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Disorders of Neural Tube Closure Jan M. Eckermann, MD, PGY-3 Department of Neurosurgery Loma 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 Review of Neuroembryology • Dorsal induction • Primary neurolation: 3 -4 wks gestation Brain and upper spine • Secondary neurolation: 4 -5 wks gestation Distal spine

Neural groove Neural groove

Neural tube Neural tube

Whereyouat? ? Whereyouat? ?

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

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

Chiari I: displaced tonsils Chiari I: displaced tonsils

Chiari I: syringomyelia Chiari I: syringomyelia

Chiari I: syringomyelia Chiari I: syringomyelia

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

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

Whereyouat? ? Whereyouat? ?

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

Chiari II: Brain MRI Chiari II: Brain MRI

Chiari II: lueckenschaedel, falx Chiari II: lueckenschaedel, falx

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

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

Chiari III and IV Chiari III Most severe form. Chiari II + low occiptial 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

Whereyouat? ? Whereyouat? ?

Enephaloceles • Anatomy • Failure of the anterior neural tube to close due to 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

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

Encephaloceles • Parietal encephalocele • Frontoethmoidal encephalocele Encephaloceles • Parietal encephalocele • Frontoethmoidal encephalocele

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

Anencephaly • 1/1000 • Anencephaly is a defect in the closure of the neural 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.

Anencephaly Anencephaly

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

Whereyouat? ? Whereyouat? ?

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

Spinal Dysraphism • Spina bifida occulta Spinal Dysraphism • Spina bifida occulta

Spinal Dysraphism • Meningocele Spinal Dysraphism • Meningocele

Spinal Dysraphism • Myelomeningocele Spinal Dysraphism • Myelomeningocele

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

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

Meningomyelocele Meningomyelocele

Meningomyelocele Meningomyelocele

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

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

Lipomyelomeningocele • Present with back mass, bladder problems, paralysis • Cutaneous stigmata • Symptoms 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

Whereyouat? ? Whereyouat? ?

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

Tethered Cord Syndrome Tethered Cord Syndrome

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

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

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