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  1. 1. Hatem I. Badr MD. ASS. Professor Neurosurgery Department of Neurosurgery Mansoura University, Egypt CNS congenital anomalies
  2. 2. Neural Tube Development <ul><li>Normal embryological development </li></ul><ul><ul><li>Neural plate development -16th day </li></ul></ul><ul><ul><li>Cranial closure 24th day (upper spine) </li></ul></ul><ul><ul><li>Caudal closure 28th day (lower spine) </li></ul></ul>
  3. 4. Spina Bifida Occulta <ul><li>Very mild & common form. </li></ul><ul><li>Very rarely causes disability. </li></ul><ul><li>Can only detected by x-ray or investigating a back injury. </li></ul><ul><li>May be associated with tethered cord </li></ul>
  4. 5. <ul><li>Usually associated with skin visible signs on the back. </li></ul><ul><ul><li>Dimple </li></ul></ul><ul><ul><li>small hair growth </li></ul></ul><ul><ul><li>Nevus flaminous (red spot) or port wine </li></ul></ul><ul><ul><li>Pad of subcutaneous fat </li></ul></ul>
  5. 6. Dimple
  6. 7. Tuft of hair
  7. 8. Dimple with nauves port wine
  8. 9. Tethered cord <ul><li>The spinal cord could be caught against the vertebrae </li></ul><ul><li>Normal cord ends at lower end of L 1 </li></ul><ul><li>Motor weakness of lower limbs </li></ul><ul><li>Sphincteric problems such as inefficient bladder control. </li></ul>
  9. 10. Autopsy of Infant with tethered cord
  10. 11. Meningocele <ul><li>Least common form </li></ul><ul><li>Sac contains meninges and cerebro-spinal fluid. </li></ul><ul><li>Cerebro-spinal fluid protects the brain and spinal cord. </li></ul><ul><li>The nerves are not badly damaged and able to function normally. </li></ul><ul><li>Limited disability is present . </li></ul>
  11. 12. Myelomeningocele <ul><li>Most serious and common </li></ul><ul><li>The cyst not only contains meninges and c.s.f but also the nerves and spinal cord. </li></ul><ul><li>The spinal cord is damaged or not properly developed resulting in motor and sensory deficit. </li></ul><ul><li>Majority have bowel and bladder problems. </li></ul>
  12. 13. Myelomeningocele
  13. 14. Intact Mylomeningocele Thin transparent membrane
  14. 15. Intact Mylomeningocele covered by thin membrane surrounded by hyper pigmentation
  15. 16. 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
  16. 17. How do you get diagnosis?
  17. 18. preNatal detection of NTD <ul><li>Serum alpha-fetoprotein (AFP) </li></ul><ul><li>Normal fetal glycoprotein (MW= 70,000) </li></ul><ul><li>Present normally in amniotic fluid and mother serum start 12 week increase steadily till 32 week </li></ul><ul><li>High maternal serum AFP > 2 multiples of median for appropriate week of gestation is diagnostic </li></ul><ul><li>91% sensitivity in spina bifida </li></ul>
  18. 19. Ultrasound <ul><li>Detect 90-95 % of cases of spina bifida </li></ul><ul><li>100% cases of anencephaly </li></ul><ul><li>In cases of elevated AFP diffrentiate NTD fron non-neurological causes of elevated AFP </li></ul><ul><li>e.g. omphalocele </li></ul>
  19. 20. Amniocentesis <ul><li>Indication: </li></ul><ul><li>Pregnancies subsequent to NTD </li></ul><ul><li>Elevated AFP with normal US </li></ul><ul><li>Show elevated AFP between 12-15 week earlier than serum AFP </li></ul><ul><li>Carries 6% risk of abortion and fetal loss </li></ul>
  20. 21. Is prophylaxis feasible?
  21. 22. Factors Associated With Increased Risk of NTDs. . . <ul><li>Family history of NTD </li></ul><ul><li>A previous pregnancy affected with NTD </li></ul><ul><li>Maternal insulin-dependent diabetes </li></ul><ul><li>Maternal obesity </li></ul><ul><li>Anti-epileptic drugs (Valporic Acid, Carbamazapine) </li></ul><ul><li>Lower socioeconomic/educational level, dietry deficiency specially folic acid </li></ul>
  22. 23. <ul><li>The only most significant risk factor associated with NTDs is folic acid deficiency </li></ul>
  23. 24. Folic Acid For Women <ul><li>As NTD occur before diagnosis of pregnancy. </li></ul><ul><li>All women of childbearing age should receive 400 micrograms (0.4 mg) of folic acid daily. </li></ul><ul><li>Women who have had a previous child with NTD should receive 4000 micrograms (4 mg) of folic acid daily. </li></ul>
  24. 25. What is the proper management?
  25. 26. Nursing Care <ul><li>Like any other neonate with congenital anomalies efforts should be towards careful examination and investigations to rule out other anomalies. </li></ul><ul><li>Nursed in Trendlenburg position aiming to reduce pressure and keep it away from cystic lesion. </li></ul><ul><li>Much care not to disturb intact membrane (high incidence of infection and urgent surgery). </li></ul>
  26. 27. <ul><li>Cover lesion with Gauze ring soaked with normal saline or Ringer solution to prevent dryness </li></ul><ul><li>Avoid antiseptics e.g betadine as it is Neurotoxic affecting functioning roots in placode </li></ul><ul><li>Avoid mechanical trauma to placode </li></ul><ul><li>no need for ultra frequent dressing </li></ul>
  27. 28. General assessment <ul><li>Assess whether lesion is ruptured or unruptured </li></ul><ul><ul><li>Ruptured lesions start prophylactic antibiotic </li></ul></ul><ul><ul><li>Urgent surgery </li></ul></ul><ul><li>Measure size and site of defect for proper planing for closure </li></ul><ul><li>Evaluation by neonatologist </li></ul><ul><ul><li>Other anomalies (average 2-2.5% additional anomalies) </li></ul></ul><ul><ul><li>Condition oppose with surgery e.g lung immaturity </li></ul></ul><ul><li>Bladder </li></ul><ul><ul><li>Start with on regular urinery catheterization </li></ul></ul><ul><ul><li>Urological consultation </li></ul></ul><ul><li>Orthopedic consultation for sever kyphotic or scoliotic deformities and hip, knee and foot deformities </li></ul>
  28. 29. Neurological preop. Assessment <ul><li>Watch for spontaneous movement of lower limbs which associated with better outcome. </li></ul><ul><li>Assess lowest level of neurological function </li></ul><ul><ul><li>Response to painful stimuli </li></ul></ul><ul><ul><li>Differentiate between voluntary movement from reflex movement which is stereotyped and not persist after stimulus </li></ul></ul><ul><li>Evaluate other neurological associations </li></ul><ul><ul><li>Hydrocephalus </li></ul></ul><ul><ul><ul><li>Anterior fontanel </li></ul></ul></ul><ul><ul><ul><li>Head circumference </li></ul></ul></ul><ul><ul><li>Chiari II </li></ul></ul><ul><ul><ul><li>Check for inspiratory stridor and apneic episodes </li></ul></ul></ul>
  29. 30. Intra-operative Cauda equina Dural edge placode
  30. 31. Post-operative
  31. 32. FOR THE FUTURE
  32. 33. 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
  33. 34. Sara Switzer Photo: July 1 st /99; Born August 22 nd /99 BORN TWICE
  34. 35. Hydrocephalus
  35. 36. Thank You
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