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What is Acalvaria?

Acalvaria is an extremely rare congenital birth defect that is noted by the absence ofΠtheΠflat skull bonesΠof the brain. The size of the malformation is variable. In extremely rare instances, it may involve the entire skull. Dura mater, and other muscles of the scalp are also not found in the affected area. The skull base and facial features are fully formed and usually appear normal. The central nervous system is rarely affected. The prevalence is less than 1 per 100,000 births.

What are the symptoms of Acalvaria?

Common symptoms of Acalvaria include the following:
  • Absence of flat bones of cranial vault
  • Extra little finger
  • Duramater missing as well as associated muscles
  • Absence of skull cap
  • Failure to produce adequate levels of cortisol

What causes Acalvaria?

Acalvaria is a very rare disorder. The malformation of the skull is a postneurulation defect.

How is Acalvaria diagnosed?

Ultrasound is usually the first way Acalvaria is diagnosed. MRI is used to confirm the diagnosis because the Acalvaria is very similar to anencephaly or encephalocele and an MRI is accurate enough to differentiate the diagnosis.

What are the treatments for Acalvaria?

Acalvaria is usually lethal at birth, but there have been some supports of infants who’ve survived birth. As of 2004, there were only two reported living cases. The treatment is conservative in nature.

Where can I find out more about Acalvaria?

Acalvaria Articles