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NEURORADIOLOGY Table of Contents   
Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 715-717
CT And MR appearance of interhemispheric cyst with agenesis of corpus callosum - a case report


Department of Radio-Diagnosis and imaging, KLES Hospital and MRC and J.N.Medical college, Belgaum-10, Karnataka, India

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Date of Submission28-Feb-2006
Date of Acceptance10-Aug-2006
 

Keywords: Agenesis of the corpus callosum, Interhemispheric cyst.

How to cite this article:
Manjunatha Y C, Kishor V H, Patil P H, Mali R V, Patil A, Hattiholi V V. CT And MR appearance of interhemispheric cyst with agenesis of corpus callosum - a case report. Indian J Radiol Imaging 2006;16:715-7

How to cite this URL:
Manjunatha Y C, Kishor V H, Patil P H, Mali R V, Patil A, Hattiholi V V. CT And MR appearance of interhemispheric cyst with agenesis of corpus callosum - a case report. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:715-7. Available from: http://www.ijri.org/text.asp?2006/16/4/715/32330

   Introduction Top


Interhemispheric cysts associated with callosal agenesis are relatively rare lesions and have been subject to a varied and confusion terminology. The origin of interhemispheric cyst in Agenesis of Corpus Callosum (ACC) is controversial. Arachnoid cyst, neuroepithelial cyst and ependymal cysts have all been suggested are possible cause. We present a case of Interhemispheric cyst associated with callosal agenesis where in large interhemispheric cyst is adjacent to midline on either side with multiple loculations within, and enlarged ventricles without any apparent associated anomalies.


   Case Report Top


A 10 month old male baby presents to pediatric department with macrocephaly, seizures and signs of raised intracranial tension. No history of antenatal sonographic checkup.

Physical examination revealed increase head circumference, wide and tense anterior fontanelle. Hematological investigations showed no abnormality.

Patient underwent both CT scan and MRI of Brain. CT scan of brain showed large midline cyst with multiple loculations of CSF density, on either side of midline displacing the lateral ventricles and non-communicating with the ventricular system. The lateral and third ventricles were dilated, however fourth ventricle appeared normal. Contrast enhanced CT shows non enhancing cysts with enhancing cyst walls and falces cerebri.

MRI brain confirmed the complete agenesis of corpus callosum and large cyst with multiple loculations. The cingulum or medial ventricular walls could not be assessed because of sever distortion by the cysts and accompanying hydrocephalus. No other brain anomalies were identified. Open surgery was performed. The histopathological diagnosis was a neuroepithelial cyst with the feature of choroid plexus epithelia.


   Discussion Top


The association of both interhemispheric cysts with ACC is well recognized [1],[2]. In 2001, Barkovich et al [3] presented a new system for classifying morphologically distinct cases of interhemispheric cysts that occur in the presence of ACC. This classification scheme grouped 25 cases on the basis of ventricular, cystic, and gross morphologic abnormalities, with the aim of associating types of cysts that may share elements of common origin and, possibly, prognosis. The various subtypes of cysts were classified with the assumption that the presence of a specific type of underlying cyst, ventricular defect, diencephalic malformation, or genetic anomaly produces malformations, deformations, or disruptions because of mass effect that may have similar imaging appearance.

The Barkovich classification divides cases of Interhemispheric cysts associated with ACC into type 1 cysts, which are diverticula of the lateral or third ventricles, and type 2 cysts, which are loculated and do not appear to communicate with the ventricular system (Table).

Classification of agenesis of the corpus callosum with Interhemispheric cyst after Barkovich et al [3]

In some cases, classification of congenital intracranial cysts can be performed on the basis of autopsy prognosis and surgical specimens. Although definitive correlation of intracranial imaging of a cyst and histologic type has not been completed to date. On the basis of their location, morphology, and imaging characteristics, intracranial cysts associated with ACC are most likely Arachnoid, neurenteric, or ependymal in origin [3].

According to Barkovich et al classification, this case is an example of type 2a cyst, where in the cyst is multiloculated associated with ACC and noncommunicating to the ventricles.

It is difficult to understand why multilobulated cysts develop in the interhemispheric region in association with callosal agenesis and hydrocephalus unless it is, again, related to abnormal differentiation of the primitive mesenchyme; the interhemispheric mesenchyme forms the permissive substrate for axonal crossing of the midline commissures and later differentiates into the subarachnoid space. [4],[5]

The incidence is high in male patients [6], more careful analysis of the male: female ratio suggests that some types of callosal anomalies with Interhemispheric cyst are more common in males whereas others are more common in females. This finding again emphasizes that callosal agenesis with Interhemispheric cyst is not a single malformation, but a heterogeneous group of disorders that have in common callosal agenesis or hypogenesis and a midline cyst [7].

Several studies have revealed that this disorder is complex, being associated with hypoplasia or absence of the corticospinal tracts, dysplasia of the cerebral mantle, a large massa intermedia, a small brainstem, atrophy of the anterior cerebellar vermian lobules, and diffuse hypoplasia of the cerebral white matter [8].

The most important condition that must be distinguished from interhemispheric cysts is the alobar form of holoprosencephaly because to treat them as early as possible in order to prevent gross developmental deficits [9].

 
   References Top

1.Probst FP. Agenesis of the corpus callosum. Acta Radiol 1973; 331(Suppl):1-152  Back to cited text no. 1    
2.Brihaye J, Gilet P, Parmentier R, Peetrons E. Agenesie de la commissure calleuse associe ave un kyst ependymaire. Schwietz Arch Neurol Psychiatr 1956; 77:615-631.  Back to cited text no. 2    
3.Barkovich AJ, Simon EM, Walsh CA. Callosal agenesis with cyst: a better understanding and new classification. Neurology 2001; 56: 220-227.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Truwit CL, Barkovich AJ. Pathogenesis of intracranial lipoma: an MR study in 42 patients. AJNR Am J Neuroradiol 1990; 11:665-674.  Back to cited text no. 4    
5.Osaka K, Handa R, Matsumoto S, Yasuda M. Development of the cerebrospinal fluid pathway in the normal and abnormal human embryos. Childs Brain 1980; 6:26-38.  Back to cited text no. 5    
6.13. Lena G, van Calenberg F, Genitori L, Choux M. Supratentorial interhemispheric cysts associated with callosal agenesis: surgical treatment and outcome in 16 children. Childs Nerv Syst 1995; 11:568-573.  Back to cited text no. 6    
7.Bickers DS, Adams RD. Hereditary stenosis of the aqueduct of Sylvius as a cause of congenital hydrocephalus. Brain 1949; 72:246-262.  Back to cited text no. 7    
8.Yamasaki M, Arita N, Hiraga S, et al. A clinical and neuroradiological study of X-linked hydrocephalus in Japan. J Neurosurg 1995; 83:50-55.  Back to cited text no. 8  [PUBMED]  
9.Gabriel Lena, Franck van Cālenberg, Lorenzo Genitori and Maurice Choux. Supratentorial interhemispheric cysts associated with callosal agenesis: surgical treatment and outcome in 16 children. Child's Nervous System (Historical Archive) October 1995; 11: 568 - 573.   Back to cited text no. 9    

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Correspondence Address:
Y C Manjunatha
Department of Radio-Diagnosis and imaging, KLES Hospital and MRC and J.N.Medical college, Belgaum-10, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32330

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