Skip to main content

Advertisement

SpringerLink
Log in

Occult tethered cord syndrome: a rare, treatable condition

  • Original Article
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

Occult tethered cord syndrome (OTCS) is an entity that shows tethered cord syndrome (TCS) with normal spinal MRI findings. The definition and treatment of OTCS have been controversial since first proposal. The purpose of this study was to evaluate the existence, prevalence, histological characteristics, and surgical outcomes of OTCS.

Methods

We retrospectively analyzed patients who underwent untethering surgery for OTCS from January 2010 to December 2019. Inclusion criteria were (1) clinical manifestation of TCS; (2) supported by urodynamic study (UDS) or electromyography/nerve conduction study; (3) no structural lesions in the urological tract or spinal cord, and no developmental delay; and (4) postoperative follow-up for > 6 months. Sectioned fila from OTCS patients were histologically compared with those from cases of thickened filum or low-lying conus.

Results

Five (four female, one male) of 439 patients (1.1%) who underwent untethering surgeries for occult spinal dysraphism corresponded to OTCS. Mean age at the time of surgery was 16 years (7–22 years). Mean postoperative follow-up duration was 45 months (15–114 months). The main symptom was urinary dysfunction in four patients and leg pain in one. All patients had detrusor-sphincter dyssynergia. Fila from OTCS patients revealed increased fibrous tissue as in TCS patients. Four patients showed postoperative improvement and one with preoperative static course had no improvement.

Conclusions

This study suggests that OTCS is a definitely existing entity although rare. OTCS is curable when timely treatment is given. Sudden onset with rapid progression of symptom seems the best indication for surgery.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

Availability of data and materials

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

References

  1. Albright AL (2005) Occult tight filum terminale syndrome: results of surgical untethering. Pediatr Neurosurg 41:58. https://doi.org/10.1159/000084869

    Article  PubMed  Google Scholar 

  2. Austin PF, Bauer SB, Bower W, Chase J, Franco I, Hoebeke P, Rittig S, Walle JV, von Gontard A, Wright A, Yang SS, Nevéus T (2016) The standardization of terminology of lower urinary tract function in children and adolescents: update report from the standardization committee of the International Children’s Continence Society. Neurourol Urodyn 35:471–481. https://doi.org/10.1002/nau.22751

    Article  PubMed  Google Scholar 

  3. Bao N, Chen ZH, Gu S, Chen QM, Jin HM, Shi CR (2007) Tight filum terminale syndrome in children: analysis based on positioning of the conus and absence or presence of lumbosacral lipoma. Childs Nerv Syst 23:1129–1134. https://doi.org/10.1007/s00381-007-0376-8

    Article  PubMed  Google Scholar 

  4. Brown E, Matthes JC, Bazan CI, Jinkins JR (1994) Prevalence of incidental intraspinal lipoma of the lumbosacral spine as determined by MRI. Spine 19:833–836. https://doi.org/10.1097/00007632-199404000-00018

    Article  CAS  PubMed  Google Scholar 

  5. Bulsara KR, Zomorodi AR, Enterline DS, George TM (2004) The value of magnetic resonance imaging in the evaluation of fatty filum terminale. Neurosurgery 54:375–380. https://doi.org/10.1227/01.NEU.0000103451.63301.0B

    Article  PubMed  Google Scholar 

  6. Cools MJ, Al-Holou WN, Stetler WR, Wilson TJ, Muraszko KM, Ibrahim M, Marca FL, Garton HJL, Maher CO (2014) Filum terminale lipomas: imaging prevalence, natural history, and conus position. J Neurosurg Pediatr 13:559–567. https://doi.org/10.3171/2014.2.peds13528

    Article  PubMed  Google Scholar 

  7. Hendson G, Dunham C, Steinbok P (2016) Histopathology of the filum terminale in children with and without tethered cord syndrome with attention to the elastic tissue within the filum. Childs Nerv Syst 32:1683–1692. https://doi.org/10.1007/s00381-016-3123-1

