Agenesis of the Corpus Callosum





KEY FACTS


Terminology





  • Complete absence of corpus callosum (CC)



  • May be partially present but abnormal (dysgenesis)



Imaging





  • Mild ventriculomegaly often 1st clue



  • Absent cavum septi pellucidi (CSP)




    • CSP: Anechoic, box-shaped midline structure between frontal horns of lateral ventricles



    • Do not mistake paired fornices for CSP




  • Axial plane




    • Absent CSP, teardrop-shaped ventricles (colpocephaly) , parallel lateral ventricles



    • May see associated interhemispheric cyst or lipoma




  • Coronal plane




    • Absent CSP, Texas longhorn morphology to anterior horns of lateral ventricles, nonvisualization of CC




  • Midline sagittal plane




    • Absent or abnormal CC



    • Radial, spoke-wheel, sunray appearance of medial gyri radiating to 3rd ventricle



    • Abnormal vertical course and branching of anterior cerebral artery




      • Normally pericallosal branch parallel to CC, callosomarginal parallel to cingulate gyrus





Top Differential Diagnoses





  • Mild ventriculomegaly: Ventricles have normal shape



Scanning Tips





  • Scan in all 3 planes in any fetus with absent CSP



  • Look at shape of ventricles: Colpocephaly strongly associated with callosal abnormalities



  • Look for AVID complex




    • A symmetric v entriculomegaly, i nterhemispheric cyst, d ysgenesis of CC




  • ACC seldom isolated; look for other anomalies in brain (50%) and body (60%)







Axial brain US shows teardrop-shaped lateral ventricles , pointed in the front and wide in the back. This is known as colpocephaly, which is strongly associated with agenesis of the corpus callosum and helps to distinguish it from hydrocephalus of another etiology.








Sagittal US shows radially oriented gyri converging on the 3rd ventricle . Use color Doppler in this plane to look for abnormal course/branching of anterior cerebral artery into pericallosal and callosomarginal branches.

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Nov 10, 2024 | Posted by in ULTRASONOGRAPHY | Comments Off on Agenesis of the Corpus Callosum

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