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Atretic cephalocele--report of two cases with special reference to embryology
T Yamazaki1, T Enomoto, M Iguchi
1Department of Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki 305-8575, Japan.
Insights
We describe two rare cases of atretic cephalocele, a form of cranium bifidum, in young girls. These parieto-occipital lesions, lacking neural tissue, suggest a neural crest remnant origin.
Area of Science:
- Neuroscience
- Developmental Biology
- Pathology
Background:
- Atretic cephalocele is a rare congenital anomaly, a subtype of cranium bifidum.
- Its exact nature and embryological origin remain subjects of debate in medical literature.
Observation:
- Two pediatric patients presented with hard, nonpulsatile, nonreducible parieto-occipital lumps covered by alopecic scalp.
- Surgical intervention was performed for both cases.
- Microscopic examination in one case revealed meninges beneath the mass, but no neural tissue.
Findings:
- The observed lesions lacked nervous tissue, distinguishing them from typical encephaloceles.
- Histopathological analysis supports the hypothesis of a neural crest remnant as the developmental origin.
Implications:
- Understanding the embryological basis of atretic cephalocele is crucial for accurate diagnosis and management.
- These cases contribute to the limited existing literature, aiding further research into rare craniofacial malformations.
Abstract:
We report two cases of atretic cephalocele, a diverse form of cranium bifidum. The patients were 15-year-old and 3-month-old girls, who each had a hard, nonpulsatile, nonreducible lump covered by alopecic scalp in the parieto-occipital area. They were surgically treated. In case 2, microscopical examination of the operative specimen revealed a meninges under the mass, which was devoid of nervous tissue. Such lesions have rarely been reported, and their essential nature is still the subject of controversy. Pathological and embryological aspects of atretic cephalocele are discussed on the basis of the findings; the neural crest remnant was assumed to be the developmental origin of the lesion in each of these cases.