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

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1 Years  

Male  

Head-Brain and brain nerves  

circulatory  

A one year old male child was brought with history of grossly delayed milestones and repeated bouts of seizure. History of premature delivery was given by parents.  

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.  

<- view MRI 1

MRI 1: Axial T1WI showing dilated and separated ventricles with bilateral fronto-parietal encephalomalacic changes.





<- view MRI 2

MRI 2: Coronal T1WI showing absent corpus callosum with dilated ventricles and bilateral fronto-parietal encephalomalacea.





<- view MRI 3

MRI 3: Coronal T1WI showing colpocephaly.





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MRI 4: Sagittal T1WI showing complete absence of corpus callosum with encephalomalacic changes involving fronto-parietal white matter.





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MRI 5: Axial T2WI showing dilated ventricles with bilateral encephalomalacic changes.





<- view MRI 6

MRI 6: Axial T2WI showing absence corpus callosum more clearly.





<- view MRI 7

MRI 7: Coronal FLAIR image showing similar findings to coronal T1WI.



 

Expert's opinion  

Developmental anomaly , Infective lesion etc.  

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
 

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.  

N/A  

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, Agenesis, Encephalomalacea, Colpocephaly, child, childhood, pediatric radiology  

Sanjeeb Kumar, GS Ahmed. Agenesis Of The Corpus Callosum. PedRad [serial online] vol 9, no. 4.
URL: www.PedRad.info/?search=20090427151244