CT - Dandy Walker Syndrome

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  • Dandy Walker Syndrome – refer to as DWS
  • CT - Dandy Walker Syndrome

    1. 1. <ul><li>a.k.a. Dandy-Walker Malformation </li></ul><ul><li>First described by Dandy and Blackfan (1914) </li></ul><ul><li>Reported by Taggart and Walker (1942) </li></ul><ul><li>Benda (1954) coined the term DWS </li></ul>(Johns Hopkins University, n.d.)
    2. 2. <ul><li>Congenital disorder </li></ul><ul><li>CSF cystic malformation of the posterior fossa </li></ul><ul><li>Many other p. fossa malformations </li></ul><ul><ul><li>Just to confuse you </li></ul></ul><ul><ul><li>Dandy-Walker variant – less severe, no enlargement of posterior fossa </li></ul></ul>
    3. 3. <ul><li>Triad of abnormalities </li></ul><ul><ul><li>Incomplete development of vermis </li></ul></ul><ul><ul><li>Retro-vermian cyst continuous to 4 th ventricle </li></ul></ul><ul><ul><li>Hydrocephalus </li></ul></ul><ul><li>Enlargement of posterior cranial fossa </li></ul><ul><li>Elevation of tentorium </li></ul><ul><li>Other congenital/developmental problems </li></ul>
    4. 4. <ul><li>Dandy-Walker Syndrome Studied by Computed Tomography and Pneumoencephalography </li></ul><ul><ul><ul><ul><li>Masdeu, Dobben, Azar-kia </li></ul></ul></ul></ul><ul><li>Dandy-Walker malformation: Prenatal Diagnosis and Prognosis </li></ul><ul><ul><ul><ul><li>Klein, Pierre-Kahn, Boddaert, Parisot, Brunelle </li></ul></ul></ul></ul><ul><li>With supporting information </li></ul>
    5. 5. <ul><li>The cause of Dandy Walker Syndrome is largely unknown </li></ul><ul><li>DWS is a congenital disorder that may be the result of multiple predisposing factors </li></ul><ul><ul><li>exposure to rubella </li></ul></ul><ul><ul><li>cytomegalovirus </li></ul></ul><ul><ul><li>Toxoplasmosis </li></ul></ul><ul><ul><li>Alcohol during the first trimester </li></ul></ul>NORD, 2003
    6. 6. <ul><li>Diagnosis generally takes place between 3-4 years of age </li></ul><ul><li>Symptoms in early infancy: </li></ul><ul><ul><li>slow motor development </li></ul></ul><ul><ul><li>progressive enlargement of the skull </li></ul></ul><ul><li>DWS affects 1/25000 to 1/35000 births </li></ul><ul><li>Klein et al (2003) </li></ul>
    7. 7. <ul><li>Intelligence </li></ul><ul><ul><li>40% normal </li></ul></ul><ul><ul><li>40% severely retarded </li></ul></ul><ul><ul><li>20% borderline </li></ul></ul><ul><li>Hard to make prognosis </li></ul><ul><ul><li>Severity </li></ul></ul><ul><ul><li>Structures affected </li></ul></ul>
    8. 8. <ul><li>Klein et al (2003) found that vermis development is a good indicator for prognosis </li></ul><ul><li>Boddaert et al (2006) - lobulation of the vermis good prognostic factor </li></ul><ul><li>Other problems </li></ul>
    9. 9. <ul><li>Vermian lobulation </li></ul>(Boddaert et al, 2006)
    10. 10. (Boddaert et al, 2006)
    11. 11. <ul><li>Prenatal screening is possible with ultrasound </li></ul>Chen et al. 2002
    12. 12. <ul><li>CT can distinguish between DWS related hydrocephalus from simple hydrocephalus </li></ul><ul><li>CT can be used to suspect DWS, cheap, fast </li></ul><ul><li>Distinguishing subtypes of DWS is harder on CT </li></ul><ul><li>Cannot be done prenatally </li></ul>Lutfi Incescu (2003)
    13. 13. <ul><li>Agenesis of the cerebellar vermis and a cystic 4 th ventricle </li></ul>NORD, 2003
    14. 14. <ul><li>MRI is considered the GOLD STANDARD in imaging DWS </li></ul><ul><li>Excellent soft tissue representation on T2 weighted images </li></ul><ul><li>Can be done prenatally </li></ul><ul><li>Expensive, may require sedation </li></ul>
    15. 15. <ul><li>Klein et al , 2003 </li></ul>
    16. 16. <ul><li>Pneumoencephalography </li></ul><ul><li>An invasive technique first proposed by Walter Edward Dandy in 1919 </li></ul><ul><li>Consisted of replacing about 30 cc of CSF with air, creating “artificial contrast” </li></ul>GE Healthcare (2008).
    17. 17. <ul><li>Pneumoencephalography </li></ul><ul><li>Allowed the viewing of the ventricles and CSF spaces on radiographs </li></ul><ul><li>This technique was dismissed worldwide after the introduction of noninvasive computed tomography scans by Sir Godfrey Hounsfield in 1972 </li></ul>GE Healthcare (2008).
    18. 18. <ul><li>Using 4 slice pediatric protocols with the Siemens Somatom Plus-4 Volume Zoom… </li></ul>Fisherman, Elliot K. (2007). Technique < 6mo of age 6mo – 3yrs of age 3yrs – 8yrs of age Type Spiral Spiral Spiral kV 120 120 120 mAs 70 150 220 Scan Time 8.95 8.95 8.95 Pitch 3.5 3.5 3.5
    19. 19. <ul><li>For adult cases, using 64 slice protocols with the GE Lightspeed VCT… </li></ul>Fisherman, Elliot K. (2007) Technique Adult Head Type Helical kV 120 mAs 330 Speed/mm sec 21.24 Pitch 0.531
    20. 20. <ul><li>CT Cysternography </li></ul><ul><li>Similar to pneumoencephalography </li></ul><ul><li>Consists of introducing contrast to the CSF and imaging </li></ul><ul><li>Slower process but still in use today </li></ul>
    21. 23. <ul><li>Multislice CT </li></ul><ul><ul><li>Faster imaging </li></ul></ul><ul><ul><li>Better resolution </li></ul></ul>
    22. 24. <ul><li>Ultra-fast MR </li></ul><ul><ul><li>Multi-planar </li></ul></ul><ul><ul><li>In milliseconds </li></ul></ul><ul><ul><li>Eliminate fetal artifacts </li></ul></ul><ul><li>CSF flow MR </li></ul>
    23. 25. (Yildiz et al, 2006)
    24. 26. <ul><li>Multiplanar 3D sonography </li></ul>(Sanz-Cortes et al, 2007 )

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