Spina bifida

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Spina bifida

  1. 1. SPINAL DYSRAPHISM Deepak Agrawal Assistnat professor, Department of Neurosurgery & Gamma Knife, All India Institute of Medical Sciences, New Delhi
  2. 2. Neural Tube Development <ul><li>Normal embryological development </li></ul><ul><li>Neural plate development -18th day </li></ul><ul><li>Cranial closure 24th day (upper spine) </li></ul><ul><li>Caudal closure 26th day (lower spine) </li></ul>
  3. 5. Hypothesis <ul><li>Failure of Neural tube to close </li></ul><ul><li>Secondary rupture of neural tube </li></ul><ul><li>Hydrodynamic theory </li></ul><ul><li>Overgrowth theory </li></ul>
  4. 6. Etiology <ul><li>Teratogenic agent </li></ul><ul><li>Maternal deficiency </li></ul><ul><ul><li>Folic acid </li></ul></ul><ul><ul><li>Folate antagonist (Aminopterin) </li></ul></ul><ul><li>Genetic aspect-Pax-3 gene product </li></ul><ul><ul><li>Disrupted Actin microfilament </li></ul></ul>
  5. 7. TYPES OF SPINAL DYSRAPHISM <ul><li>Open neural tube defect </li></ul><ul><li>(Spina bifida aperta </li></ul><ul><li>Closed neural tube defect </li></ul><ul><li>(Spina bifida occulta) </li></ul>
  6. 8. Types of Open NTD <ul><li>Spina bifida occulta </li></ul><ul><li>Lipomeningocele </li></ul><ul><li>Meningocele </li></ul><ul><li>Myelomeningocele </li></ul>
  7. 9. Types of Closed NTD <ul><li>Tethered cord syndrome </li></ul><ul><li>Congenital tumors </li></ul><ul><li>Split cord malformation </li></ul><ul><li>Spinal lipomatous malformation </li></ul>
  8. 11. Meningomyelocele <ul><li>Commonest ONTD </li></ul><ul><li>Associated with ACM in 80% </li></ul><ul><li>Hydrocephalus in 80% </li></ul><ul><li>There may be occult SCM </li></ul>
  9. 12. Lipomyelomeningocele <ul><li>Conus lipoma </li></ul><ul><li>Dorsal lipoma </li></ul><ul><li>Transitional lipoma </li></ul><ul><li>Caudal lipoma </li></ul><ul><li>Filum lipoma </li></ul><ul><li>Lipomyelocystocele </li></ul>
  10. 13. TRANSISTIONAL LIPOMYELOMENINGOCELE
  11. 14. CAUDAL LIPOMYELOMENINGOCELE
  12. 15. DORSAL LIPOMYELOMENINGOCELE
  13. 16. Neurological profile <ul><li>Motor weakness </li></ul><ul><li>Sensory problem </li></ul><ul><li>Autonomic problem </li></ul><ul><li>Problem of micturation </li></ul><ul><li>Asymptomatic </li></ul>
  14. 17. Clinical problems in lipoma <ul><li>Progressive neurological deficit </li></ul><ul><li>Rarely present with rapid deterioration </li></ul><ul><li>Neurological progress can start at any age </li></ul><ul><li>Symptoms exaggerated during rapid growth, pregnancy, fall </li></ul>
  15. 18. Bony abnormalities in Lipoma <ul><li>Hemivertebra 5% </li></ul><ul><li>Butterfly vertebra 10% </li></ul><ul><li>Scoliosis 50% </li></ul><ul><li>Sacral agenesis 20% </li></ul><ul><li>Blocked vertebra 10% </li></ul>
  16. 19. Split cord Malformations-Incidence <ul><li>7-8/10,000 live birth </li></ul><ul><li>In US 4-14/10,000 live birth </li></ul><ul><li>Low in Africa (1/10,000 live birth) </li></ul>
  17. 20. SCM-types <ul><li>Type I </li></ul><ul><li>Type II </li></ul><ul><li>Mixed </li></ul><ul><li>Posterior spur </li></ul>
  18. 21. SCM <ul><ul><ul><ul><li>Type I 54% </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Type II 41% </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Composite 5% </li></ul></ul></ul></ul>
  19. 22. 1989-2007 <ul><li>Spinal dysraphism operated 850 </li></ul><ul><li>SCM 300 </li></ul><ul><li>Lipoma 240 </li></ul><ul><li>Dermal sinus 50 </li></ul>
  20. 23. SCM-Clinical features <ul><li>Cutaneous </li></ul><ul><li>Neural abnormality </li></ul><ul><li>Osteo-skeletal abnormalities </li></ul><ul><li>Foot abnormality </li></ul>
  21. 24. SCM-Cutaneous anomalies <ul><li>Dimple </li></ul><ul><li>Dermal sinus </li></ul><ul><li>Haemangioma, lipoma </li></ul><ul><li>Hypertrichosis </li></ul><ul><li>Rarely nipple, phallus </li></ul>
  22. 25. SCM-Neural anomalies <ul><li>Asymptomatic </li></ul><ul><li>Sensory, motor </li></ul><ul><li>autonomic disturbances </li></ul><ul><li>Non healing ulcer </li></ul>
  23. 26. SCM- Osteo-skeletal anomalies <ul><li>Kyphoscoliosis </li></ul><ul><li>Scoliosis </li></ul><ul><li>Foot deformity </li></ul>
  24. 27. INVESTIGATIONS <ul><li>Radiological </li></ul><ul><li>Electrophysiological </li></ul><ul><li>Kidney function test </li></ul>
