Classification

Entity Imaging Features Notes / Associations
Cranium bifidum (encephalocele) Midline calvarial defect with herniation of meninges ± brain tissue (meningocele / meningoencephalocele). Common sites: occipital > frontoethmoidal > parietal.
Aplasia cutis congenita Localized absence of skin and bone, most often at vertex; smooth margins. Usually isolated; may be seen in trisomy 13 or fetal exposure syndromes.
Parietal foramina Bilateral, symmetric, oval defects in posterior parietal bones near sagittal suture. Normal emissary vein openings if <1 cm; large = developmental variant.
Cleidocranial dysostosis Persistent wide fontanelles, wormian bones, thin calvarium, absent clavicles. Delayed suture closure, frontal bossing.
Achondroplasia Small skull base, depressed nasal bridge; no true bone “defect” but cranial dysmorphism.
Neural tube defect (acrania / exencephaly / anencephaly) Absence of cranial vault bones with exposed or absent brain tissue. Diagnosed antenatally by US/MRI.