Clinical manifestations in semilobar holoprosencephaly

  • Elisa Elisa Pediatric Intensive Care Unit, ]ogja International Hospital, Yogyakarta, Central Java
  • A. H. Putranti Department of Child Health, Diponegoro University Medical School/Dr. Karyadi Hospital, Semarang, Central Java
  • E. Mulyono Department of Radiology, Gadjah Mada University Medical School/Dr. Sardjito Hospital, Yogyakarta, Central Java
DOI: https://doi.org/10.14238/pi51.3.2011.182-6
Keywords: Holoprosencephaly, HPE, brain malformation

Abstract

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

References

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Published
2011-06-30
How to Cite
1.
Elisa E, Putranti AH, Mulyono E. Clinical manifestations in semilobar holoprosencephaly. PI [Internet]. 30Jun.2011 [cited 26Apr.2024];51(3):182-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/722
Issue
Vol 51 No 3 (2011): May 2011
Section
Case Report

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Received 2016-09-28
Accepted 2016-09-28
Published 2011-06-30