J Korean Med Assoc.  2009 Jan;52(1):78-90. 10.5124/jkma.2009.52.1.78.

Spinal Dysraphism and Tethered Cord Syndrome

• Kim DS

• KMID: 2137736
• DOI: http://doi.org/10.5124/jkma.2009.52.1.78

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.

Keyword

Spinal dysraphism; Neural tube defect; Spina bifida

MeSH Terms

Child
Congenital Abnormalities
Humans
Joints
Neural Tube Defects
Specialization
Spinal Cord
Spinal Dysraphism
Urinary Bladder

Figure

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  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.

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  Figure 2 Spina bifida, open. (A) Myeloschisis, (B) Spina bifida cystica-meningocle, (C) Spina bifida cystica-myelomeningocle.

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  Figure 3 Various dermatologic feature in closed spina bifida. (A) Lipoma, (B) Skin depression, (C) Capillary telangiectasis.

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  Figure 4 Location of conus medullaris. (A) Postovulation 8 wks, (B) Postovulation 24 wks, (C) Birth, (D) Normal adult.

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  Figure 5 (A) Dorsal lipomyelomeningocele, (B) Transitional lipomyelomeningocele.

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  Figure 6 Caudal lipomyelomeingocele Preop (left) and postop (right).

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  Figure 7 Fetal sonography (left) and fetal MRI (right) showing mylelomeningocele at post gestation 22wks.

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  Figure 8 Spina bifida cystica-myelomeningocele.

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  Figure 9 (A) Initial incision follows the circumferential white line formed by the junction of the arachnoid and skin, (B) Cross section of A.

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