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Synonyms: Primary acalvaria

A rare malformation characterized by missing scalp and flat bones over an area of the cranial vault. The size of the affected area is variable. In rare cases acalvaria involves the whole of the dome-like superior portion of the cranium comprising the frontal parietal and occipital bones. Dura mater and associated muscles are absent in the affected area but the central nervous system is usually unaffected although some neuropathological abnormality is often present (e.g. holoprosencephaly or gyration anomalies). Skull base and facial bones are normal.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version July 2024

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