spinal lipomas: clinical spectrum, embryology, and treatment. Neurosurg Focus. 2007;23(2), E10.PubMed
26.
go back to reference Pang D, Dias MS, Ahab-Barmada M. Split cord malformation: part I: a unified theory of embryogenesis for double spinal cord malformations. Neurosurgery. 1992;31(3):451–80.CrossRefPubMed Pang D, Dias MS, Ahab-Barmada M. Split cord malformation: part I: a unified theory of embryogenesis for double spinal cord malformations. Neurosurgery. 1992;31(3):451–80.CrossRefPubMed
27.
go back to reference Pang D. Ventral tethering in split cord malformation. Neurosurg Focus. 2001;10(1), e6.PubMed Pang D. Ventral tethering in split cord malformation. Neurosurg Focus. 2001;10(1), e6.PubMed
28.
go back to reference Proctor MR, Scott RM. Long-term outcome for patients with split cord malformation. Neurosurg Focus. 2001;10(1), e5.PubMed Proctor MR, Scott RM. Long-term outcome for patients with split cord malformation. Neurosurg Focus. 2001;10(1), e5.PubMed
29.
go back to reference Proctor MR, Bauer SB, Scott RM. The effect of surgery for split spinal cord malformation on neurologic and urologic function. Pediatr Neurosurg. 2000;32(1):13–9.CrossRefPubMed Proctor MR, Bauer SB, Scott RM. The effect of surgery for split spinal cord malformation on neurologic and urologic function. Pediatr Neurosurg. 2000;32(1):13–9.CrossRefPubMed
30.
go back to reference Hayden JA, Cote P, Bombardier C. Evaluation of the quality of prognosis studies in systematic reviews. Ann Intern Med. 2006;144(6):427–37.CrossRefPubMed Hayden JA, Cote P, Bombardier C. Evaluation of the quality of prognosis studies in systematic reviews. Ann Intern Med. 2006;144(6):427–37.CrossRefPubMed
31.
go back to reference Abrahamsson K, Olsson I, Sillen U. Urodynamic findings in children with myelomeningocele after untethering of the spinal cord. J Urol. 2007;177(1):331–4. discussion 334.CrossRefPubMed Abrahamsson K, Olsson I, Sillen U. Urodynamic findings in children with myelomeningocele after untethering of the spinal cord. J Urol. 2007;177(1):331–4. discussion 334.CrossRefPubMed
32.•
go back to reference Broderick KM, et al. Utility of urodynamics in the management of the asymptomatic tethered cord in children. World J Urol. 2015;33(8):1139–42. The authors performed a retrospective review of tethered cord syndrome after untethering in patients with abnormal MRI findings. Though the authors believed that surgery in asymptomatic patients was not indicated, this study actually supported the natural history of the disease as discussed by Wang et al. Broderick KM, et al. Utility of urodynamics in the management of the asymptomatic tethered cord in children. World J Urol. 2015;33(8):1139–42. The authors performed a retrospective review of tethered cord syndrome after untethering in patients with abnormal MRI findings. Though the authors believed that surgery in asymptomatic patients was not indicated, this study actually supported the natural history of the disease as discussed by Wang et al.
33.••
go back to reference Kearns J et al. Urodynamic studies in spinal cord tethering. Childs Nerv Syst. 2013;29(9):1589–600. Thorough description of the urodynamic evaluation and treatment of patients with tethered cord syndrome both pre- and post-tethered cord release.CrossRefPubMed Kearns J et al. Urodynamic studies in spinal cord tethering. Childs Nerv Syst. 2013;29(9):1589–600. Thorough description of the urodynamic evaluation and treatment of patients with tethered cord syndrome both pre- and post-tethered cord release.CrossRefPubMed
34.
35.
go back to reference Heuer GG, Adzick NS, Sutton LN. Fetal myelomeningocele closure: technical considerations. Fetal Diagn Ther. 2015;37(3):166–71.CrossRefPubMed Heuer GG, Adzick NS, Sutton LN. Fetal myelomeningocele closure: technical considerations. Fetal Diagn Ther. 2015;37(3):166–71.CrossRefPubMed
36.
go back to reference Phuong LK, Schoeberl KA, Raffel C. Natural history of tethered cord in patients with meningomyelocele. Neurosurgery. 2002;50(5):989–93. discussion 993.PubMed Phuong LK, Schoeberl KA, Raffel C. Natural history of tethered cord in patients with meningomyelocele. Neurosurgery. 2002;50(5):989–93. discussion 993.PubMed
37.
