[Congenital malformations of the brain. 2: Malformations of the corpus callosum and holoprocencephalies].
The corpus callosum is formed between the 7th and the 20th gestational week. If this process is disrupted, partial or complete callosal agenesis may ensue. As large parts of the supra- and infratentorial brain are created during this critical period, associated anomalies need always to be searched for when callosal agenesis is present. Associations with neuro-genetic syndromes also exist. The corpus callosum is generally formed from front to back ("front-to-back rule"). Therefore, a partial callosal agenesis usually involves the posterior portion of the corpus callosum, while a secondary lesion of the corpus callosum does not follow this rule. Holoprosencephalies are a notable exception to this rule, as the frontal part of the corpus callosum is absent in spite of their classification as congenital malformations. They represent a disturbance of the differentiation and cleavage of the prosencephalon with a disruption of the separation of the cerebral hemispheres. Holoprosencephalies can be due to genetic causes, but also to intrauterine infections or other teratogenic causes. The holoprosencephalies are subdivided into alobar, semilobar and lobar holoprosencephalies. This article aims to describe the most important features of callosal agenesis and holoprosencephalies highlighting the respective imaging characteristics.
Rummeny
C
C
Institut für Klinische Radiologie, Klinikum der Universität München, Grosshadern, München. claudia.rummeny@ikra.med.uni-muenchen.de
Ertl-Wagner
B
B
Reiser
M F
MF
D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000196:embryology / Q000473:pathology; D061085:Agenesis of Corpus Callosum; D002648:Child; D003337:Corpus Callosum / Q000196:embryology / Q000473:pathology; D005260:Female; D016142:Holoprosencephaly / Q000175:diagnosis* / Q000196:embryology; D006801:Humans; D007089:Image Enhancement; D007091:Image Processing, Computer-Assisted; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D009421:Nervous System Malformations / Q000175:diagnosis* / Q000196:embryology / Q000473:pathology; D011247:Pregnancy; D011296:Prenatal Diagnosis; D013577:Syndrome