Sanjeeb Kumar Sarma (Down Town Hospital/Guwahati/India), GS Ahmed (Down Town Hospital/Guwahati/India)  

Für angemeldete Besucher einsehbar (Anmeldung)  

1 Jahre  

Männlich  

Kopf-Hirn und Hirnnerven  

zirkulatorisch  

1 Jahr alter Junge wurde vorgestellt wegen grob verzoegerter Entwicklungsstoerungen und wiederholter Krampfanfaelle. Fruehgeburt per Elternangabe.  

Noch nicht aus dem Englischen uebersetzt

The corpus callosum is a white matter structure located in the midline and composed of fibers that connect both cerebral hemispheres. The development of the corpus callosum begins during the fifth week of fetal life with the formation of the primitive lamina terminalis, which thickens to form the commissural plate. Glial cells coalesce to form a bridge like structure that serves as a guide for the callosal fibers crossing the longitudinal cerebral fissure to their targets on the contra lateral side of the brain. The mature corpus callosum is formed by the seventeenth week of gestation
Complete agenesis of the corpus callosum may occur after inflammatory or vascular lesions before its complete development. Later insults result in partial agenesis of a previously normal corpus callosum; therefore, generally only the posterior portion is affected.
Agenesis of corpus callosum (ACC) may be an isolated finding; however, it is frequently associated with other malformations and genetic syndromes including chromosomal aberrations and inborn errors of metabolism. Associated central nervous system abnormalities include Chiari malformations, anomalies of neuronal migration including lissencephaly, schizencephaly, pachygyria and polymicrogyria, encephalocele, Dandy-Walker malformations, holoprosencephaly, and olivopontocerebellar degeneration. Extra cranial malformations include abnormalities of the face and of the cardiovascular, genitourinary, gastrointestinal, respiratory, and musculoskeletal systems.
Here, in this case it is associated with bilateral fronto-parietal gross encephalomalacea mainly involving the white matter, possibly suggesting hypoxic ischemic encephalopathy.  

<- MRT 1 betrachten

MRT 1: Axial T1 gewichtete MRT zeigt erweiterte und separierte Ventrikel mit bilateraler fronto-parietaler Encephalomalazie.





<- MRT 2 betrachten

MRT 2: Coronal T1 gewichtete MRT zeigt erweiterte und separierte Ventrikel mit bilateraler fronto-parietaler Encephalomalazie.





<- MRT 3 betrachten

MRT 3: Coronal T1 gewichtete MRT zeigt Kolpozephalie.





<- MRT 4 betrachten

MRT 4: Sagittal T1 gewichtete MRT zeigt komplettes Fehlen des Corpus callosum mit Encephalomalazie in fronto-parietaler weisser Substanz.





<- MRT 5 betrachten

MRT 5: Axial T2 gewichtete MRT zeigt erweiterte Ventrikel mit bilateraler Encephalomalazie.





<- MRT 6 betrachten

MRT 6: Axial T2 gewichtete MRT zeigt noch deutlicher Fehlen des Corpus callosum.





<- MRT 7 betrachten

MRT 7: Coronales FLAIR Bild zeigt aehnliche Befund wie in der coronalen T1 gewichteten Sequenz.



 

Experten-Meinung  

Entwicklungsstoerung, Infektion etc.  

Noch nicht aus dem Englischen uebersetzt

Prognosis is dependent upon the cause of the malformation. In approximately 90% of the cases of isolated ACC, development is normal. ACC can even be an occasional finding in the investigation of children with mental retardation or microcephaly. There is no treatment for this condition
 

Noch nicht aus dem Englischen uebersetzt

In this case the parents were very poor to carry out any investigation as a result this was the first scan, in fact any investigation done after birth.The milestones were so much delayed that even the head lifting was not proper till one year. We did this MR scan on academic interest in our hospital and found this condition. Only palliative treatments were given. No definite improvement till date.  

Keine Angabe  

1. Medline:
Parrish ML, Roessmann U, Levinshon MW
Agenesis of the corpus callosum: a study of the frequency of associated malformations
Ann Neurol 1979;6:349-354

2. Medline:
Jeret JS, Serur D, Wisniewski K, et al.
Clinicopathological findings associated with agenesis of the corpus callosum
Brain Dev 1987;9:255-60

3. Medline:
Barkovitch AJ, Norman D
Anomalies of the corpus callosum: correlation with further anomalies of the brain
AJR AM J Roentgenol 1988;151:171-179
 

Corpus callosum, Agenesie, Enzephalomalazie, Colpozephalie, Kind, Kindheit, Pädiatrie, Kinderheilkunde  

Sanjeeb Kumar, GS Ahmed. Agenesie des Corpus callosum. PedRad [serial online] vol 9, no. 4.
URL: www.Kinderradiologie-online.de/?search=20090427151244