Skip to main content


Log in

Cervical split cord malformation (diastematomyelia) with associated Klippel–Feil deformity presenting in adulthood with bimanual synkinesis

  • Case Report
  • Published:
European Spine Journal Aims and scope Submit manuscript



Split cord malformation (SCM) is a rare congenital malformation of the spinal cord in which the cord is split longitudinally. Identification and diagnosis in adulthood is rare, with the majority of cases diagnosed in the paediatric population. Isolated segmental cervical SCM is rarer still.

Case presentation

Here, the authors present the case of a 26-year-old female who presented with neck pain and longstanding bimanual synkinesis secondary to an isolated type II SCM in the C4–C7 region. The authors present this novel presenting symptom in adulthood and finding of isolated cervical SCM with associated blocked cervical vertebrae, in an otherwise normal neuroaxis and spinal column.


The case serves to highlight the importance of knowledge of this rare congenital condition to surgeons, physicians and radiologists involved in the care of both adult and paediatric patients presenting with spinal pathology.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others


  1. Hamidi H, Foladi N (2019) Misdiagnosed adult presentation of diastematomyelia and tethered cord. Radiol case reports 14:1123–1126.

    Article  Google Scholar 

  2. Bekki H, Morishita Y, Kawano O et al (2015) Diastematomyelia: a surgical case with long-term follow-up. Asian Spine J 9:99–102.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Srinivasan ES, Mehta VA, Smith GC et al (2020) Klippel–Feil syndrome with cervical diastematomyelia in an adult with extensive cervicothoracic fusions: case report and review of the literature. World Neurosurg 139:274–280.

    Article  PubMed  Google Scholar 

  4. Adelson PD (2014) Diastematomyelia. In: Encyclopedia of the neurological sciences. Academic Press, pp 996–997

  5. Rossi A, Biancheri R, Cama A et al (2004) Imaging in spine and spinal cord malformations. Eur J Radiol 50:177–200.

    Article  PubMed  Google Scholar 

  6. Cheng B, Li FT, Lin L (2012) Diastematomyelia. J Bone Joint Surg Br 94-B:365–372.

    Article  Google Scholar 

  7. Dickerman RD, Colle KO, Mittler MA (2004) Intramedullary inflammatory mass dorsal to the Klippel–Feil deformity: error in development or response to an abnormal motion segment? Spinal Cord 42:720–722.

    Article  CAS  PubMed  Google Scholar 

  8. David KM, Copp AJ, Stevens JM et al (1996) Split cervical spinal cord with Klippel–Feil syndrome: seven cases. Brain 119:1859–1872.

    Article  PubMed  Google Scholar 

  9. Hudson EW, Makis W (2016) Klippel–Feil syndrome with spinal dysraphism: Diastematomyelia on 18F-NaF Bone PET, CT, and MRI imaging. Clin Nucl Med 41:405–406.

    Article  PubMed  Google Scholar 

  10. Lopez-Vicchi M, Da Prat G, Gatto EM (2015) Pseudodystonic posture secondary to Klippel–Feil syndrome and Diastematomyelia. Tremor and Other Hyperkinetic Movements 5:325.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Bae Y (2014) Effects of cervical deep muscle strengthening in a neck pain: a patient with Klippel–Feil syndrome. J Phys Ther Sci 26:1999–2001.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Mahapatra AK (2011) Split cord malformation—a study of 300 cases at AIIMS 1990–2006. J Pediatr Neurosci 6:S41–S45.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Korinth MC, Kapser A, Nolte K, Gilsbach JM (2004) Cervical diastematomyelia associated with an intradural epidermoid cyst between the hemicords and multiple vertebral body anomalies. Pediatr Neurosurg 40:253–256.

    Article  PubMed  Google Scholar 

  14. Balci S, Oguz KK, Firat MM, Boduroglu K (2002) Cervical diastematomyelia in cervico-oculo-acoustic (Wildervanck) syndrome: MRI findings. Clin Dysmorphol 11:125–128.

    Article  PubMed  Google Scholar 

Download references


The authors would like to acknowledge the patient for providing fully informed consent for this case report.

Author information

Authors and Affiliations


Corresponding author

Correspondence to Edward Goacher.

Ethics declarations

Conflict of interest

The authors declare no conflicts of interest. No funding was received for this article.

Additional information

Publisher's Note

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

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Goacher, E., Lee, C. Cervical split cord malformation (diastematomyelia) with associated Klippel–Feil deformity presenting in adulthood with bimanual synkinesis. Eur Spine J (2024).

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • DOI: