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Spinal Dysraphism and Malformations
Research Guide
What is Spinal Dysraphism and Malformations?
Spinal dysraphism and malformations refer to a cluster of congenital neural tube defects, including spina bifida, Chiari malformation, syringomyelia, tethered cord syndrome, and associated conditions like hydrocephalus, characterized by incomplete closure of the neural tube during embryonic development.
This field encompasses 33,071 papers on management, outcomes, and research related to spina bifida and linked disorders such as neural tube defects, fetal surgery, prenatal diagnosis, neurosurgical management, and quality of life. Key studies address prenatal versus postnatal repair of myelomeningocele, showing reduced need for shunting and improved motor outcomes at 30 months with prenatal surgery. Research also covers Chiari I malformation, redefined by clinical and radiographic findings in 364 symptomatic patients, supporting its basis in para-axial mesoderm underdevelopment.
Topic Hierarchy
Research Sub-Topics
Fetal Surgery for Myelomeningocele
This sub-topic covers prenatal surgical interventions for open neural tube defects like spina bifida, including the MOMS trial outcomes. Researchers evaluate long-term neurodevelopmental and orthopedic benefits versus risks.
Tethered Cord Syndrome Management
This sub-topic addresses neurosurgical release of tethered spinal cords in spina bifida patients to alleviate neurological deficits. Researchers study postoperative outcomes, re-tethering rates, and urological improvements.
Chiari Malformation in Spina Bifida
This sub-topic examines hindbrain herniation associated with myelomeningocele and its symptomatic management. Researchers investigate decompression surgery efficacy and ventriculoperitoneal shunt interactions.
Prenatal Diagnosis of Neural Tube Defects
This sub-topic focuses on ultrasound, MRI, and biomarkers for early detection of spina bifida and related malformations. Researchers assess diagnostic accuracy and counseling impacts on parental decisions.
Hydrocephalus in Spinal Dysraphism
This sub-topic studies ventriculoperitoneal shunting and endoscopic third ventriculostomy for hydrocephalus comorbid with spina bifida. Researchers analyze infection risks, shunt revisions, and cognitive correlations.
Why It Matters
Spinal dysraphism impacts neurosurgical management and long-term quality of life, with prenatal repair of myelomeningocele demonstrating a 40% reduction in ventriculoperitoneal shunt placement and improved leg function at 30 months in the Management of Myelomeningocele Study (MOMS trial), as reported by Adzick et al. (2011) in "A Randomized Trial of Prenatal versus Postnatal Repair of Myelomeningocele". Chiari I malformation affects 364 symptomatic patients with tonsillar herniation often less than 5 mm, linked to posterior cranial fossa underdevelopment, guiding precise diagnosis and intervention per Milhorat et al. (1999) in "Chiari I Malformation Redefined: Clinical and Radiographic Findings for 364 Symptomatic Patients". These findings influence fetal surgery protocols and postnatal care, reducing hydrocephalus and tethered cord complications in public health settings.
Reading Guide
Where to Start
"A Randomized Trial of Prenatal versus Postnatal Repair of Myelomeningocele" by Adzick et al. (2011), as it provides foundational clinical trial evidence on fetal surgery outcomes for myelomeningocele, central to spinal dysraphism management.
Key Papers Explained
Adzick et al. (2011) in "A Randomized Trial of Prenatal versus Postnatal Repair of Myelomeningocele" establishes prenatal repair benefits, building on Milhorat et al. (1999) in "Chiari I Malformation Redefined: Clinical and Radiographic Findings for 364 Symptomatic Patients", which details associated hindbrain malformations. Sekhon and Fehlings (2001) in "Epidemiology, Demographics, and Pathophysiology of Acute Spinal Cord Injury" contextualizes injury mechanisms relevant to secondary spinal dysraphism complications, while Hakim and Adams (1965) in "The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure" links hydrocephalus management.
Paper Timeline
Most-cited paper highlighted in red. Papers ordered chronologically.
Advanced Directions
Research emphasizes neurosurgical interventions for tethered cord and syringomyelia, with focus on quality-of-life metrics post-fetal surgery; no recent preprints available, indicating consolidation around MOMS trial extensions and Chiari diagnostics.
