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Research ArticlePEDIATRIC NEUROIMAGING

Prognostic Significance of Central Skull Base Remodeling in Chiari II Malformation

John T. Freiling, Nilesh K. Desai, Stephen F. Kralik, William E. Whitehead and Thierry A.G.M. Huisman
American Journal of Neuroradiology March 2024, DOI: https://doi.org/10.3174/ajnr.A8142
John T. Freiling
aFrom the Edward B. Singleton Department of Radiology (J.T.F., N.K.D., S.F.K., T.A.G.M.H.), Texas Children’s Hospital & Baylor College of Medicine, Houston, Texas
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Nilesh K. Desai
aFrom the Edward B. Singleton Department of Radiology (J.T.F., N.K.D., S.F.K., T.A.G.M.H.), Texas Children’s Hospital & Baylor College of Medicine, Houston, Texas
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Stephen F. Kralik
aFrom the Edward B. Singleton Department of Radiology (J.T.F., N.K.D., S.F.K., T.A.G.M.H.), Texas Children’s Hospital & Baylor College of Medicine, Houston, Texas
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William E. Whitehead
bDepartment of Neurosurgery (W.E.W.), Texas Children’s Hospital & Baylor College of Medicine, Houston, Texas
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Thierry A.G.M. Huisman
aFrom the Edward B. Singleton Department of Radiology (J.T.F., N.K.D., S.F.K., T.A.G.M.H.), Texas Children’s Hospital & Baylor College of Medicine, Houston, Texas
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  • FIG 1.
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    FIG 1.

    Comparison of normal and remodeled basiocciput and posterior atlanto-occipital membranes. A and B, Sagittal CT images demonstrate the normal appearance of the basiocciput (arrow in A) and outward convexity of the basiocciput (arrow in B). C and D, Sagittal T2-weighted images demonstrate the normal appearance of the PAOM (arrow in C) and outward convexity of the PAOM (arrow in D). Image insets demonstrate larger FOV sagittal images and the location of corresponding zoomed-in FOV.

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    FIG 2.

    Sagittal CT of the skull base demonstrating outward convexity of the basiocciput in a 10-month-old infant with CM2. Basiocciput length is measured from the basion to the dorsal spheno-occipital synchondrosis. Convexity is measured as the maximum distance orthogonal to the length line.

  • FIG 3.
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    FIG 3.

    Sagittal T2-weighted image of the posterior craniocervical junction in a 1-month-old infant with CM2. C1-opisthion length is measured from the posterior C1 arch to the opisthion. Posterior atlanto-occipital membrane convexity is measured as the maximum distance orthogonal to the length line.

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    FIG 4.

    Basiocciput convexity is increased in patients requiring HT. Basiocciput convexity (millimeters) was measured in all patients requiring HT and not requiring HT. Patients were then stratified by prenatal or postnatal repair of the ONTD and basiocciput convexities and were compared. Bars represent the mean (SD) with triple asterisks P < .001 representing statistical significance as determined by an unpaired Student t test.

  • FIG 5.
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    FIG 5.

    An increase in basiocciput convexity was associated with a higher percentage of patients requiring HT. The percentage of patients requiring HT at varying basiocciput convexities (1–4+ mm) was determined in the combined prenatal and postnatal cohorts.

Tables

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    Table 1:

    CT and MR imaging data of patients who underwent either prenatal or postnatal surgical repair of an ONTD, according to whether they required HTa

