Jan Eckermann - August 2005 - "Disorders of Neural Tube Closure"

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Jan Eckermann - August 2005 - "Disorders of Neural Tube Closure"

  1. 1. Disorders of Neural TubeDisorders of Neural Tube ClosureClosure Jan M. Eckermann, MD, PGY-3Jan M. Eckermann, MD, PGY-3 Department of NeurosurgeryDepartment of Neurosurgery Loma Linda University Medical CenterLoma Linda University Medical Center
  2. 2. Review of NeuroembryologyReview of Neuroembryology • Dorsal inductionDorsal induction • Primary neurolation: 3-4 wks gestationPrimary neurolation: 3-4 wks gestation Brain and upper spineBrain and upper spine • Secondary neurolation: 4-5 wks gestationSecondary neurolation: 4-5 wks gestation Distal spineDistal spine
  3. 3. Neural grooveNeural groove
  4. 4. Neural tubeNeural tube
  5. 5. Whereyouat??Whereyouat??
  6. 6. Disorders of neural tube closureDisorders of neural tube closure • Chiari I-IVChiari I-IV • EncephalocelesEncephaloceles • AnencephalyAnencephaly • Corpus Callosum AgenesisCorpus Callosum Agenesis • Spinal Dysraphism (Spina bifida)Spinal Dysraphism (Spina bifida) • - Menigoceles- Menigoceles • - Myelomeningoceles- Myelomeningoceles • Tethered chordTethered chord
  7. 7. Chiari IChiari I ANATOMYANATOMY • Peglike cerebellar tonsils displaced intoPeglike cerebellar tonsils displaced into upper cervical canal ( >10mm)upper cervical canal ( >10mm) • HCP 25%HCP 25% • Syringomyelia 60%Syringomyelia 60% • Skeletal anomalies 25%Skeletal anomalies 25%
  8. 8. Chiari I: displaced tonsilsChiari I: displaced tonsils
  9. 9. Chiari I: syringomyeliaChiari I: syringomyelia
  10. 10. Chiari I: syringomyeliaChiari I: syringomyelia
  11. 11. Chiari IChiari I ClinicalClinical • Asympotmatic (30%)Asympotmatic (30%) • HeadacheHeadache • WeaknessWeakness • Cerebellar signsCerebellar signs • Herniation >12mm invariably symptomaticHerniation >12mm invariably symptomatic
  12. 12. Chiari IChiari I TreatmentTreatment • Observation if asymptomaticObservation if asymptomatic • Surgery if symptomatic: p-fossaSurgery if symptomatic: p-fossa decompression +- cervical laminectomydecompression +- cervical laminectomy
  13. 13. Whereyouat??Whereyouat??
  14. 14. Chiari IIChiari II AnatomyAnatomy • Calvarial defects “Lueckenschaedel”Calvarial defects “Lueckenschaedel” • Small p-fossaSmall p-fossa • Fenestrated falxFenestrated falx • HCP in 90%HCP in 90% • Myelomeningocele in 100%Myelomeningocele in 100% • Syringohydromyelia in 50%-90%Syringohydromyelia in 50%-90%
  15. 15. Chiari II: Brain MRIChiari II: Brain MRI
  16. 16. Chiari II: lueckenschaedel, falxChiari II: lueckenschaedel, falx
  17. 17. Chiari IIChiari II ClinicalClinical • DysphagiaDysphagia • Apneic spellsApneic spells • StridorStridor • AspirationAspiration • Arm weaknessArm weakness
  18. 18. Chiari IIChiari II TreatmentTreatment • ShuntShunt • P-fossa decompressionP-fossa decompression • Repair of MMRepair of MM
  19. 19. Chiari III and IVChiari III and IV Chiari IIIChiari III Most severe form. Chiari II + low occiptial or highMost severe form. Chiari II + low occiptial or high cervical encephalocele. Usually incompatiblecervical encephalocele. Usually incompatible with lifewith life Chiar IVChiar IV Severe cerebellar hypoplasia or absence. NoSevere cerebellar hypoplasia or absence. No herniationherniation Extremely rareExtremely rare
  20. 20. Whereyouat??Whereyouat??
  21. 21. EnephalocelesEnephaloceles • AnatomyAnatomy • Failure of the anterior neural tube to closeFailure of the anterior neural tube to close due to genetic, infection, or toxic reasons.due to genetic, infection, or toxic reasons. • 1/10001/1000 • Distorted parts of the (covered) brainDistorted parts of the (covered) brain protruding extracalvariallyprotruding extracalvarially
  22. 22. EncephaloceleEncephalocele AnatomyAnatomy Occipital 90%Occipital 90% Parietal 10%Parietal 10% TranssphenoidalTranssphenoidal FrontoethmoidalFrontoethmoidal NasalNasal
  23. 23. EncephalocelesEncephaloceles • ParietalParietal encephaloceleencephalocele • FrontoethmoidalFrontoethmoidal encephaloceleencephalocele
