CNS Developmental Anomalies

Posted by e-Medical PPT
Classifications
Neural tube defect
Aqueductal stenosis and hydrocephalus
Chiari malformation
Dandy walker malformation
Craniosynostosis
Arachnoid cyst
Tethered cord syndrome
Split cord malformation

Neural tube defect
1- Neurulation: results in open lesions.
Anterior neuropore: craniorachischisis, anencephaly.
Posterior neuropore: meningomyelocele, meningocele
2- post neurulation: results in skin covered lesions.
Cranial :
   microcephaly, hydrencephaly , holoprocencephaly, lessencephaly, porencephaly, agenesis of corpus callosum

Spinal:
 diastomatomyelia, syringomyelia

Neuronal migration disorders
Agyria
Pachygyria
Hetrotopia
schizencephaly

Spinal dysraphysim
Spina bifida occulta
Congenital absence of spinous process and lamina, no visible meninges or neural tissue
Often incidental finding, no important when it occurs alone
May be as apart of deep seated lesions

Spina bifida cystica
Meningocele :
congenital defect in vertebral arches with cystic distension of meninges, no abnormality of neural tissue

Meningomyelocele:
as above but with structural or functional abnormality of spinal cord or cauda equina
1-2/1000 live birth

Lipo myeloschisis
A subcutaneous lipoma passes via a mid line defect in the lumbodorsal fascia , vertebral neural arch and dura with tethered cord
Is also called cauda equina lipoma
Spinal cord may be split myeloschisis
Dura is dehiscent
May be associated with dermal sinus which may end with epidermoid or dermoid cyst
Causing progressive neurological, urological and orthopaedic dysfunction via tethering of cord or compression
It presents with back mass, skin stigmata, bladder dysfunction, foot deformities or weakness
MRI tool of choice
Surgical treatment is aiming to untethering the cord and debulking the fatty tumour....

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