congenital anomalies of nervous system
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Congenital Anomalies of Nervous SystemTRANSCRIPT
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Congenital Malformations of the Central Nervous System
Neural Tube DefectsDr. Dhaval ShuklaAssociate Professor
Department of NeurosurgeryNIMHANS, Bangalore.
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Epidemiology
• 1/3rd of all congenital malformations
• 75% of fetal deaths
• 40% of deaths during the first year of life
• Cause not known in 75%
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Epidemiology
• Most fetuses with major malformations are stillborn or die
during the neonatal period
• Survivors are seriously incapacitated, ranging from
unresponsiveness throughout their entire life to profound
mental retardation, muteness, severe motor deficits, and
seizures
• Minor anomalies manifest with mental retardation,
behavioral changes, motor disorders, and seizures.
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Etiology
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Development
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Major Developmental Events
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Major Developmental Events
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Normal and abnormal spinal cord
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Normal and abnormal spinal cord
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Neural Tube Defects
• 0.5 to 2 per 1,000 births
• Genetic risk factors, maternal diabetes mellitus,
and the use of the anticonvulsants valproic acid
and carbamazepine
• Brain or the spinal cord, or both, with their
coverings
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Classification
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Cutaneous manifestations
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Cutaneous manifestations
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Spina bifida occulta
• Sacral or lumbosacral is commonest
• Requires no treatment at birth
• Potential for the spinal cord to become fixed
(tethered) at the site of the lesion during
growth of child
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Meningocele
• Neurological function outcome is usually more
favorable
• Surgery to close the lesion
• Long-term needs will depend on the extent of
neurological deficits and level of involvement
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Myelomeningocele
• Apparent at birth
• Legs, bladder and bowel are usually affected
• Hydrocephalus is usually present
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Preoperative care
• Prevent infection– At the site of the lesion– Meningitis– Ventriculitis– Urinary infections
• Avoid drying and injury• Dressing– Clear Film– Non-abrasive– Non-adherent
Nurse proneMeticulous nappy care
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Surgery for open defects
• Within 24 hours of birth if no other life
threatening malformations
• Dissecting the neural tissue
• Covering the tissue with fibrous dura
• Skin graft may be necessary
• Shunt may be inserted
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Postoperative care
• Maintaining the airway and providing adequate
oxygenation
• Maintaining adequate circulatory fluid volumes and
replacing blood loss
• Ensuring hypoprothrombinaemia is minimized by
giving vitamin K preoperatively
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Postoperative care
• Ensuring the environment minimizes heat loss
• Monitoring and preventing metabolic
abnormalities such as hypoglycaemia,
hypocalcaemia or acidosis
• Maintaining strict precautions to minimize the
risk of infections
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Postoperative care
• Nurse the infant prone
• The wound must be observed for CSF leak and
signs of infection
• Nylon sutures are removed after 10–14 days
• Hydrocephalus is likely to develop within 10
days
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Neurological care
• Correct positioning of the limbs
• Observation of the skin for any signs of pressure
damage
• Regular position changes
• Regular passive exercisesPerform above with other routines such as
feeding and nappy care
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Bladder care
• Continuous urine leakage or full bladder after voiding
• Regular renal ultrasound scans
• Intermittent catheterization
• Prophylactic antibioticsExpressing the bladder by applying pressure over the lower abdomen during nappy changes may increase the risk of
urinary reflux into the ureters
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Care of parents
• Future predictions relating to the potential physical and cognitive outcomes
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Antenatal Detection
• Maternal ultrasound examination
• High α – fetoproteins in amniotic fluid– Maternal serum - 12 weeks
– 30 per cent chance of a false-positive
• Amniocentesis is offered at 16–18 weeks for chromosomal
aberrations by karyotyping
• DNA studies on chorionic villi - 8 weeks
• Magnetic resonance imaging (MRI)
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Prevention
Before and during pregnancy
• Folic acid (0.4mg daily)
• Increase to 5mg daily for high risk women
• Avoid smoking and alcohol intake
• Avoid aminopterin, methotrexate, trimethoprim,
valproic acid, carbamazepine, and phenobarbitone
If you are not taking contraceptives take FOLIC ACID