  24. 24. EncephalocelesEncephaloceles • ClinicalClinical Depends on involvementDepends on involvement • TreatmentTreatment Surgical excision of sac with water-tightSurgical excision of sac with water-tight dural closuredural closure
  25. 25. AnencephalyAnencephaly • 1/10001/1000 • Anencephaly is a defect in the closure ofAnencephaly is a defect in the closure of the neural tube during fetal development.the neural tube during fetal development. • Large defect of the calvarium, meninges,Large defect of the calvarium, meninges, and scalp.and scalp. • Incompatible with life.Incompatible with life.
  26. 26. AnencephalyAnencephaly
  27. 27. Corpus Callosum AgenesisCorpus Callosum Agenesis • Expansion of third ventricleExpansion of third ventricle • May present with HCP, seizuresMay present with HCP, seizures • May be incidental finding without anyMay be incidental finding without any clinical significanceclinical significance
  28. 28. Whereyouat??Whereyouat??
  29. 29. Spinal Dysraphism (spina bifida)Spinal Dysraphism (spina bifida) • Spina bifida occulta:Spina bifida occulta: 20-30% in North20-30% in North Americans, often incidental, cutaneousAmericans, often incidental, cutaneous manifestationsmanifestations • Spina bifida aperta:Spina bifida aperta: • MeningoceleMeningocele • MyelomeningoceleMyelomeningocele
  30. 30. Spinal DysraphismSpinal Dysraphism • Spina bifida occultaSpina bifida occulta
  31. 31. Spinal DysraphismSpinal Dysraphism • MeningoceleMeningocele
  32. 32. Spinal DysraphismSpinal Dysraphism • MyelomeningoceleMyelomeningocele
  33. 33. MeningoceleMeningocele • 1-2/10001-2/1000 • 1/3 have neurological deficits1/3 have neurological deficits • Surgical repair with water-tight duralSurgical repair with water-tight dural closureclosure
  34. 34. MeningomyeloceleMeningomyelocele • 1-2/1000 live birth1-2/1000 live birth • Failure of completeFailure of complete closure of caudalclosure of caudal neural tubeneural tube • 85% occur in lumbar85% occur in lumbar regionregion
  35. 35. MeningomyeloceleMeningomyelocele
  36. 36. MeningomyeloceleMeningomyelocele
  37. 37. MyelomeningoceleMyelomeningocele • ClinicalClinical • Mild to complete LE paralysisMild to complete LE paralysis • Ruptured vs unrupturedRuptured vs unruptured • Urinary incontinenceUrinary incontinence • Skeletal abnormalitiesSkeletal abnormalities
  38. 38. MyelomeningoceleMyelomeningocele • TreatmentTreatment • If open, Gent and Naf IVIf open, Gent and Naf IV • Prone, bottom upProne, bottom up • Telfa with wet gauze over lesionTelfa with wet gauze over lesion • Surgical closure within 36hrsSurgical closure within 36hrs • Shunt if overt HCPShunt if overt HCP • Urologic and Orthopaedic consultationUrologic and Orthopaedic consultation
  39. 39. LipomyelomeningoceleLipomyelomeningocele • Present with back mass, bladderPresent with back mass, bladder problems, paralysisproblems, paralysis • Cutaneous stigmataCutaneous stigmata • Symptoms are due to tethered cord andSymptoms are due to tethered cord and cord compression from fatty masscord compression from fatty mass • Treatment is surgical decompressionTreatment is surgical decompression
  40. 40. Whereyouat??Whereyouat??
  41. 41. Tethered Cord SyndromeTethered Cord Syndrome • AnatomyAnatomy • Low conus medullarisLow conus medullaris • Short, thick filum terminaleShort, thick filum terminale • Intradural lipomaIntradural lipoma
  42. 42. Tethered Cord SyndromeTethered Cord Syndrome
  43. 43. Tethered Cord SyndromeTethered Cord Syndrome • ClinicalClinical • Cutaneous findingsCutaneous findings • Gait difficultiesGait difficulties • Visible muscle atrophyVisible muscle atrophy • LE sensory deficitsLE sensory deficits • Bladder dysfunctionBladder dysfunction • ScoliosisScoliosis
  44. 44. Tethered Cord SyndromeTethered Cord Syndrome • TreatmentTreatment • Laminectomy with division of filumLaminectomy with division of filum terminaleterminale • Removal of lipoma if presentRemoval of lipoma if present • Followed with MRIFollowed with MRI
  45. 45. Thank youThank you

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