go back to reference Bowman RM et al. Tethered cord release: a long-term study in 114 patients. J Neurosurg Pediatr. 2009;3(3):181–7.CrossRefPubMed Bowman RM et al. Tethered cord release: a long-term study in 114 patients. J Neurosurg Pediatr. 2009;3(3):181–7.CrossRefPubMed
38.
go back to reference Brown E et al. Prevalence of incidental intraspinal lipoma of the lumbosacral spine as determined by MRI. Spine. 1994;19(7):833–6.CrossRefPubMed Brown E et al. Prevalence of incidental intraspinal lipoma of the lumbosacral spine as determined by MRI. Spine. 1994;19(7):833–6.CrossRefPubMed
39.
go back to reference Nogueira M et al. Tethered cord in children: a clinical classification with urodynamic correlation. J Urol. 2004;172(4 Pt 2):1677–80. discussion 1680.CrossRefPubMed Nogueira M et al. Tethered cord in children: a clinical classification with urodynamic correlation. J Urol. 2004;172(4 Pt 2):1677–80. discussion 1680.CrossRefPubMed
40.
go back to reference Guerra LA et al. Outcome in patients who underwent tethered cord release for occult spinal dysraphism. J Urol. 2006;176(4 Pt 2):1729–32.CrossRefPubMed Guerra LA et al. Outcome in patients who underwent tethered cord release for occult spinal dysraphism. J Urol. 2006;176(4 Pt 2):1729–32.CrossRefPubMed
41.
go back to reference Metcalfe PD et al. Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale. J Urol. 2006;176(4 Pt 2):1826–9. discussion 1830.CrossRefPubMed Metcalfe PD et al. Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale. J Urol. 2006;176(4 Pt 2):1826–9. discussion 1830.CrossRefPubMed
42.•
go back to reference Frainey BT et al. Predictors of urinary continence following tethered cord release in children with occult spinal dysraphism. J Pediatr Urol. 2014;10(4):627–33. This is a retrospective review of occult spinal dysraphism patients searching for predictors of post-operative continence based on pre-surgical parameters. The conclusion that pre-operative continence (lack of pre-operative symtpoms) is predictive of post-operative continence (good urological outcomes) supports the work of Wang et al.CrossRefPubMed Frainey BT et al. Predictors of urinary continence following tethered cord release in children with occult spinal dysraphism. J Pediatr Urol. 2014;10(4):627–33. This is a retrospective review of occult spinal dysraphism patients searching for predictors of post-operative continence based on pre-surgical parameters. The conclusion that pre-operative continence (lack of pre-operative symtpoms) is predictive of post-operative continence (good urological outcomes) supports the work of Wang et al.CrossRefPubMed
43.••
go back to reference Yener S et al. The effect of untethering on urologic symptoms and urodynamic parameters in children with primary tethered cord syndrome. Urology. 2015;85(1):221–6. Authors performed a prospective review comparing pre- and post-operative urinary symptoms and urodynamic parameters. Untethering improved both symptoms and urodynamics; no coordination was seen between these parameters. Furthermore they demonstrated that the most significant improvements were seen in patients without pre-operative symptoms.CrossRefPubMed Yener S et al. The effect of untethering on urologic symptoms and urodynamic parameters in children with primary tethered cord syndrome. Urology. 2015;85(1):221–6. Authors performed a prospective review comparing pre- and post-operative urinary symptoms and urodynamic parameters. Untethering improved both symptoms and urodynamics; no coordination was seen between these parameters. Furthermore they demonstrated that the most significant improvements were seen in patients without pre-operative symptoms.CrossRefPubMed
44.
go back to reference Khoury AE et al. Occult spinal dysraphism: clinical and urodynamic outcome after division of the filum terminale. J Urol. 1990;144(2 Pt 2):426–8. discussion 428–9, 443–4.PubMed Khoury AE et al. Occult spinal dysraphism: clinical and urodynamic outcome after division of the filum terminale. J Urol. 1990;144(2 Pt 2):426–8. discussion 428–9, 443–4.PubMed
45.
go back to reference Atala A et al. Bladder functional changes resulting from lipomyelomeningocele repair. J Urol. 1992;148(2 Pt 2):592–4.PubMed Atala A et al. Bladder functional changes resulting from lipomyelomeningocele repair. J Urol. 1992;148(2 Pt 2):592–4.PubMed
46.
