Hypogenesis of corpus callosum (partial agenesis of corpus callosum)
All 5 parts of CC are not present → shortening of CC in AP dimension
Hypoplasia of corpus callosum
Diffusely thin CC with normal AP length & all parts present
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Sileo FG et al: Role of prenatal magnetic resonance imaging in fetuses with isolated agenesis of corpus callosum in the era of fetal neurosonography: a systematic review and meta-analysis. Acta Obstet Gynecol Scand. 100(1):7-16, 2021
Yang E et al: A practical approach to supratentorial brain malformations: what radiologists should know. Radiol Clin North Am. 55(4):609-27, 2017
Cesaretti C et al: Variability of forebrain commissures in callosal agenesis: a prenatal MR imaging study. AJNR Am J Neuroradiol. 37(3):521-7, 2016
Wiechec M et al: Four steps in diagnosing complete agenesis of the corpus callosum in prenatal life. Ultraschall Med. 37(1):92-9, 2016
Neal JB et al: Morphometric variability of neuroimaging features in children with agenesis of the corpus callosum. BMC Neurol. 15:116, 2015
Rüland AM et al: Prenatal diagnosis of anomalies of the corpus callosum over a 13-year period. Ultraschall Med. 37(6):598-603, 2016
Shen O et al: Abnormal shape of the cavum septi pellucidi: an indirect sign of partial agenesis of the corpus callosum. Ultrasound Obstet Gynecol. 46(5):595-9, 2015
Edwards TJ et al: Clinical, genetic and imaging findings identify new causes for corpus callosum development syndromes. Brain. 137(Pt 6):1579-613, 2014
Palmer EE et al: Agenesis of the corpus callosum: a clinical approach to diagnosis. Am J Med Genet C Semin Med Genet. 166C(2):184-97, 2014
Díaz-Guerrero L et al: Assessment of pericallosal arteries by color Doppler ultrasonography at 11-14 weeks: an early marker of fetal corpus callosum development in normal fetuses and agenesis in cases with chromosomal anomalies. Fetal Diagn Ther. 34(2):85-9, 2013
Ho ML et al: Lesions of the corpus callosum. AJR Am J Roentgenol. 200(1):W1-16, 2013
Paladini D et al: Agenesis of the fetal corpus callosum: sonographic signs change with advancing gestational age. Ultrasound Obstet Gynecol. 42(6):687-90, 2013
Barkovich AJ et al: Pediatric Neuroimaging: 5th Edition. Lippincott Williams & Wilkins. 368-82, 2012
Santo S et al: Counseling in fetal medicine: agenesis of the corpus callosum. Ultrasound Obstet Gynecol. 40(5):513-21, 2012
Hanna RM et al: Distinguishing 3 classes of corpus callosal abnormalities in consanguineous families. Neurology. 76(4):373-82, 2011
Thompson DK et al: Characterization of the corpus callosum in very preterm and full-term infants utilizing MRI. Neuroimage. 55(2):479-90, 2011
Ghi T et al: Prenatal diagnosis and outcome of partial agenesis and hypoplasia of the corpus callosum. Ultrasound Obstet Gynecol. 35(1):35-41, 2010
Raybaud C: The corpus callosum, the other great forebrain commissures, and the septum pellucidum: anatomy, development, and malformation. Neuroradiology. 52(6):447-77, 2010
Tang PH et al: Agenesis of the corpus callosum: an MR imaging analysis of associated abnormalities in the fetus. AJNR Am J Neuroradiol. 30(2):257-63, 2009
Hopkins B et al. Neuroimaging aspects of Aicardi syndrome. Am J Med Genet A. 146A(22):2871-8, 2008
Hetts SW et al: Anomalies of the corpus callosum: an MR analysis of the phenotypic spectrum of associated malformations. AJR Am J Roentgenol. 187(5):1343-8, 2006
Sztriha L: Spectrum of corpus callosum agenesis. Pediatr Neurol. 32(2):94-101, 2005
Lee SK et al: Diffusion tensor MR imaging visualizes the altered hemispheric fiber connection in callosal dysgenesis. AJNR Am J Neuroradiol. 25(1):25-8, 2004
Küker W et al: Malformations of the midline commissures: MRI findings in different forms of callosal dysgenesis. Eur Radiol. 13(3):598-604, 2003
Barkovich AJ et al: Callosal agenesis with cyst: A better understanding and new classification. Neurology. 23;56(2):220-7, 2002
Sato N et al: MR evaluation of the hippocampus in patients with congenital malformations of the brain. AJNR Am J Neuroradiol. 22(2):389-93, 2001
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Definitions
Heterogeneous condition of congenital hypoplasia/dysplasia or absence of corpus callosum
Complete agenesis of corpus callosum (ACC)
