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Journal of Pediatric Neurology 2013; 11(03): 197-200
DOI: 10.3233/JPN-130618
Georg Thieme Verlag KG Stuttgart – New York

Lobar holoprosencephaly with Arnold-Chiari type III malformation

Suman Das
a   Department of Pediatrics, Krishnagar District Hospital, Nadia, India
,
Jayitri Mazumdar
b   Department of Pediatrics, Calcutta National Medical College, Kolkata, India
,
Kaushani Chatterjee
c   Department of Pediatrics, Calcutta Medical College, Kolkata, India
,
Nirmalya Sarkar
d   Department of Neonatology, Institute of Post Graduate Medical Education and Research, Kolkata, India
,
Bholanath Aich
e   Department of Pediatrics, Behrampore Medical College, Behrampore, Murshidabad, India
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Publication History

04 February 2013

29 April 2013

Publication Date:
30 July 2015 (online)

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Abstract

Holoprosencephaly (HPE) sequence is a rare spectrum of cerebral and facial malformations resulting from incomplete division of embryonic prosencephalon into diencephalon and telencephalon. De Meyer classified three ranges in order of increasing severity: lobar, semilobar and alobar HPE. A subtype called middle inter-hemispheric fissure variant has also been described. Arnold-Chiari III malformation is extremely rare and comprises of occipital encephalocele and associated cerebellar and brain stem abnormalities. This case report presents a male infant with lobar HPE and Arnold-Chiari III malformation without any facial defects and chromosomal abnormalities.

Keywords

Holoprosencephaly - Arnold-Chiari III malformation - agenesis of corpus callosum - occipital encephalocele