    Article  PubMed  Google Scholar 

  8. Lee JY, Kim KH, Park K, Wang K-C (2020) Retethering: a neurosurgical viewpoint. J Korean Neurosurg Soc 63:346–357. https://doi.org/10.3340/jkns.2020.0039

    Article  PubMed  PubMed Central  Google Scholar 

  9. Loening-Baucke V (1997) Urinary incontinence and urinary tract infection and their resolution with treatment of chronic constipation of childhood. Pediatrics 100:228–232. https://doi.org/10.1542/peds.100.2.228

    Article  CAS  PubMed  Google Scholar 

  10. Ogiwara H, Lyszczarz A, Alden TD, Bowman RM, McLone DG, Tomita T (2011) Retethering of transected fatty filum terminales. J Neurosurg Pediatr 7:42–46. https://doi.org/10.3171/2010.10.peds09550

    Article  PubMed  Google Scholar 

  11. Schultz-Lampel D, Steuber C, Hoyer PF, Bachmann CJ, Marschall-Kehrel D, Bachmann H (2011) Urinary incontinence in children. Dtsch Arztebl Int 108:613–620. https://doi.org/10.3238/arztebl.2011.0613

    Article  PubMed  PubMed Central  Google Scholar 

  12. Selçuki M, Coşkun K (1998) Management of tight filum terminale syndrome: with special emphasis on normal level conus medullaris (NLCM). Surg Neurol 50:318–322. https://doi.org/10.1016/S0090-3019(97)00377-7

    Article  PubMed  Google Scholar 

  13. Selcuki M, Ünlü A, Uğur HÇ, Soygür T, Arıkan N, Selcuki D (2000) Patients with urinary incontinence often benefit from surgical detethering of tight filum terminale. Childs Nerv Syst 16:150–154. https://doi.org/10.1007/s003810050482

    Article  CAS  PubMed  Google Scholar 

  14. Selden NR (2006) Occult tethered cord syndrome: the case for surgery. J Neurosurg Pediatr 104:302–304. https://doi.org/10.3171/ped.2006.104.5.302

    Article  Google Scholar 

  15. Selden NR (2007) Minimal tethered cord syndrome: what’s necessary to justify a new surgical indication? Neurosurg Focus 23:1–5. https://doi.org/10.3171/FOC-07/08/E1

    Article  Google Scholar 

  16. Selden NR, Nixon RR, Skoog SR, Lashley DB (2006) Minimal tethered cord syndrome associated with thickening of the terminal filum. J Neurosurg Pediatr 105:214–218. https://doi.org/10.3171/ped.2006.105.3.214

    Article  Google Scholar 

  17. Steinbok P, Garton HJ, Gupta N (2006) Occult tethered cord syndrome: a survey of practice patterns. J Neurosurg Pediatr 104:309–313. https://doi.org/10.3171/ped.2006.104.5.309

    Article  Google Scholar 

  18. Steinbok P, Kariyattil R, MacNeily AE (2007) Comparison of section of filum terminale and non-neurosurgical management for urinary incontinence in patients with normal conus position and possible occult tethered cord syndrome. Neurosurgery 61:550–556. https://doi.org/10.1227/01.neu.0000290902.07943.e6

    Article  PubMed  Google Scholar 

  19. Steinbok P, MacNeily AE, Hengel AR, Afshar K, Landgraf JM, Hader W, Pugh J (2016) Filum section for urinary incontinence in children with occult tethered cord syndrome: a randomized, controlled pilot study. J Urol 195:1183–1188. https://doi.org/10.1016/j.juro.2015.09.082

    Article  PubMed  Google Scholar 

  20. Tehli O, Hodaj I, Kural C, Solmaz I, Onguru O, Izci Y (2011) A comparative study of histopathological analysis of filum terminale in patients with tethered cord syndrome and in normal human fetuses. Pediatr Neurosurg 47:412–416. https://doi.org/10.1159/000338981

    Article  PubMed  Google Scholar 

  21. Thompson EM, Strong MJ, Warren G, Woltjer RL, Selden NR (2014) Clinical significance of imaging and histological characteristics of filum terminale in tethered cord syndrome. J Neurosurg Pediatr 13:255–259. https://doi.org/10.3171/2013.12.peds13370

