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Acrania

Case contributed by Rupesh Namdev
Diagnosis certain

Presentation

Second trimester scan for fetal anomalies. History of neural defect in previous pregnancy and history of consanguinity present

Patient Data

Age: 22 weeks
Gender: Female
ultrasound

The cranium or skull vault is absent with exposed neural tissue. The fetus has 'frog face' appearance.

Case Discussion

Acrania is the failure of calvarial mesenchymal migration under the calvarial ectoderm resulting in the absence of development of osseous cranium, scalp muscles and dura 1. Also there is failure of formation of two lobes in cerebrum. The exposure of cerebral hemispheres is called as exencephaly. Prolonged exposure of the neural tissue results in repetitive mechanical and chemical trauma to the tissue resulting in damage and distortion and finally anencephaly 2.

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