Spinal Dysraphism and Tethered Cord Syndrome

Dong Seok Kim

doi:10.5124/jkma.2009.52.1.78

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Kim: Spinal Dysraphism and Tethered Cord Syndrome

Continuing Education Column

Journal of the Korean Medical Association

Journal of the Korean Medical Association 2009; 52(1): 78-90.

Published online: 31 January 2009

DOI: https://doi.org/10.5124/jkma.2009.52.1.78

Spinal Dysraphism and Tethered Cord Syndrome

Dong Seok Kim, MD

Department of Neurosurgery, Yonsei University College of Medicine, Korea. dskim33@yuhs.ac

Abstract

Spinal dysraphism is a common birth defect that causes different kinds of secondary impairments, including joint deformities, reduced mobility, and bowel or bladder dysfunction. Various dysraphic spinal abnormalities result in tethered cord syndrome, a progressive form of neurological deterioration that results from spinal cord tethering. The surgery and management of children who have spinal dysraphism require multidisciplinary care and long-term follow-up by multiple specialists in birth defects. This article reviews the clinical presentation, pathophysiology, diagnostic strategies, and therapeutic management of spinal dysraphism in infancy.

Keywords: Spinal dysraphism, Neural tube defect, Spina bifida

Figures and Tables

Figure 1

Diagram of spina bifida and MR axial view. (A, E): normal, (B): spina bifida occulta, (C, F): spina bifida cytica-meningocele, (D, G): spina bifida cytica-myelomeningocele.

Figure 2

Spina bifida, open.

(A) Myeloschisis, (B) Spina bifida cystica-meningocle, (C) Spina bifida cystica-myelomeningocle.

Figure 3

Various dermatologic feature in closed spina bifida.

(A) Lipoma, (B) Skin depression, (C) Capillary telangiectasis.

Figure 4

Location of conus medullaris.

(A) Postovulation 8 wks, (B) Postovulation 24 wks, (C) Birth, (D) Normal adult.

Figure 5

(A) Dorsal lipomyelomeningocele, (B) Transitional lipomyelomeningocele.

Figure 6

Caudal lipomyelomeingocele Preop (left) and postop (right).

Figure 7

Fetal sonography (left) and fetal MRI (right) showing mylelomeningocele at post gestation 22wks.

Figure 8

Spina bifida cystica-myelomeningocele.

Figure 9

(A) Initial incision follows the circumferential white line formed by the junction of the arachnoid and skin, (B) Cross section of A.

Table 1

Congenital neurologic malformation depending on postovulatory age

^*Neurosurg Clin North Am, 1995: p184

Table 2

Congenital spinal malformation due to abnormal early neural development

Table 3

Criteria for shunt

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