Agenesis of CC & hippocampal commissure with presence of anterior commissure: Most common
ACC with presence of anterior & hippocampal commissures
Hypogenesis of corpus callosum (partial agenesis of corpus callosum)
All 5 parts of CC are not present → shortening of CC in AP dimension
Hypoplasia of corpus callosum
Diffusely thin CC with normal AP length & all parts present
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Sileo FG et al: Role of prenatal magnetic resonance imaging in fetuses with isolated agenesis of corpus callosum in the era of fetal neurosonography: a systematic review and meta-analysis. Acta Obstet Gynecol Scand. 100(1):7-16, 2021
Yang E et al: A practical approach to supratentorial brain malformations: what radiologists should know. Radiol Clin North Am. 55(4):609-27, 2017
Cesaretti C et al: Variability of forebrain commissures in callosal agenesis: a prenatal MR imaging study. AJNR Am J Neuroradiol. 37(3):521-7, 2016
Wiechec M et al: Four steps in diagnosing complete agenesis of the corpus callosum in prenatal life. Ultraschall Med. 37(1):92-9, 2016
Neal JB et al: Morphometric variability of neuroimaging features in children with agenesis of the corpus callosum. BMC Neurol. 15:116, 2015
Rüland AM et al: Prenatal diagnosis of anomalies of the corpus callosum over a 13-year period. Ultraschall Med. 37(6):598-603, 2016
Shen O et al: Abnormal shape of the cavum septi pellucidi: an indirect sign of partial agenesis of the corpus callosum. Ultrasound Obstet Gynecol. 46(5):595-9, 2015
Edwards TJ et al: Clinical, genetic and imaging findings identify new causes for corpus callosum development syndromes. Brain. 137(Pt 6):1579-613, 2014
Palmer EE et al: Agenesis of the corpus callosum: a clinical approach to diagnosis. Am J Med Genet C Semin Med Genet. 166C(2):184-97, 2014
Díaz-Guerrero L et al: Assessment of pericallosal arteries by color Doppler ultrasonography at 11-14 weeks: an early marker of fetal corpus callosum development in normal fetuses and agenesis in cases with chromosomal anomalies. Fetal Diagn Ther. 34(2):85-9, 2013
Ho ML et al: Lesions of the corpus callosum. AJR Am J Roentgenol. 200(1):W1-16, 2013
Paladini D et al: Agenesis of the fetal corpus callosum: sonographic signs change with advancing gestational age. Ultrasound Obstet Gynecol. 42(6):687-90, 2013
Barkovich AJ et al: Pediatric Neuroimaging: 5th Edition. Lippincott Williams & Wilkins. 368-82, 2012
Santo S et al: Counseling in fetal medicine: agenesis of the corpus callosum. Ultrasound Obstet Gynecol. 40(5):513-21, 2012
Hanna RM et al: Distinguishing 3 classes of corpus callosal abnormalities in consanguineous families. Neurology. 76(4):373-82, 2011
Thompson DK et al: Characterization of the corpus callosum in very preterm and full-term infants utilizing MRI. Neuroimage. 55(2):479-90, 2011
Ghi T et al: Prenatal diagnosis and outcome of partial agenesis and hypoplasia of the corpus callosum. Ultrasound Obstet Gynecol. 35(1):35-41, 2010
Raybaud C: The corpus callosum, the other great forebrain commissures, and the septum pellucidum: anatomy, development, and malformation. Neuroradiology. 52(6):447-77, 2010
Tang PH et al: Agenesis of the corpus callosum: an MR imaging analysis of associated abnormalities in the fetus. AJNR Am J Neuroradiol. 30(2):257-63, 2009
Hopkins B et al. Neuroimaging aspects of Aicardi syndrome. Am J Med Genet A. 146A(22):2871-8, 2008
Hetts SW et al: Anomalies of the corpus callosum: an MR analysis of the phenotypic spectrum of associated malformations. AJR Am J Roentgenol. 187(5):1343-8, 2006
Sztriha L: Spectrum of corpus callosum agenesis. Pediatr Neurol. 32(2):94-101, 2005
Lee SK et al: Diffusion tensor MR imaging visualizes the altered hemispheric fiber connection in callosal dysgenesis. AJNR Am J Neuroradiol. 25(1):25-8, 2004
Küker W et al: Malformations of the midline commissures: MRI findings in different forms of callosal dysgenesis. Eur Radiol. 13(3):598-604, 2003
Barkovich AJ et al: Callosal agenesis with cyst: A better understanding and new classification. Neurology. 23;56(2):220-7, 2002
Sato N et al: MR evaluation of the hippocampus in patients with congenital malformations of the brain. AJNR Am J Neuroradiol. 22(2):389-93, 2001
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