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Acrania

Acrania is a rare lethal congenital anomaly characterised by an absence of the calvarium.

Epidemiology

The estimated incdence is at ~ 1:1000 pregnancies 4

Pathology 

The condition is thought to result from abnormal migration of mesenchymal tissue, which normally covers the cerebral hemispheres. The calvarial bones are partially or completely absent with relative (albeit abnormal) preservation of development of the cerebral hemispheres. The cerebral hemispheres are surrounded by a thin membrane.

Radiographic features

Antenatal ultrasound

As the ossification of the fetal cranium begins and accelerates after 9 weeks, antenatal ultrasound allows diagnosis from 11 weeks onwards. It is important to look specifically for frontal bone ossification in the axial and coronal planes.

Prognosis

Acrania is lethal and can progress to anencephaly through the acrania - anencephaly sequence 8.

Differential diagnosis

Imaging differential considerations include conditions such as 

 

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