CNS Developmental Anomalies

Posted by e-Medical PPT Friday, September 14, 2012
Classification
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

Dandy walker malformation
Atresia of foramina of magendie and luschka results in agenesis of the cerebellar vermis with lage post fossa cyst communicating with enlarged 4th vent
Associated with hydrocephalus in 90%
Symptoms and signs related to post fossa syndrome
Shunting the cyst alone may solve the problem

craniosynostosis
Premature fusion of suture causes failure of  normal skull growth
0.6%/1000 live birth
It is developmental but may occur after shunts or brain atrophy
Lazy lambdoid due to positional flattening
Sagittal is the most common causing scaphocephaly
Coronal in females, unilateral plagicephaly or bilateral brachycephaly usually with syndromes
Metopic causes trigoncephaly
Multiple fusion oxycephaly (tower)
Treatment is surgery by suturectomy only or using advanced techniques

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

Tethered cord
Abnormally low located conus medullaris below L2 associated with thick and short filum terminale or lipoma
Common with MM
Causes progressive weakness scoliosis or foot deformity, increasing spasticity, worsening gait, worsening back pain.
Surgical treatment is untethering
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