Papers at a Glance
| # | Paper | Year | Venue | Citations | Open Access |
|---|---|---|---|---|---|
| 1 | A Randomized, Controlled Trial of Methylprednisolone or Naloxo... | 1990 | New England Journal of... | 2.7K | ✓ |
| 2 | Comparative aspects of the brain growth spurt | 1979 | Early Human Development | 2.5K | ✕ |
| 3 | A Randomized Trial of Prenatal versus Postnatal Repair of Myel... | 2011 | New England Journal of... | 2.2K | ✓ |
| 4 | Traumatic spinal cord injury | 2017 | Nature Reviews Disease... | 2.2K | ✕ |
| 5 | A dural lymphatic vascular system that drains brain interstiti... | 2015 | The Journal of Experim... | 2.0K | ✓ |
| 6 | Graded Histological and Locomotor Outcomes after Spinal Cord C... | 1996 | Experimental Neurology | 1.5K | ✕ |
| 7 | Epidemiology, Demographics, and Pathophysiology of Acute Spina... | 2001 | Spine | 1.4K | ✕ |
| 8 | The glymphatic pathway in neurological disorders | 2018 | The Lancet Neurology | 1.4K | ✓ |
| 9 | Chiari I Malformation Redefined: Clinical and Radiographic Fin... | 1999 | Neurosurgery | 1.3K | ✕ |
| 10 | The special clinical problem of symptomatic hydrocephalus with... | 1965 | Journal of the Neurolo... | 1.2K | ✕ |
Frequently Asked Questions
What is the efficacy of prenatal repair for myelomeningocele?
Prenatal surgery for myelomeningocele reduced the need for shunting and improved motor outcomes at 30 months compared to postnatal repair. This was shown in a randomized trial by Adzick et al. (2011) in "A Randomized Trial of Prenatal versus Postnatal Repair of Myelomeningocele". Maternal and fetal risks were associated with the procedure.
How is Chiari I malformation diagnosed?
Chiari I malformation is characterized by underdevelopment of the posterior cranial fossa and overcrowding of the hindbrain, with tonsillar herniation of less than 5 mm not excluding diagnosis. Milhorat et al. (1999) reported clinical and radiographic findings in 364 symptomatic patients in "Chiari I Malformation Redefined: Clinical and Radiographic Findings for 364 Symptomatic Patients". Symptoms include headache and syringomyelia.
What defines symptomatic hydrocephalus with normal CSF pressure?
Symptomatic hydrocephalus with normal cerebrospinal fluid pressure presents as a distinct clinical entity. Hakim and Adams (1965) described it in "The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure". It requires specific diagnostic and management approaches distinct from high-pressure hydrocephalus.
What are the outcomes of graded spinal cord contusion models?
Graded histological and locomotor outcomes differ between NYU weight-drop contusion and transection models in spinal cord injury. Basso et al. (1996) demonstrated this in "Graded Histological and Locomotor Outcomes after Spinal Cord Contusion Using the NYU Weight-Drop Device versus Transection". Contusion models better mimic clinical heterogeneity.
What is the incidence of acute spinal cord injury?
Acute spinal cord injury has an annual incidence of 15 to 40 cases per million worldwide. Sekhon and Fehlings (2001) outlined epidemiology, demographics, and pathophysiology in "Epidemiology, Demographics, and Pathophysiology of Acute Spinal Cord Injury". Causes include motor vehicle accidents and falls.
Open Research Questions
- ? How does para-axial mesoderm underdevelopment precisely contribute to posterior cranial fossa crowding in Chiari I malformation?
- ? What long-term neurodevelopmental outcomes follow prenatal myelomeningocele repair beyond 30 months?
- ? Can lymphatic drainage mechanisms mitigate hydrocephalus in spinal dysraphism?
- ? Which neurosurgical techniques best address tethered cord syndrome while preserving neural function?
- ? How do brain growth spurts influence vulnerability to neural tube defects?
Recent Trends
The field maintains 33,071 papers with no specified 5-year growth rate; high-citation works like Adzick et al. continue dominating prenatal diagnosis and fetal surgery discussions, while Milhorat et al. (1999) informs Chiari classification; no recent preprints or news in last 12 months signals steady-state research integration.
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