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Occipital encephalocele: a pathologic and anatomic analysis.
Acta Neuropathologica
|September 6, 1975
Summary
This study examines a rare congenital brain malformation in an infant, identifying a rhombic roof ventriculocele and occipital lobe encephalocele. These conditions led to cerebrospinal fluid flow obstruction and subsequent brain herniation.
Area of Science:
- Neuroscience
- Developmental Biology
- Pediatric Pathology
Background:
- Congenital brain malformations present significant diagnostic and therapeutic challenges.
- Understanding the embryological basis of these anomalies is crucial for predicting outcomes.
Purpose of the Study:
- To characterize a rare case of a cystic occipital mass present at birth.
- To elucidate the pathogenesis of a rhombic roof ventriculocele and associated encephalocele.
Main Methods:
- Serial sectioning of the brain from a neonate with a congenital occipital mass.
- Histopathological examination to identify the nature and extent of the malformation.
Main Results:
- The occipital mass was identified as a rhombic roof ventriculocele.
- An occipital lobe encephalocele, a diverticulum of the left lateral ventricle, was associated with a microgyric cortical defect.
- Posterior cerebral artery occlusion and herniation of cerebral hemispheres were observed.
- Dilated aqueduct and spinal cord central canal with collapsed lateral ventricles were noted.
Conclusions:
- Hydrocephalus secondary to cerebrospinal fluid obstruction likely initiated a cascade of events.
- This cascade led to the formation of the rhombic roof ventriculocele, posterior cerebral artery occlusion, and lateral ventricle diverticulation.