  25. 28. INVESTIGATIONS <ul><li>Radiological </li></ul><ul><li>Plain X’ray’s </li></ul><ul><li>MRI of spine at suspected area </li></ul><ul><li>C.T.Head, C.T.Spine </li></ul>
  26. 29. INVESTIGATIONS <ul><li>Neurophysiological tests </li></ul><ul><li>Sensory evoked potentials </li></ul><ul><li>EMG </li></ul><ul><li>Nerve conduction studies </li></ul>
  27. 30. INVESTIGATIONS <ul><li>Kidney function tests </li></ul><ul><li>Urodynamic studies </li></ul><ul><li>Radioactive study for kidney morphology and function </li></ul><ul><li>MCU </li></ul>
  28. 31. Rationale of surgery <ul><li>Aim of surgery to prevent neural tissue damage </li></ul><ul><li>Surgery is relatively safe </li></ul><ul><li>One deteriorated complete recovery does not occur </li></ul><ul><li>Operation necessary before deterioration </li></ul>
  29. 32. WHEN TO OPERATE? <ul><li>If leaking meningomyelocele it is an emergency </li></ul><ul><li>If no leak-ASAP </li></ul><ul><li>In occult dysraphism operate when pt. presents </li></ul>
  30. 33. Timing of surgery- Assoc hydrocephalus <ul><li>OPTIONS </li></ul><ul><li>V.P.Shunt first </li></ul><ul><li>V.P.Shunt at time of spinal surgery </li></ul><ul><li>Spinal surgery then shunt </li></ul>
  31. 34. Aim of Surgery <ul><li>Prevent damage </li></ul><ul><li>Stabilization </li></ul><ul><li>Improvement of existing deficit </li></ul>
  32. 35. Surgical steps <ul><li>Laminotomy </li></ul><ul><li>Separation of soft tissue from bifida spine </li></ul><ul><li>Separation of dura from soft tissue </li></ul><ul><li>Identification of Neural placode, conus and root </li></ul><ul><li>Removal of sac, lipoma, spur or tumor </li></ul><ul><li>Reconstruction of conus </li></ul><ul><li>Water tight closure of dura </li></ul>
  33. 36. Complications <ul><ul><li>Spinal cord or root damage </li></ul></ul><ul><ul><li>Inadequate dura closure </li></ul></ul><ul><ul><li>Postop CSF leak, wound break down </li></ul></ul><ul><ul><li>Wound infection flap necrosis </li></ul></ul><ul><ul><li>Meningitis </li></ul></ul>
  34. 37. Surgical adjuncts <ul><li>Operating microscope </li></ul><ul><li>High speed drill for laminotomy </li></ul><ul><li>Use of intraoperative EP </li></ul><ul><li>Use of laser, vaporiser </li></ul><ul><li>Dural graft </li></ul>
  35. 38. Surgical Outcome <ul><li>Results % </li></ul><ul><li>Overall recovery 50 </li></ul><ul><li>Stabilization 45 </li></ul><ul><li>Deterioration 05 </li></ul><ul><li>Healing of trophic ulcer 40 </li></ul><ul><li>Autonomic improvement 30 </li></ul><ul><li>Retethering(5 yr) 20 </li></ul>
  36. 39. FACTORS INFLUENCE OUTCOME <ul><li>Age of the patients </li></ul><ul><li>Presence of paraplegia </li></ul><ul><ul><li>(Neurological Deficit) </li></ul></ul><ul><li>Associated Hydrocephalus </li></ul><ul><li>Other cranial anomalies </li></ul><ul><li>Other systemic anomalies </li></ul>
  37. 40. CONTROVERSIES <ul><li>Operate paraplegic or not </li></ul><ul><li>Urodynamic studies in children </li></ul><ul><li>Operation in asymptomatic cases </li></ul><ul><li>Extent of removal of lipoma </li></ul><ul><li>Surgery in multicentric spinal dysraphism </li></ul><ul><li>Timing of V P Shunt / Scoliosis surgery </li></ul>
  38. 41. Our Recommendations <ul><li>Early surgery </li></ul><ul><li>Careful handling of neural tissue </li></ul><ul><li>Adequate dural closure </li></ul><ul><li>Surgery for asymptomatic cases </li></ul>
  39. 42. Prognosis <ul><li>Spina bifida is a: </li></ul><ul><li>static </li></ul><ul><li>non-progressive defect </li></ul><ul><li>with worsening from secondary problems. </li></ul><ul><li>The prognosis for a normal life span is generally good for a child with good health habits and a supportive family/caregiver. </li></ul>
  40. 43. CONCLUSIONS <ul><li>Patient neurological deficit improve in 50% & stabilize in another 40% following surgery </li></ul>
  41. 44. CONCLUSIONS <ul><li>Is a multidisciplinary problem </li></ul><ul><li>Planning necessary </li></ul><ul><li>Asymptomatic cases should be operated </li></ul><ul><li>Surgery for paraplegic patient controversial </li></ul><ul><li>(We operate after full discussion with family) </li></ul>
  42. 45. THANK YOU

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