Congenital malformations of middle line associated to migration defects

Abstract

Objectives: Establish the study protocols by specific images in the middle line malformations and embryologically correlate their association to other central nervous system malformations.
Material and methods: We review 11 cases of dygenesesis of the corpus callosum with an ultrasonography, CT and MR. The associated malformations of the central nervous system, the non-neurological malformations and the association to chromosomopathies were evaluated.
Results: In 50% of the cases, we found associated neurological abnormalities which included: interhemispheric arachnoid cyst, holoprosencephaly, heterotopias of the gray matter and encephalocele. 70% presented non-neurological congenital abnormalities and 30% chromosomopathies, one of which was a genetic transmission joined to chromosome X.
Conclusions: 90% of the dygenesesis of the corpus callosum were detected by ultrasonography. The MR was the best diagnostic method in the migration disorders and in the associated central nervous system malformations. The karyotype examination in polymalformative syndromes showed 50% genetic disorders or chromosomopathies.