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Parietal cephalocele: clinical importance of its atretic form and associated malformations
A Yokota1, H Kajiwara, M Kohchi
1Department of Neurosurgery, University of Occupational and Environmental Health, Kitakyushu, Japan.
Insights
Parietal cephaloceles have a poorer prognosis than occipital ones, often linked to severe brain malformations. Atretic cephaloceles in the parietal midline are associated with poor outcomes, unlike those in the occipital midline.
Area of Science:
- Pediatric Neurosurgery
- Developmental Biology
- Congenital Malformations
Background:
- Cephaloceles are congenital herniations of intracranial contents through a defect in the skull.
- Parietal cephaloceles represent a significant subset, with distinct clinical and morphological characteristics.
- Understanding the varied prognoses and associated anomalies is crucial for patient management.
Purpose of the Study:
- To investigate the clinical and morphological features of parietal cephaloceles.
- To compare the outcomes of parietal cephaloceles with those in other locations.
- To differentiate types of atretic cephaloceles and their associated pathologies.
Main Methods:
- Retrospective analysis of infants with cephaloceles.
- Classification based on location (parietal vs. occipital) and type (encephalocele, meningocele, atretic cephalocele).
- Clinical assessment and morphological evaluation of cephaloceles and associated intracranial anomalies.
Main Results:
- Parietal cephaloceles occurred in 15 infants (37.5% of cases) and had a less favorable prognosis than occipital cephaloceles.
- 87% of parietal cephalocele patients had grave congenital anomalies, with brain malformations frequently observed.
- Two distinct types of atretic cephaloceles were identified: parietal midline (poor prognosis, associated with dorsal cyst malformations) and occipital midline (normal development, no cerebral anomalies).
Conclusions:
- Parietal cephaloceles are associated with a higher incidence and severity of cerebral malformations, leading to a poorer prognosis.
- The location and type of atretic cephalocele significantly influence developmental outcomes.
- Further research into the pathogenesis of atretic cephaloceles and associated intracranial anomalies is warranted.
Abstract:
In this study of atretic cephaloceles, the authors have considered the pedunculated or sessile type of cephalocele and also small nonpedunculated scalp defects developing in the vertex midline. Parietal cephaloceles were found in 15 infants (10 boys and five girls), and accounted for 37.5% of all cephaloceles. They consisted of four encephaloceles, six meningoceles, and five atretic cephaloceles. The clinical and morphological characteristics of parietal cephaloceles were investigated and compared with those arising at other locations. Parietal cephaloceles carried a much less favorable prognosis than those in the occipital region, regardless of the type of cephalocele; they were associated with cerebral malformations more frequently and were more severe than occipital cephaloceles. Grave congenital anomalies were found in 87% of patients with parietal cephalocele, and only two patients (neither of whom had any other malformation) attained normal development. Brain malformations were closely related to the site from which the cephalocele issued, and dorsal cyst malformation was found in eight patients with parietal cephalocele. Two types of atretic cephaloceles were found, each in a different location. The first type was an alopecic lesion occurring in the parietal midline; all five patients with this type had dorsal cyst malformations and none developed normally. The second type was a nodular lesion developing at the occipital midline, not associated with cerebral anomalies; all five patients with this type showed normal development. The pathogenesis of atretic cephaloceles and their associated intracranial malformations are discussed.