Affiliations: Department of Pediatrics, Krishnagar District
Hospital, Nadia, India | Department of Pediatrics, Calcutta National Medical
College, Kolkata, India | Department of Pediatrics, Calcutta Medical College,
Kolkata, India | Department of Neonatology, Institute of Post Graduate
Medical Education and Research, Kolkata, India | Department of Pediatrics, Behrampore Medical College,
Behrampore, Murshidabad, India
Note: [] Correspondence: Dr. Suman Das, 44 Talpukur Road, Deulpara,
Naihati, North 24 Parganas, West Bengal, India. Tel.: +91 98 36721415, +91 33
25804519; E-mail: [email protected]
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