go back to reference Wu HY et al. Long-term benefits of early neurosurgery for lipomyelomeningocele. J Urol. 1998;160(2):511–4.CrossRefPubMed Wu HY et al. Long-term benefits of early neurosurgery for lipomyelomeningocele. J Urol. 1998;160(2):511–4.CrossRefPubMed
47.
go back to reference Macejko AM et al. Clinical urological outcomes following primary tethered cord release in children younger than 3 years. J Urol. 2007;178(4 Pt 2):1738–42. discussion 1742–3.CrossRefPubMed Macejko AM et al. Clinical urological outcomes following primary tethered cord release in children younger than 3 years. J Urol. 2007;178(4 Pt 2):1738–42. discussion 1742–3.CrossRefPubMed
48.
go back to reference Kumar R et al. Evaluation of clinico-urodynamic outcome of bladder dysfunction after surgery in children with spinal dysraphism—a prospective study. Acta Neurochir (Wien). 2008;150(2):129–37.CrossRef Kumar R et al. Evaluation of clinico-urodynamic outcome of bladder dysfunction after surgery in children with spinal dysraphism—a prospective study. Acta Neurochir (Wien). 2008;150(2):129–37.CrossRef
49.•
go back to reference Kim SW et al. Six-month postoperative urodynamic score: a potential predictor of long-term bladder function after detethering surgery in patients with tethered cord syndrome. J Urol. 2014;192(1):221–7. Retrospective review of urodynamic data in occult spinal dysraphism patients both pre- and post-tethered cord release as reflected in a urodynamic score. They proposed that the urodynamic score at six months post-untethering can predict long-term urologic outcomes.CrossRefPubMed Kim SW et al. Six-month postoperative urodynamic score: a potential predictor of long-term bladder function after detethering surgery in patients with tethered cord syndrome. J Urol. 2014;192(1):221–7. Retrospective review of urodynamic data in occult spinal dysraphism patients both pre- and post-tethered cord release as reflected in a urodynamic score. They proposed that the urodynamic score at six months post-untethering can predict long-term urologic outcomes.CrossRefPubMed
50.
go back to reference Hoffman HJ et al. Management of lipomyelomeningoceles. Experience at the Hospital for Sick Children, Toronto. J Neurosurg. 1985;62(1):1–8.CrossRefPubMed Hoffman HJ et al. Management of lipomyelomeningoceles. Experience at the Hospital for Sick Children, Toronto. J Neurosurg. 1985;62(1):1–8.CrossRefPubMed
51.
go back to reference Kanev PM et al. Management and long-term follow-up review of children with lipomyelomeningocele, 1952–1987. J Neurosurg. 1990;73(1):48–52.CrossRefPubMed Kanev PM et al. Management and long-term follow-up review of children with lipomyelomeningocele, 1952–1987. J Neurosurg. 1990;73(1):48–52.CrossRefPubMed
52.
go back to reference Pierre-Kahn A et al. Intraspinal lipomas with spina bifida. Prognosis and treatment in 73 cases. J Neurosurg. 1986;65(6):756–61.CrossRefPubMed Pierre-Kahn A et al. Intraspinal lipomas with spina bifida. Prognosis and treatment in 73 cases. J Neurosurg. 1986;65(6):756–61.CrossRefPubMed
53.
go back to reference Arikan N et al. Role of magnetic resonance imaging in children with voiding dysfunction: retrospective analysis of 81 patients. Urology. 1999;54(1):157–60. discussion 160–1.CrossRefPubMed Arikan N et al. Role of magnetic resonance imaging in children with voiding dysfunction: retrospective analysis of 81 patients. Urology. 1999;54(1):157–60. discussion 160–1.CrossRefPubMed
54.
go back to reference Lavallee LT et al. Urodynamic testing—is it a useful tool in the management of children with cutaneous stigmata of occult spinal dysraphism? J Urol. 2013;189(2):678–83.CrossRefPubMed Lavallee LT et al. Urodynamic testing—is it a useful tool in the management of children with cutaneous stigmata of occult spinal dysraphism? J Urol. 2013;189(2):678–83.CrossRefPubMed
Metadata
Title
Systematic Review of Urologic Outcomes from Tethered Cord Release in Occult Spinal Dysraphism in Children
Authors
Jeffrey T. White
Derek C. Samples
Juan C. Prieto
Izabela Tarasiewicz
Publication date
01-11-2015
Publisher
Springer US
Published in
Current Urology Reports / Issue 11/2015
Print ISSN: 1527-2737
Electronic ISSN: 1534-6285
DOI
https://doi.org/10.1007/s11934-015-0550-6

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