     HTNo HTP Value
    Combined prenatal and postnatal    
     Mean basiocciput convexity (mm)2.6 (SD, 0.9)1.6 (SD, 0.9)<.001
     Mean basiocciput length (mm)15.2 (SD, 1.8)17.3 (SD, 2.2)<.001
     Mean basiocciput convexity/length ratio0.17 (SD, 0.06)0.1 (SD, 0.06)<.001
     Mean convexity of PAOM (mm)2.6 (SD, 1.6)1.9 (SD, 1.4).003
     Mean lateral ventricle width (mm)35.1 (SD, 26.2)29.4 (SD, 9.9).10
     Mean FOD (mm)134.8 (SD, 17.2)152.2 (SD, 15.5)<.001
     Mean PFD (mm)51.4 (SD, 9.2)65.9 (SD, 9.6)<.001
     Mean FOD/PFD ratio2.7 (SD, 0.4)2.3 (SD, 0.2)<.001
    Prenatal only   
     Mean basiocciput convexity (mm)2.3 (SD, 0.7)1.5 (SD, 0.8)<.001
     Mean basiocciput length (mm)15.6 (SD, 2.1)17.7 (SD, 2.2)<.001
     Mean basiocciput convexity/length ratio0.15 (SD, 0.05)0.08 (SD, 0.05)<.001
     Mean convexity of PAOM (mm)2.6 (SD, 1.9)1.9 (SD, 1.4).09
     Mean lateral ventricle width (mm)35 (SD, 10.9)28.8 (SD, 9.9).02
     Mean FOD (mm)140.5 (SD, 19.0)154.2 (SD, 12.6)<.001
     Mean PFD (mm)56 (SD, 10.4)67.3 (SD, 8.0<.001
     Mean FOD/PFD ratio2.6 (SD, 0.3)2.3 (SD, 0.2)<.001
    Postnatal only   
     Mean basiocciput convexity (mm)2.8 (SD, 0.9)1.9 (SD, 1.0)<.001
     Mean basiocciput length (mm)15 (SD, 1.6)16.5 (SD, 2.0).002
     Mean basiocciput convexity/length ratio0.19 (SD, 0.06)0.12 (SD, 0.07)<.001
     Mean convexity of PAOM (mm)2.7 (SD, 1.5)1.8 (SD, 1.5).02
     Mean lateral ventricle width (mm)35.1 (SD, 31.2)30.7 (SD, 10.0).53
     Mean FOD (mm)131.6 (SD, 15.5)148 (SD, 20.0)<.001
     Mean PFD (mm)48.9 (SD, 7.5)62.8 (SD, 11.8)<.001
     Mean FOD/PFD ratio2.7 (SD, 0.4)2.4 (SD, 0.2)<.001
    • ↵a Data are presented as mean (SD). P values were determined by an unpaired Student t test.

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    Table 2:

    CT and MR imaging data of all patients regardless of whether they received HT, according to whether they received prenatal or postnatal surgical repair of an ONTDa

     All patientsPrenatalPostnatalP Value
    Mean basiocciput convexity (mm)1.8 (SD, 0.9)2.5 (SD, 1.0)<.001
    Mean basiocciput length (mm)16.9 (SD, 2.4)15.4 (SD, 1.9)<.001
    Mean basiocciput convexity/length ratio0.11 (SD, 0.06)0.17 (SD, 0.07)<.001
    Mean convexity of PAOM (mm)2.2 (SD, 1.6)2.4 (SD, 1.5).35
    Mean lateral ventricle width (mm)31.1 (SD, 10.6)33.8 (SD, 27.0).44
    Mean FOD (mm)149.1 (SD, 16.6)136.6 (SD, 18.4)<.001
    Mean PFD (mm)63.1 (SD, 10.5)53.1 (SD, 11.1)<.001
    Mean FOD/PFD ratio2.4 (SD, 0.3)2.6 (SD, 0.4)<.001
    • ↵a Data are presented as mean (SD). P values were determined by an unpaired Student t test.

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Cite this article
John T. Freiling, Nilesh K. Desai, Stephen F. Kralik, William E. Whitehead, Thierry A.G.M. Huisman
Prognostic Significance of Central Skull Base Remodeling in Chiari II Malformation
American Journal of Neuroradiology Mar 2024, DOI: 10.3174/ajnr.A8142

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Prognostic Significance of Central Skull Base Remodeling in Chiari II Malformation
John T. Freiling, Nilesh K. Desai, Stephen F. Kralik, William E. Whitehead, Thierry A.G.M. Huisman
American Journal of Neuroradiology Mar 2024, DOI: 10.3174/ajnr.A8142
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