Acalvaria

Common Name(s)

Acalvaria

Primary acalvaria is an extremely rare malformation characterized by the absence of the flat skull bones of the brain, dura mater, and scalp muscles. The skull base and facial features are fully formed and usually appear normal.  The cause of acalvaria is still unknown.   Acalvaria can be distinguished from anencephaly, the most common differential diagnosis, by the presence of a layer of skin overlying the brain matter and normal cerebral hemispheres.  This malformation is most often lethal at birth due to other associated anomalies or to trauma during delivery, but a few surviving infants have been reported.   Prenatal diagnosis via transvaginal ultrasound and/or magnetic resonance imaging is critical for better pregnancy management.   The initial treatment is conservative, mainly aimed at supportive care and management of any associated anomalies, if present.   Infants with acalvaria are managed conservatively in the newborn period because spontaneous bone growth has been seen in some newborns with other skull abnormalities, such as aplasia cutis congenita.   Skull reconstruction by bone grafting and cranioplasty at school age has been discussed in the literature.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Acalvaria" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Acalvaria" returned 2 free, full-text research articles on human participants. First 3 results:

Acalvaria.
 

Author(s): V V Khadilkar, A V Khadilkar, A A Nimbalkar, A S Kinnare

Journal: Indian Pediatr. 2004 Jun;41(6):618-20.

 

Acalvaria is a rare congenital malformation in which the flat bones of the cranial vault, duramater and associated muscles are absent but the central nervous system is usually unaffected. Acalvaria has usually been described as a fatal anomaly and reports are rare in the Indian literature. ...

Last Updated: 31 Dec 1969

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Fetal acalvaria with amniotic band syndrome.
 

Author(s): S Chandran, M K Lim, V Y Yu

Journal: Arch. Dis. Child. Fetal Neonatal Ed.. 2000 Jan;82(1):F11-3.

 

A case of amniotic band syndrome (ABS) presenting with acalvaria is reported. ABS includes a spectrum of non-genetic anomalies, varying from simple digital band constriction to major craniofacial and visceral defects, and even fetal death. Acalvaria is a rare congenital malformation ...

Last Updated: 31 Dec 1969

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Reviews from the PubMed Database

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The terms "Acalvaria" returned 0 free, full-text review articles on human participants.

 
 
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Symptoms, Diagnosis, and Treatment

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