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Developmental disease of spinal cord

congenital and developmental
treatment of spina bifida,tethered cord and syringomyelia

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Developmental disease of spinal cord

  1. 1. A N D R E A Developmental disease of spinal cord
  2. 2. Embryology  Human nervous system develops from a specialized plate of cells along the back of the embryo  Early in the development the edges of this plate begin to curl up towards each other creating the neural tube  Anterior end becomes brain and the posterior become spinal cord.Complete by 28th day of pregnancy
  3. 3. Spina Bifida  SPLIT/CLEFT SPINE  Most common NTD  In lumbosacral region  1/1000 births
  4. 4.  What causes spina bifida?  Risk factors  Pathogenesis  Types-spina bifida cystica(open type) spina bifida occulta(closed type)
  5. 5. Spina bifida apperta  Myelocele-spinal cord is exposed so that the nerve tissue lies exposed on the surface of the back without even covering of meninges  Myelomeningocele-neural placode and meninges protrude above the skin.Almost always seen with Arnold Chiari 2 malformation  Hemimyelocele-myelocele+diastematomyelia  Hemimyelomeningocele
  6. 6. Spina bifida occulta  Often unnoticed  It can be associated with other conditions that could lead to problems with movements and bladder control  Meningocele  Lipomyelomeningocele  Dermal sinus  Diastematomyelia
  7. 7. Diagnostic evaluation  Neural tube defects can usually be detected during pregnancy by testing the mother's blood (AFP screening) or a detailed foetal ultrasound.  Increased levels of maternal serum alpha- fetoprotein (MSAFP) should be followed up by two tests - an ultrasound of the foetal spine and amniocentesis (to test for alpha- fetoprotein and acetylcholinesterase).
  8. 8. Treatment  There is no known cure for nerve damage caused by spina bifida.  The spinal cord and its nerve roots are put back inside the spine and covered with meninges.  In addition, a shunt may be surgically installed to provide a continuous drain for the excess cerebrospinal fluid produced in the brain, as happens with hydrocephalus.  Shunts most commonly drain into the abdomen or chest wall.
  9. 9.  Monitor growth and development  Treat and evaluate nervous system issues, such as seizure disorders.  Physical therapy  Speech therapy
  10. 10. Immediate Treatment  Place the child in prone position.  Cover the affected area with sterile gauze piece dipped in normal saline.  Maintain hydration.  Monitor for associated defects.
  11. 11. Neurological disorder caused by tissue attachments that limit the movements of spinal cord within spinal column. Tethered cord syndrome
  12. 12. causes Signs and symptoms  Myelomeningocele  Lipomyelomeningoce le  Dermal sinus  Diastematomyelia  Tumor  Lesion on lower back  Fatty tumor or deep dimple  Hairy patch  Back pain  Leg pain and numbness  Leg deformity  Spine tenderness  Scoliosis  Bowel and bladder problems
  13. 13. Diagnosis MYELOGRAM USG CT MRI
  14. 14. TREATMENT  If the patient has predominantly back pain and mild weakness, a course of physical therapy may provide tethered cord treatment.  In most cases, surgical treatment of tethered cord is needed to prevent neurologic deterioration. A laminectomy is performed, the dura is opened and using the operating microscope, the spinal cord is freed from the tethering structure.
  15. 15. Syringomyelia  Development of tubular CSF filled cavity in the substance of spinal cord  Can be congenital or acquired  Congenital-associated with Chiari 1 defect  Present with loss of pain and temperature in upper limb
  16. 16.  Treatment  Chiari defect decompressed by suboccipital craniectomy or upper cervical laminectomy  Large syrinx cavity –direct decompression or drainage
  17. 17. THANK YOU