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. 2010 Jul;5(2):121-3.
doi: 10.4103/1817-1745.76106.

Septal agenesis and lissencephaly with colpocephaly presenting as the 'Crown Sign'

Namit Singhal  1 , Sunil Agarwal
Affiliations

Septal agenesis and lissencephaly with colpocephaly presenting as the 'Crown Sign'

Namit Singhal et al. J Pediatr Neurosci. 2010 Jul.

Abstract

Developmental malformations of the cortex in neuronal migration disorders result in constellation of findings on radiological scanning. Isolated defects are common but sometimes they occur in varying degrees of combination giving a unique appearance on the imaging studies. We describe a case of neuronal migration disorder in which the computed tomography scan showed the presence of lissencephaly, colpocephaly. and Septal agenesis. These findings make the ventricular system appear in shape of a crown, which we refer to as "CROWN SIGN", first described in neurosurgical literature..

Keywords: colpocephaly; crown sign; lissencephaly; septal agenesis.

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Conflict of interest statement

Conflict of Interest: None declared

Figures

Figure 1
Figure 1
Noncontrast axial CT scan showing the presence of lissencephaly with pachygyria-agyria complex
Figure 2
Figure 2
Noncontrast axial CT scan showing the presence of colpocephaly with septal agenesis
Figure 3
Figure 3
Dandy-Walker variant presenting as the ‘key sign’
Figure 4
Figure 4
Ventricular system presenting as the ‘Crown Sign’
See this image and copyright information in PMC

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