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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

Authors

  • 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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Further Information

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