Spinal Dysraphism refers to a variety of

congenital anomalies resulting from failed

fusion of dorsal spinal elements.

Types Spina bifida occulta:

often incidental

cutaneous manifestations

Spina bifida aperta:

Meningocele

Myelomeningocele

Myelocystocele

lipomeningocele

MeningoceleMeningocele

is a midline mass usually occurring in the dorsal lumbar area composed of

CSF

meninges

and skin

Neural elements are absent

neurologic deficit rarely present

• 1-2/1000

1/3 have neurological deficits

Surgical repair with water-tight dural

closure

Meningomyelocele Myelomeningocele

is another defect, almost always associated with neurological deficit.

The herniated mass arises midline usually in the thoracolumbar region.

It contains

CSF

meninges

and neural tissue.

1-2/1000 live birth

Failure of complete closure of caudal neural tube

85% occur in lumbar region

Associated conditions include

Chiari malformation, club foot, hydrocephalus, and various cerebral and cerebellar malformations

Myelomeningocele is subclassified into spina

bifida cystica and aperta depending on whether

the contents of the mass communicate with the

environment.

Myelocystocele

refers to a terminal swelling of the neural axis probably secondary to hydromyelia in utero.

The expanding mass contains dilated cord as well as meninges

CSF

and fatty/fibrous tissue that herniates midline through

a spina bifida defect. This results in a tethered cord syndrome.

Extrophy of the bladder is a common associated defect.

Sacrococcygeal teratoma should be considered in the differential diagnosis.

Lipomeningocele refers to the fatty contents of a spina bifida lesion.

These may be intradural, extradural, or both.

Present with back mass, bladder problems, paralysis

Cutaneous stigmata

Symptoms are due to tethered cord and cord compression from fatty mass

Treatment is surgical decompression

Anterior Meningocele is an abnormal

communication between the spinal subarachnoid

space and a pelvic (or rarely thoracic) mass via a

channel in the anterior vertebral column

Radiological features Antenatal US

Plain film findings

structural vertebral anomalies such as hemivertebra,

butterfly vertebra, or incomplete fusion of posterior

elements; it does not allow imaging of the spinal cord.

CT ,MR

Antenatal ultrasonogram shows a lumbar meningocele.

Antenatal ultrasonogram shows a lemon sign and a banana sign.

Transverse cranial sonogram of a 20-week-old fetus with spina bifida. Image obtained at the level of the ventricles demonstrates the lemon like configuration of the fetal skull due to biconcavity (arrows) of the frontal bones.

Defects of laminae of lower cervical spine

Plain radiograph of same pt showing spina bi fida occulta of s1

Films of the spine demonstrate spina bifida involving L2 - S1.

Plain anteroposterior (AP) lumbar spinal radiograph in a 7-year-old patient shows a defect within the laminae of L4-5 and S1. Note the diastematomyelia.

Axial CT scans through the lumbosacral junction shows absence of the posterior spinal elements at L5-S1. Note sclerosis of the laminaeand the wide spinal canal.

When spinal malformations are suspected, investigation of the spinal canal and its contents are best performed by MRI

T2-weighted sagittal MRIs of the sacrum show an anterior sacral meningocele

Sagittal MRIs of the lumbar spine show diastematomyelia. Note the congenital fusion of L1 and L2

Myelograms in a 5-year-old patient show the dorsal region of the spine and an anterior thoracic meningocele. Note the gross dorsal kyphosis

Myelograms in a 4-year-old patient show the lumbosacral region; a long, tethered cord; and diastematomyelia.

Unfused arch of C1 at CT

T1w image,showing ventriculomegaly

Banan Sign: Abnormal shape of cerebelum from compression in spina bifida and other neural tube defects

Tethered Cord Syndrome

spine MR of T1-weighted (A) and T2-weighted images (B) shows tethered cornus medullaris to the level of sacrum and two isolated lipomyelomeningoceles, a transitional type from L3 to L5 (arrows), and a terminal type below S1 (arrow heads)

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