Hatem I. BadrHatem I. Badr MD.MD.
ASS. Professor NeurosurgeryASS. Professor NeurosurgeryDepartment of NeurosurgeryDepartment of Neurosurgery
Mansoura University, EgyptMansoura University, Egypt
CNS congenital anomaliesCNS congenital anomaliesCNS congenital anomaliesCNS congenital anomalies
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Neural Tube Neural Tube DevelopmentDevelopment
Normal embryological development Neural plate
development -16th dayCranial closure 24th
day (upper spine)Caudal closure 28th
day (lower spine)
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Spina Bifida OccultaSpina Bifida Occulta
Very mild & common form.
Very rarely causes disability.
Can only detected by x-ray or investigating a back injury.
May be associated with tethered cord
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Usually associated with skin visible signs on the back.
– Dimple
– small hair growth
– Nevus flaminous (red spot) or port wine
– Pad of subcutaneous fat
6Dimple Dimple
7Tuft of hairTuft of hair
8Dimple with nauves port wineDimple with nauves port wine
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Tethered cord Tethered cord The spinal cord could be
caught against the vertebrae
Normal cord ends at lower end of L 1
Motor weakness of lower limbs
Sphincteric problems such as inefficient bladder control.
10Autopsy of Infant with tethered cordAutopsy of Infant with tethered cord
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MeningoceleMeningoceleMeningoceleMeningocele
Least common form Least common form
Sac contains meninges and Sac contains meninges and cerebro-spinal fluid. cerebro-spinal fluid.
Cerebro-spinal fluid protects Cerebro-spinal fluid protects the brain and spinal cord. the brain and spinal cord.
The nerves are not badly The nerves are not badly damaged and able to function damaged and able to function normally. normally.
Limited disability is presentLimited disability is present. .
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MyelomeningoceleMyelomeningoceleMyelomeningoceleMyelomeningocele Most serious and common Most serious and common
The cyst not only contains The cyst not only contains meninges and c.s.f but also the meninges and c.s.f but also the
nerves and spinal cord.nerves and spinal cord.
The spinal cord is damaged or The spinal cord is damaged or not properly developed not properly developed resulting in motor and sensory resulting in motor and sensory deficit. deficit.
Majority have bowel and Majority have bowel and
bladder problems.bladder problems.
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MyelomeningoceleMyelomeningocele
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Intact MylomeningoceleIntact Mylomeningocele
Thin transparent membrane
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Intact MylomeningoceleIntact Mylomeningocelecovered by thin membranecovered by thin membrane
surrounded by hyper pigmentationsurrounded by hyper pigmentation
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Financial CostsFinancial Costs:: Average estimated lifetime cost of $532,000 for each infant born with spina bifida (CDC 1999) adds an estimated 19 million dollars every year to Missouri resident lifetime costs associated with spina bifida.
Physical CostsPhysical Costs:: Possible paralysis (the leading cause of childhood paralysis), bowel and bladder control problems, learning disabilities, hydrocephalus, surgical procedures, latex allergies, increased health problems with age
Emotional CostsEmotional Costs:: Miscarriage, stillbirth, infant mortality (death before 1st birthday), disability, feeling “different”
The High Cost of NTDsThe High Cost of NTDs
How do you get diagnosis? How do you get diagnosis?
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preNatal detection of NTDpreNatal detection of NTD
Serum alpha-fetoprotein (AFP)Serum alpha-fetoprotein (AFP)Normal fetal glycoprotein (MW= 70,000)Present normally in amniotic fluid and mother
serum start 12 week increase steadily till 32 week
High maternal serum AFP > 2 multiples of median for appropriate week of gestation is diagnostic
91% sensitivity in spina bifida
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UltrasoundUltrasound
Detect 90-95 % of cases of spina bifida 100% cases of anencephaly
In cases of elevated AFP diffrentiate NTD fron non-neurological causes of elevated AFP
e.g. omphalocele
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AmniocentesisAmniocentesis
Indication:
Pregnancies subsequent to NTD
Elevated AFP with normal US
Show elevated AFP between 12-15 week earlier than serum AFP
Carries 6% risk of abortion and fetal loss
Is prophylaxis feasible?Is prophylaxis feasible?
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Factors Associated With Factors Associated With Increased Risk of NTDs. . .Increased Risk of NTDs. . .Factors Associated With Factors Associated With
Increased Risk of NTDs. . .Increased Risk of NTDs. . . Family history of NTD
A previous pregnancy affected with NTD
Maternal insulin-dependent diabetes
Maternal obesity
Anti-epileptic drugs (Valporic Acid, Carbamazapine)
Lower socioeconomic/educational level, dietry deficiency specially folic acid
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The only most significant risk factor associated with NTDs is folic acid deficiency
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Folic Acid For WomenFolic Acid For WomenFolic Acid For WomenFolic Acid For Women As NTD occur before diagnosis
of pregnancy.
All women of childbearing age should receive 400 micrograms (0.4 mg) of folic acid daily.
Women who have had a previous child with NTD should receive 4000 micrograms (4 mg) of folic acid daily.
What is the proper management?What is the proper management?
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Nursing CareNursing Care
Like any other neonate with congenital anomalies efforts should be towards careful examination and investigations to rule out other anomalies.
Nursed in Trendlenburg position aiming to reduce pressure and keep it away from cystic lesion.
Much care not to disturb intact membrane (high incidence of infection and urgent surgery).
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Cover lesion with Gauze ring soaked with normal saline or Ringer solution to prevent dryness
Avoid antiseptics e.g betadine as it is Neurotoxic affecting functioning roots in placode
Avoid mechanical trauma to placode
no need for ultra frequent dressing
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General assessmentGeneral assessment Assess whether lesion is ruptured or unruptured
– Ruptured lesions start prophylactic antibiotic– Urgent surgery
Measure size and site of defect for proper planing for closure
Evaluation by neonatologist – Other anomalies (average 2-2.5% additional anomalies)– Condition oppose with surgery e.g lung immaturity
Bladder – Start with on regular urinery catheterization– Urological consultation
Orthopedic consultation for sever kyphotic or scoliotic deformities and hip, knee and foot deformities
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Neurological preop. AssessmentNeurological preop. Assessment Watch for spontaneous movement of lower limbs which
associated with better outcome. Assess lowest level of neurological function
– Response to painful stimuli– Differentiate between voluntary movement from reflex
movement which is stereotyped and not persist after stimulus
Evaluate other neurological associations– Hydrocephalus
Anterior fontanel Head circumference
– Chiari II Check for inspiratory stridor and apneic episodes
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Intra-operativeIntra-operative
Cauda equina Dural edge
placode
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Post-operativePost-operative
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Baby Samuel Armas's tiny fingers grasped the Baby Samuel Armas's tiny fingers grasped the doctor's huge hand - - not at birth but, at 21 weeksdoctor's huge hand - - not at birth but, at 21 weeksduring surgery for repair of MMduring surgery for repair of MMSamuel Armas Photo 8/19/99; Born 12/2/99Samuel Armas Photo 8/19/99; Born 12/2/99
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Sara Switzer Photo: July 1Sara Switzer Photo: July 1stst /99; Born August 22 /99; Born August 22ndnd /99 /99 BORN TWICEBORN TWICE
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HydrocephalusHydrocephalus
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