    Article  PubMed  Google Scholar 

  22. Tu A, Steinbok P (2013) Occult tethered cord syndrome: a review. Childs Nerv Syst 29:1635–1640. https://doi.org/10.1007/s00381-013-2129-1

    Article  PubMed  Google Scholar 

  23. Tuite GF, Thompson DN, Austin PF, Bauer SB (2018) Evaluation and management of tethered cord syndrome in occult spinal dysraphism: recommendations from the International Children’s Continence Society. Neurourol Urodyn 37:890–903. https://doi.org/10.1002/nau.23382

    Article  PubMed  Google Scholar 

  24. Warder DE, Oakes WJ (1993) Tethered cord syndrome and the conus in a normal position. Neurosurgery 33:374–378. https://doi.org/10.1227/00006123-199309000-00004

    Article  CAS  PubMed  Google Scholar 

  25. Wehby MC, O’Hollaren PS, Abtin K, Hume JL, Richards BJ (2004) Occult tight filum terminale syndrome: results of surgical untethering. Pediatr Neurosurg 40:51–57. https://doi.org/10.1159/000078908

  26. Wilson DA, Prince JR (1989) John Caffey award. MR imaging determination of the location of the normal conus medullaris throughout childhood. Am J Roentgenol 152:1029–1032. https://doi.org/10.2214/ajr.152.5.1029

    Article  CAS  Google Scholar 

  27. Yamada S, Won DJ, Pezeshkpour G, Yamada BS, Yamada SM, Siddiqi J, Zouros A, Colohan AR (2007) Pathophysiology of tethered cord syndrome and similar complex disorders. Neurosurg Focus 23:1–10. https://doi.org/10.3171/FOC-07/08/E6

    Article  Google Scholar 

  28. Yundt KD, Park TS, Kaufman BA (1997) Normal diameter of filum terminate in children: in vivo measurement. Pediatr Neurosurg 27:257–259. https://doi.org/10.1159/000121263

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Center for Rare Cancers, Neuro-Oncology Clinic, National Cancer Center, 323 Ilsan-ro, Ilsandong-gu, Goyang, Gyeonggi-do, 10408, Korea

    Jeyul Yang & Kyu-Chang Wang

  2. Department of Pathology, Seoul National University Hospital and Seoul National University College of Medicine, Seoul, Korea

    Jae-Kyung Won

  3. Division of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul, Korea

    Kyung Hyun Kim, Ji Yeoun Lee & Seung-Ki Kim

  4. Department of Anatomy, Seoul National University College of Medicine, Seoul, Korea

    Ji Yeoun Lee

  5. Department of Rehabilitation Medicine, Seoul National University Hospital and Seoul National University College of Medicine, Seoul, Korea

    Hyung-Ik Shin

  6. Division of Pediatric Urology, Seoul National University Children’s Hospital, 101 Daehak-no, Jongno-gu, Seoul, 03080, Korea

    Kwanjin Park

Authors
  1. Jeyul Yang
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jae-Kyung Won
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kyung Hyun Kim
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ji Yeoun Lee
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Seung-Ki Kim
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Hyung-Ik Shin
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Kwanjin Park
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Kyu-Chang Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by Jeyul Yang, Jae-Kyung Won, Kwanjin Park, and Kyu-Chang Wang. The first draft of the manuscript was written by Jeyul Yang, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding authors

Correspondence to Kwanjin Park or Kyu-Chang Wang.

Ethics declarations

Ethics approval

The present study was approved by the institutional review board of the Seoul National University Hospital (IRB no. 2004–104-1118). Informed consent was waived by the IRB since this study was retrospective.

Conflict of interest

The authors declare no conflicts of interest.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Yang, J., Won, JK., Kim, K.H. et al. Occult tethered cord syndrome: a rare, treatable condition. Childs Nerv Syst 38, 387–395 (2022). https://doi.org/10.1007/s00381-021-05353-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-021-05353-y

Keywords

Search

Navigation