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Split Cord Malformations

  • Brandon G. RocqueEmail author
Chapter

Abstract

Split cord malformations (SCM) are a form of spinal dysraphism in which the spinal cord itself is split into two hemicords. In type 1 SCM, the two hemicords are contained within two separate dural sleeves, often separated by a bony or cartilaginous septum. In type 2 SCM, both hemicords are contained within the same dural sleeve. This chapter discusses the anatomy and classification of SCM, including a discussion of the cutaneous abnormalities associated with SCM and the other dysraphisms that may accompany SCM. In addition, there is a review of the theory of embryologic insult that leads to SCM formation. Finally, the chapter discusses the natural history, details of surgical treatment, and outcomes from surgical treatment. The natural history of SCM is thought to be unfavorable, with progressive neurological deficits. Surgical treatment strategies should focus on complete release of all tethering elements.

Keywords

Split cord malformation Diastematomyelia Diplomyelia Hypertrichosis Tethered cord Accessory neurenteric canal 

Supplementary material

Video 12.1

This video shows the surgical treatment of a type 1 SCM. (From AANS Neurosurgeon [25]; with permission) (MP4 239633 kb)

References

  1. 1.
    Recklinghausen von F. The classic: studies on spina bifida. 1886. Clin Orthopaedics Relat Res. 2011;469:1227–9.Google Scholar
  2. 2.
    Saker E, Loukas M, Fisahn C, Oskouian RJ, Tubbs RS. Historical perspective of split cord malformations: a tale of two cords. Pediatr Neurosurg. 2016;52(1):1–5.PubMedGoogle Scholar
  3. 3.
    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.PubMedGoogle Scholar
  4. 4.
    Dias MS, Pang D. Split cord malformations. Neurosurg Clin N Am. 1995;6(2):339–58.PubMedGoogle Scholar
  5. 5.
    İzci Y, Gonul M, Gonul E. The diagnostic value of skin lesions in split cord malformations. J Clin Neurosci. 2007;14(9):860–3.PubMedGoogle Scholar
  6. 6.
    Mahapatra AK. Split cord malformation – a study of 300 cases at AIIMS 1990–2006. J Pediatr Neurosci. 2011;6(3):41.Google Scholar
  7. 7.
    Ozturk E, Sonmez G, Mutlu H, Sildiroglu HO, Velioglu M, Basekim CC, et al. Split-cord malformation and accompanying anomalies. J Neuroradiol. 2008;35(3):150–6.PubMedGoogle Scholar
  8. 8.
    Yamada S, Mandybur GT, Thompson JR. Dorsal midline proboscis associated with diastematomyelia and tethered cord syndrome. Case report. J Neurosurg. 1996;85(4):709–12.PubMedGoogle Scholar
  9. 9.
    Jankowitz BT, Albright AL. Cutaneous manifestations of split cord malformations. J Neurosurg Pediatr. 2007;107(3):240–3.Google Scholar
  10. 10.
    Starnoni D, Duff JM, Viswanathan GC. Duplicated filum terminale in non-split cord malformations: an underrecognized cause for treatment failure in tethered cord syndrome. J Spinal Cord Med. 2016;2(3):1–4.Google Scholar
  11. 11.
    Singh N, Singh D, Kumar R. Diastematomyelia with hemimyelomeningocele: an exceptional and complex spinal dysraphism. J Pediatr Neurosci. 2015;10(3):237.PubMedPubMedCentralGoogle Scholar
  12. 12.
    Higashida T, Sasano M, Sato H, Sekido K, Ito S. Myelomeningocele associated with split cord malformation type I -three case reports. Neurol Med Chir (Tokyo). 2010;50(5):426–30.Google Scholar
  13. 13.
    Iskandar BJ, McLaughlin C, Oakes WJ. Split cord malformations in myelomeningocele patients. Br J Neurosurg. 2000;14(3):200–3.PubMedGoogle Scholar
  14. 14.
    Babu R, Reynolds R, Moreno JR, Cummings TJ, Bagley CA. Concurrent split cord malformation and teratoma: dysembryology, presentation, and treatment. J Clin Neurosci. 2014;21(2):212–6.PubMedGoogle Scholar
  15. 15.
    Pang D. Split cord malformation: part II: clinical syndrome. Neurosurgery. 1992;31(3):481–500.PubMedGoogle Scholar
  16. 16.
    Shen J, Zhang J, Feng F, Wang Y, Qiu G, Li Z. Corrective surgery for congenital scoliosis associated with split cord malformation. J Bone Joint Surg Am. 2016;98(11):926–36.PubMedGoogle Scholar
  17. 17.
    Emura T, Asashima M, Furue M, Hashizume K. Experimental split cord malformations. Pediatr Neurosurg. . Karger Publishers. 2002;36(5):229–35.PubMedGoogle Scholar
  18. 18.
    Erşahin Y. Split cord malformation types I and II: a personal series of 131 patients. Childs Nerv Syst. 2013;29(9):1515–26.PubMedGoogle Scholar
  19. 19.
    Barutcuoglu M, Selcuki M, Selcuki D, Umur S, Mete M, Gurgen SG, et al. Cutting filum terminale is very important in split cord malformation cases to achieve total release. Childs Nerv Syst. 2014;31(3):425–32.PubMedGoogle Scholar
  20. 20.
    Pang D. Ventral tethering in split cord malformation. Neurosurg Focus. 2001;10(1):e6.PubMedGoogle Scholar
  21. 21.
    Börcek AÖ, Öcal Ö, Emmez H, Zinnuroglu M, Baykaner MK. Split cord malformation: experience from a tertiary referral center. Pediatr Neurosurg. 2012;48(5):291–8.PubMedGoogle Scholar
  22. 22.
    Proctor MR, Scott RM. Long-term outcome for patients with split cord malformation. Neurosurg Focus. 2001;10(1):1–5.Google Scholar
  23. 23.
    Schijman E. Split spinal cord malformations: report of 22 cases and review of the literature. Childs Nerv Syst. 2003;19(2):96–103.PubMedGoogle Scholar
  24. 24.
    Iskandar BJ, Fulmer BB, Hadley MN, Oakes WJ. Congenital tethered spinal cord syndrome in adults. Neurosurg Focus. 2001;10(1):e7.PubMedGoogle Scholar
  25. 25.
    The untethering of a type 1 split cord malformation. AANS Neurosurgeon Neurosurg Subspecialization. 2016;25(4). In the Loupe. http://aansneurosurgeon.org/multimedia/?mm_id=5109#nav-primary.

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Pediatric NeurosurgeryChildren’s of AlabamaBirminghamUSA

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