spinal dysraphism
TRANSCRIPT
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Spinal Dysraphism refers to a variety of
congenital anomalies resulting from failed
fusion of dorsal spinal elements.
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Types Spina bifida occulta:
often incidental
cutaneous manifestations
Spina bifida aperta:
Meningocele
Myelomeningocele
Myelocystocele
lipomeningocele
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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
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• 1-2/1000
1/3 have neurological deficits
Surgical repair with water-tight dural
closure
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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.
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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
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Myelomeningocele is subclassified into spina
bifida cystica and aperta depending on whether
the contents of the mass communicate with the
environment.
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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.
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Extrophy of the bladder is a common associated defect.
Sacrococcygeal teratoma should be considered in the differential diagnosis.
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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
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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
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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
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Antenatal ultrasonogram shows a lumbar meningocele.
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Antenatal ultrasonogram shows a lemon sign and a banana sign.
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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.
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Defects of laminae of lower cervical spine
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Plain radiograph of same pt showing spina bi fida occulta of s1
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Films of the spine demonstrate spina bifida involving L2 - S1.
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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.
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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.
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When spinal malformations are suspected, investigation of the spinal canal and its contents are best performed by MRI
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T2-weighted sagittal MRIs of the sacrum show an anterior sacral meningocele
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Sagittal MRIs of the lumbar spine show diastematomyelia. Note the congenital fusion of L1 and L2
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Myelograms in a 5-year-old patient show the dorsal region of the spine and an anterior thoracic meningocele. Note the gross dorsal kyphosis
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Myelograms in a 4-year-old patient show the lumbosacral region; a long, tethered cord; and diastematomyelia.
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Unfused arch of C1 at CT
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T1w image,showing ventriculomegaly
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Banan Sign: Abnormal shape of cerebelum from compression in spina bifida and other neural tube defects
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Tethered Cord Syndrome
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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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Thank you