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Disorders of Neural Tube Closure

Disorders of Neural Tube Closure. Dr Malith Kumarasinghe (MBBS). Review of Neuroembryology. Dorsal induction Primary neurolation: 3-4 wks gestation Brain and upper spine Secondary neurolation: 4-5 wks gestation Distal spine. Disorders of neural tube closure. Chiari I-IV

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Disorders of Neural Tube Closure

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  1. Disorders of Neural Tube Closure Dr Malith Kumarasinghe (MBBS)

  2. Review of Neuroembryology • Dorsal induction • Primary neurolation: 3-4 wks gestation Brain and upper spine • Secondary neurolation: 4-5 wks gestation Distal spine

  3. Disorders of neural tube closure • Chiari I-IV • Encephaloceles • Anencephaly • Corpus Callosum Agenesis • Spinal Dysraphism (Spina bifida) • - Menigoceles • - Myelomeningoceles • Tethered chord

  4. Enephaloceles • Anatomy • Failure of the anterior neural tube to close due to genetic, infection, or toxic reasons. • 1/1000 • Distorted parts of the (covered) brain protruding extracalvarially

  5. Encephalocele Anatomy Occipital 90% Parietal 10% Transsphenoidal Frontoethmoidal Nasal

  6. Encephaloceles • Parietal encephalocele • Frontoethmoidal encephalocele

  7. Encephaloceles • Clinical Depends on involvement • Treatment Surgical excision of sac with water-tight dural closure

  8. Anencephaly • 1/1000 • Anencephaly is a defect in the closure of the neural tube during fetal development. • Large defect of the calvarium, meninges, and scalp. • Incompatible with life.

  9. Anencephaly

  10. Corpus callosum • Location? • Functions?

  11. Lateralization of Function • Lateralization of function refers to the idea that each hemisphere of the brain is specialized for different functions. • Each hemispheres controls the contralateral (opposite) side of the body. • Example: skin receptors and muscles mainly on the right side of the body. • Each hemisphere sees the opposite side of the world.

  12. Lateralization of Function • The left and right hemisphere exchange information primarily through a set of axons called the corpus callosum. • Other areas that exchange information include: • The anterior commissure. • The hippocampalcommissure. • A few other small commissures. • Information crosses to the other hemisphere with only a brief delay.

  13. Fig. 14-2, p. 418

  14. Corpus Callosum Agenesis • Expansion of third ventricle • May present with HCP, seizures • May be incidental finding without any clinical significance

  15. Spinal Dysraphism (spina bifida) • Spina bifida occulta: 20-30% in North Americans, often incidental, cutaneous manifestations • Spina bifida aperta: • Meningocele • Myelomeningocele

  16. Spinal Dysraphism • Spina bifida occulta

  17. Spinal Dysraphism • Meningocele

  18. Spinal Dysraphism • Myelomeningocele

  19. Meningocele • 1-2/1000 • 1/3 have neurological deficits • Surgical repair with water-tight dural closure

  20. Meningomyelocele • 1-2/1000 live birth • Failure of complete closure of caudal neural tube • 85% occur in lumbar region

  21. Meningomyelocele

  22. Meningomyelocele

  23. Myelomeningocele • Clinical • Mild to complete paralysis • Ruptured vsunruptured • Urinary incontinence • Skeletal abnormalities

  24. Myelomeningocele • Treatment • If open, Gent and Naf IV • Prone, bottom up • Telfa with wet gauze over lesion • Surgical closure within 36hrs • Shunt if overt HCP • Urologic and Orthopaedic consultation

  25. Lipomyelomeningocele • Present with back mass, bladder problems, paralysis • Cutaneous stigmata • Treatment is surgical decompression

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