Spinal Dysraphism

spinal dysraphism: This term encompasses all defects (open or closed) associated with a failure of closure of the posterior neural arch.

Embryology

Neural fold

Ectoderm

Neural fold

Ectoderm

Mesoderm - develops into the vertebral

3 weeks

Neural tube

weeks column

Mesoderm - develops into the vertebral

3 weeks

Developmental errors may occur early in fetal life and lead to a variety of spinal defects:

Roots

myelomeningocele The spinal cord and roots protrude through the bony defect and lie within a cystic cavity, lined with meninges and/or skin. In most patients, the meningeal covering ruptures and the spinal cord and roots lie exposed to the air - myelodysplasia. CSF may leak from the open lesion.

meningocele Cystic CSF filled cavity -lined with meninges but devoid of neural tissue. The cavity communicates with the spinal canal through the bone defect (usually lumbosacral). Meningoceles occur far less frequently than myelomeningocele; they are rarely associated with other congenital anomalies.

Site: 80% occur in the lumbosacral region. Incidence: 2/1000 births in Britain, but there is a geographical variation (0.2/1000 in Japan). A familial incidence increases the risk (5% if a sibling is affected). This suggests a genetic factor, but teratogens, e.g. sodium valproate, also have a role.

Cutaneous defect may overlie spina bifida

Cutaneous defect may overlie spina bifida

SPINA BIFIDA OCCULTA

A bony deficit - present in 5-10% of the population and not clinically significant. Those who also have a lumbosacral cutaneous abnormality however {tuft of hair, dimple, sinus or 'port wine' stain) have a high incidence of related underlying defects:

- diastomatomyelia

- lipoma

- dermoid cyst.

These defects may cause symptoms of pain or neurological impairment after many years.

410 Associated abnormalities: Hydrocephalus, Chiari type II, aqueduct forking.

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