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Case Reports
. 2014 May;9(2):136-8.
doi: 10.4103/1817-1745.139315.

Large vertex meningoencephalocele with schizencephaly: An interesting case with neurosurgical challenge

Sudhansu Sekhar Mishra  1 , Satya Bhusan Senapati  1 , Srikanta Das  1 , Rama Chandra Deo  1
Affiliations
Case Reports

Large vertex meningoencephalocele with schizencephaly: An interesting case with neurosurgical challenge

Sudhansu Sekhar Mishra et al. J Pediatr Neurosci. 2014 May.

Abstract

An encephalocele is a developmental disorder of neural tube closure, where as schizencephaly is a developmental disorder of neuronal migration. There are only few case reports suggesting association of schizencephaly with meningoencephalocele. We report a case of 6-year-old child who was brought by her parent with complaints of progressive swelling over her vertex since birth, left sided hemiparesis and intermittent generalized tonic clonic convulsion for last 3 years. Clinical examination, computed tomography (CT) and magnetic resonance imaging (MRI) of brain revealed a large calvarial defect with meningoencephalocele and associated schizencephaly. Initially, ventriculoperitoneal (VP) shunt was carried out, followed by successful repairing of the defect. This case is interesting for late presentation, management difficulties and associated anomalies "schizencephaly", which is a rare developmental disorder.

Keywords: Microcephaly; schizencephaly; vertex meningoencephalocele.

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Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
(a) MRI of brain-Sagittal T2-weighted image showing a large vertex meningoencephalocele. Note continuity of cerebrospinal fluid between right lateral ventricle and protruding sac (b) CT scan of brain after 3D reconstruction showing a large calvarial defect over vertex with shunt tube inside (c) CT scan of brain after VP shunt showing, protruding parenchyma with a cleft extending from surface to RT lateral ventricle (schizencephaly)
Figure 2
Figure 2
(a) MRI of brain Axial T1-weighted image showing. Blue arrowhead: Heterotropic grey matter. Red arrow: Pachygyria. Blue arrow: Agenesis (b) MRI of brain Axial T1-weighted image in upper cuts showing. Red arrow: Schizencephaly, note gray matter lined clef extending across the entire cerebral hemisphere (Blue arrow). Blue arrowhead: Heterotropic grey matter (c) MRI of brain Axial T2-weighted image showing. Red arrow: Schizencephaly, CSF continuity from ventricular surface to pial surface can be marked
See this image and copyright information in PMC

References

    1. Hoving E, Blaser S, Kelly E, Rutka JT. Anatomical and embryological considerations in the repair of a large vertex cephalocele. J Neurosurg. 1999;90:537–41. - PubMed
    1. Hoving EW, Vermeij-Keers C, Mommaas-Kienhuis AM, Hartwig NG. Separation of neural and surface ectoderm after closure of the rostral neuropore. Anat Embryol (Berl) 1990;182:455–63. - PubMed
    1. O’Rahilly R, Gardner E. The timing and sequence of events in the development of the human nervous system during the embryonic period proper. Z Anat Entwicklungsgesch. 1971;134:1–12. - PubMed
    1. Metin C, Baudoin JP, Rakic S, Parnavelas JG. Cell and molecular mechanisms involved in the migration of cortical interneurons. Eur J Neurosci. 2006;23:894–900. - PubMed
    1. Pang T, Atefy R, Sheen V. Malformations of cortical development. Neurologist. 2008;14:181–91. - PMC - PubMed

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