Acrania

Acrania is a rare congenital disorder that occurs in the human fetus, where the flat bones in the cranial vault are either completely or partially absent. The condition is often associated with anencephaly, a severe neural tube defect where the brain and surrounding structures fail to develop properly.

Etiology

The exact cause of Acrania is unknown, but it is believed to be multifactorial, involving both genetic and environmental factors. Some studies suggest a possible association with maternal exposure to certain drugs or toxins during pregnancy, but more research is needed to confirm these findings.

Epidemiology

Acrania is a rare condition, affecting approximately 1 in every 20,000 pregnancies. It is more common in females than in males, and the risk increases with maternal age.

Clinical Presentation

In cases of Acrania, the fetus lacks the flat bones that normally form the cranial vault, resulting in exposure of the brain tissue. This can be detected during a routine ultrasound scan during pregnancy. Other associated abnormalities may also be present, such as spina bifida or heart defects.

Diagnosis

Diagnosis of Acrania is usually made during a routine ultrasound scan during pregnancy. The absence of the cranial vault and exposure of the brain tissue is a characteristic finding. Further tests may be carried out to confirm the diagnosis and check for associated abnormalities.

Management

There is currently no cure for Acrania and management is supportive. The prognosis is extremely poor, with most affected fetuses dying in utero or shortly after birth. In some cases, termination of pregnancy may be considered.

See Also

• Anencephaly
• Neural tube defects
• Congenital disorders

References

Acrania Resources
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