Preferred Label : Acalvaria;
ICD-11 definition : Acalvaria is a rare malformation defined as 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
neuropathological abnormalities are often present (holoprosencephaly or gyration anomalies).
Skull base and facial bones are normal.;
ICD-11 synonym : Acranius; Acrania;
Origin ID : 1719021696;
Currated CISMeF NLP mapping
- Validated automatic mappings to NTBT
Acalvaria is a rare malformation defined as 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
neuropathological abnormalities are often present (holoprosencephaly or gyration anomalies).
Skull base and facial bones are normal.
