Putranti, A. H.
Indonesian Pediatric Society

Published : 1 Documents

Found 1 Documents

Clinical manifestations in semilobar holoprosencephaly Elisa, Elisa; Putranti, A. H.; Mulyono, E.
Paediatrica Indonesiana Vol 51 No 3 (2011): May 2011
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (209.588 KB) | DOI: 10.14238/pi51.3.2011.182-6


Holoprosencephaly (HPE) is a brain malformation caused by a primary defect in induction and patterning of the rostral neurotube (basal forebrain) during the first 4 weeks of embryogenesis. T his defect results in incomplete separation of the cerebral hemispheres.Based on the degree of hemispheric nonseparation, HPE traditionally has been classified into three types: alobar, semilobar, and lobar.! In 1963, DeMyer et al. mentioned that defects in brain development may frequently coexist with abnormalities on the midfacial region. T he median facio-cerebral anomalies appear in various associated gradations and combinations.When combined in patterns, these facies always predict a severe, highly characteristic brain anomaly.2