1 / 46

Disorders of Neural Tube Closure

Disorders of Neural Tube Closure. Jan M. Eckermann, MD, PGY-3 Department of Neurosurgery Loma Linda University Medical Center. Review of Neuroembryology. Dorsal induction Primary neurolation: 3-4 wks gestation Brain and upper spine

jennis
Télécharger la présentation

Disorders of Neural Tube Closure

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Disorders of Neural Tube Closure Jan M. Eckermann, MD, PGY-3 Department of Neurosurgery Loma Linda University Medical Center

  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. Neural groove

  4. Neural tube

  5. Whereyouat??

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

  7. Chiari I ANATOMY • Peglike cerebellar tonsils displaced into upper cervical canal ( >10mm) • HCP 25% • Syringomyelia 60% • Skeletal anomalies 25%

  8. Chiari I: displaced tonsils

  9. Chiari I: syringomyelia

  10. Chiari I: syringomyelia

  11. Chiari I Clinical • Asympotmatic (30%) • Headache • Weakness • Cerebellar signs • Herniation >12mm invariably symptomatic

  12. Chiari I Treatment • Observation if asymptomatic • Surgery if symptomatic: p-fossa decompression +- cervical laminectomy

  13. Whereyouat??

  14. Chiari II Anatomy • Calvarial defects “Lueckenschaedel” • Small p-fossa • Fenestrated falx • HCP in 90% • Myelomeningocele in 100% • Syringohydromyelia in 50%-90%

  15. Chiari II: Brain MRI

  16. Chiari II: lueckenschaedel, falx

  17. Chiari II Clinical • Dysphagia • Apneic spells • Stridor • Aspiration • Arm weakness

  18. Chiari II Treatment • Shunt • P-fossa decompression • Repair of MM

  19. Chiari III and IV Chiari III Most severe form. Chiari II + low occiptial or high cervical encephalocele. Usually incompatible with life Chiar IV Severe cerebellar hypoplasia or absence. No herniation Extremely rare

  20. Whereyouat??

  21. 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

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

  23. Encephaloceles • Parietal encephalocele • Frontoethmoidal encephalocele

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

  25. 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.

  26. Anencephaly

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

  28. Whereyouat??

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

  30. Spinal Dysraphism • Spina bifida occulta

  31. Spinal Dysraphism • Meningocele

  32. Spinal Dysraphism • Myelomeningocele

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

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

  35. Meningomyelocele

  36. Meningomyelocele

  37. Myelomeningocele • Clinical • Mild to complete LE paralysis • Ruptured vs unruptured • Urinary incontinence • Skeletal abnormalities

  38. 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

  39. Lipomyelomeningocele • Present with back mass, bladder problems, paralysis • Cutaneous stigmata • Symptoms are due to tethered cord and cord compression from fatty mass • Treatment is surgical decompression

  40. Whereyouat??

  41. Tethered Cord Syndrome • Anatomy • Low conus medullaris • Short, thick filum terminale • Intradural lipoma

  42. Tethered Cord Syndrome

  43. Tethered Cord Syndrome • Clinical • Cutaneous findings • Gait difficulties • Visible muscle atrophy • LE sensory deficits • Bladder dysfunction • Scoliosis

  44. Tethered Cord Syndrome • Treatment • Laminectomy with division of filum terminale • Removal of lipoma if present • Followed with MRI

  45. Thank you

More Related