Atretic cephaloceles with different imaging phenotypes – Case series with review of literature

Atretic cephaloceles (ACs) are congenital skull defects with herniation of rudimentary intracranial structures through the defect and associated with persistent falcine sinus or embryonic positioning of straight sinus. We describe five cases of ACs, out of which only one had embryonic straight sinus. Three cases had other intracranial malformations such as hypoplasia of corpus callosum, dysplastic tectum in one child and parieto-occipital polymicrogyria with falcotentorial dehiscence in the other, and frontal horn deformity and cortical dysplasia in the third. The prognosis of AC depends on the coexistent intracranial abnormalities and this highlights the role of magnetic resonance imaging in diagnosing the other associated anomalies for prediction of prognosis and planning of necessary surgical management.