CNS Developmental Anomalies

CNS Developmental Anomalies - CNS Developmental Anomalies...

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CNS Developmental Anomalies DR BARBARAWI
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classification Neural tube defect Aqueductal stenosis and hydrocephalus Chiari malformation Dandy walker malformation Craniosynostosis Arachnoid cyst Tethered cord syndrome Split cord malformation
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NTD 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
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Spinal: diastomatomyelia, syringomyelia . Neuronal migration disorders Agyria Pachygyria Hetrotopia schizencephaly
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Prenatal detection of NTD Recommended for pregnancies subsequent to NTD even if abortion is not considered it may allow for optimal post partum care Serum AFP Detailed morphology scan Amniocentesis for AFP
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Risk factors Folic acid deficiency Valproate Viral infection Exposure to heat Previous pregnancies with MM Positive family hx mainly from mom’s side Risk increase 1-2% with one previous anomaly 6-8% with 2 anomalies
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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
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Spina bifida cystica (operta ± 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
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Management Hydrocephalus develops in 65-85% with MM and 10% with meningocele Most patients have chiari type 2 Neurological assessment Watch for spontaneous movements Assess the lower functional level by checking response to painful stimulus Assess ruptured or unruptured Most of them are ruptured,
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Cover with saline soaked gauze Give IV antibiotics Surgical repair within 36-48 h to prevent infection and further neural damage only 1-3% may show neurological improvement
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continued Check HC Signs of Chiari Orthopaedic consultation Urology consultation Rehabilitation programme U/S vs CT Check for other abnormalities
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continued V-P Shunt when hydrocephalus established Decompressive sub occipital craniectomy for Chiari
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CNS Developmental Anomalies - CNS Developmental Anomalies...

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