Skip to main content

Advertisement

Log in

An atypical case of segmental spinal dysgenesis

  • Case Report
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Spinal segmental dysgenesis is a complex closed dysraphism. The diagnostic criteria are: lumbar or thoracolumbar vertebral dysgenesis causing kyphosis, focal spinal cord narrowing without exiting roots, deformity of the lower limbs and paraplegia or paraparesis. We present a newborn who showed atypical features of bifocal spinal cord narrowing, without any vertebral abnormality at the proximal level. This seems to be a variant of this rare entity, whose early diagnosis is important, as surgical stabilisation of the spine is required.

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
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Scott RM, Wolpert SM, Barthoshesky LE, et al (1988) Segmental spinal dysgenesis. Neurosurgery 22:739–744

    CAS  PubMed  Google Scholar 

  2. Desai K, Nadkarni T, Bhayani R, et al (2003) Congenital thoracic cord segmental amyelia: a rare manifestation of segmental spinal dysgenesis. Pediatr Neurosurg 38:102–106

    Article  PubMed  Google Scholar 

  3. Faciszewski T, Winter RB, Lonstein JE, et al (1995) Segmental spinal dysgenesis. A disorder different from spinal agenesis. J Bone Joint Surg Am 77:530–537

    CAS  PubMed  Google Scholar 

  4. Dias MS, Li V, Landi M, et al (1998) The embryogenesis of congenital vertebral dislocation: early embryonic buckling? Pediatr Neurosurg 29:281–289

    Article  CAS  PubMed  Google Scholar 

  5. Tortori-Donati P, Fondelli MP, Rossi A, et al (1999) Segmental spinal dysgenesis: neuroradiologic findings with clinical and embryologic correlation. AJNR 20:445–456

    CAS  PubMed  Google Scholar 

  6. van Straaten HW, Hekking JW (1991) Development of floor plate, neurons and axonal outgrowth pattern in the early spinal cord of the notochord-deficient chick embryo. Anat Embryol (Berl) 184:55–63

    Google Scholar 

  7. Rossi A, Cama A, Piatelli G, et al (2004) Spinal dysraphism: MR imaging rationale. J Neuroradiol 31:3–24

    CAS  PubMed  Google Scholar 

  8. Hughes LO, McCarthy RE, Glasier CM (1998) Segmental spinal dysgenesis: a report of three cases. J Pediatr Orthop 18:227–232

    Article  CAS  PubMed  Google Scholar 

  9. Flynn JM, Otsuka NY, Emans JB, et al (1997) Segmental spinal dysgenesis: early neurologic deterioration and treatment. J Pediatr Orthop 17:100–104

    Article  CAS  PubMed  Google Scholar 

  10. Van Allen MI, Kalousek DK, Chernoff GF (1993) Evidence of multisite closure of the neural tube in humans. Am J Med Genet 47:723–743

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Elodie Zana.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Zana, E., Chalard, F., Mazda, K. et al. An atypical case of segmental spinal dysgenesis. Pediatr Radiol 35, 914–917 (2005). https://doi.org/10.1007/s00247-005-1483-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-005-1483-x

Keywords

Navigation