Holoprosencephaly In A Nigerian Female: A Case Report

Chukuemaka Agi, Cecil Abere


BACKGROUND: Holoprosencephaly is a complex intracranial abnormality with 3 ranges of severity: Lobar, semi-lobar and alobar. The clinical presentation with typical facial anomalies is unique. Imaging with USS, CT and MRI are useful diagnostic tools. We here report the first case of holoprosencephaly in the University of Port Harcourt Teaching Hospital and highlight the clinical and radiological diagnosis of this condition.

METHODOLOGY:The medical records of the patient who presented with Holoprosencephaly (HPE) and literature review of the subject using available journals and medline search were utilised. The reviewed radiologic investigation modality establishing the diagnosis was contrast enhanced computed Tomography in which axial noncontrast/postcontrast 10mm sections were taken and the axial images and reconstructions( coronal/ sagittal ) were reviewed. The Computed Tomography machine used is a Helical 8 slices General Electric Machine and contrast medium administered was Iopamidol in standard dose for weight

RESULT:  A 1 day old female infant delivered in a peripheral hospital with absent nasal opening and neonatal asphyxia. Cranial computed tomography showed rudimentary nasal septum, hypotelorism and nasal apeture stenosis with absent interhemispheric fissure and falx cerebri, solitary widened monovertricle and fused thalami.

CONCLUSION Holoprosencephaly is a rare congenital structural anomaly of the prosencephalon that results in incomplete development of the brain. In its severe form it is incompatible with life. Its etiology is not fully established


holoprosencephaly; Radiologic diagnosis; Nigeria

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ISSN: 1597-4292