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    Ee Ee Presentation Transcript

    • Hatem I. Badr MD. ASS. Professor Neurosurgery Department of Neurosurgery Mansoura University, Egypt CNS congenital anomalies
    • Neural Tube Development
      • Normal embryological development
        • Neural plate development -16th day
        • Cranial closure 24th day (upper spine)
        • Caudal closure 28th day (lower spine)
    •  
    • Spina 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
      • Usually associated with skin visible signs on the back.
        • Dimple
        • small hair growth
        • Nevus flaminous (red spot) or port wine
        • Pad of subcutaneous fat
    • Dimple
    • Tuft of hair
    • Dimple with nauves port wine
    • 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.
    • Autopsy of Infant with tethered cord
    • Meningocele
      • Least common form
      • Sac contains meninges and cerebro-spinal fluid.
      • Cerebro-spinal fluid protects the brain and spinal cord.
      • The nerves are not badly damaged and able to function normally.
      • Limited disability is present .
    • Myelomeningocele
      • Most serious and common
      • The cyst not only contains meninges and c.s.f but also the nerves and spinal cord.
      • The spinal cord is damaged or not properly developed resulting in motor and sensory deficit.
      • Majority have bowel and bladder problems.
    • Myelomeningocele
    • Intact Mylomeningocele Thin transparent membrane
    • Intact Mylomeningocele covered by thin membrane surrounded by hyper pigmentation
    • Financial 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 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 Costs : Miscarriage, stillbirth, infant mortality (death before 1st birthday), disability, feeling “different” The High Cost of NTDs
    • How do you get diagnosis?
    • preNatal detection of NTD
      • 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
    • Ultrasound
      • 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
    • Amniocentesis
      • 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?
    • Factors Associated With 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
      • The only most significant risk factor associated with NTDs is folic acid deficiency
    • Folic 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?
    • Nursing 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).
      • 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
    • General 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
    • Neurological 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
    • Intra-operative Cauda equina Dural edge placode
    • Post-operative
    • FOR THE FUTURE
    • Baby Samuel Armas's tiny fingers grasped the doctor's huge hand - - not at birth but, at 21 weeks during surgery for repair of MM Samuel Armas Photo 8/19/99; Born 12/2/99
    • Sara Switzer Photo: July 1 st /99; Born August 22 nd /99 BORN TWICE
    • Hydrocephalus
    • Thank You