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The small midline occipital encephalomeningocele: definition of a syndrome
M Schlitt1, J P Williams, F O Bastian
1Department of Neurosurgery, University of South Alabama Medical Center, Mobile.
Insights
This study describes rare occipital encephaloceles that perforate the superior sagittal sinus. These malformations contain brain tissue but are distinct from typical occipital encephaloceles.
Area of Science:
- Neuroscience
- Developmental Biology
- Pediatric Neurosurgery
Background:
- Encephalomeningoceles are congenital malformations involving protrusion of meninges and brain tissue through a skull defect.
- Occipital encephaloceles are the most common type, typically arising from the occipital lobes.
- The embryology and classification of rare variants require further elucidation.
Observation:
- Two neonates presented with occipital midline skin lesions at birth.
- These lesions were found to involve the superior sagittal sinus and falx cerebri.
- Abnormal cerebral tissue within the lesions lacked connection to the occipital lobes.
Findings:
- The described lesions are confirmed as true encephalomeningoceles, not glial heterotopias.
- These represent a distinct subtype characterized by superior sagittal sinus involvement.
- Their embryological origin likely differs from common occipital encephaloceles.
Implications:
- This research clarifies the classification of rare encephalomeningocele subtypes.
- Understanding the unique embryology is crucial for accurate diagnosis and surgical planning.
- Further studies are warranted to explore the long-term outcomes and management of these unusual cases.
Abstract:
An unusual group of encephalomeningoceles is described. Two children were noted at birth to have a raised area of abnormal skin at the occipital midline. Both of these lesions were found to perforate the superior sagittal sinus and to end between the leaves of the falx cerebri, and to contain a small amount of abnormal cerebral tissue with no connection to either occipital lobe. Previous case reports are reviewed; these lesions are true encephalomeningoceles, rather than glial heterotopias. The embryology of such lesions is considered, and likely differs from the much more common occipital encephalocele arising from one or both occipital lobes.