Radiographic Synopsis

PA, AP Towne's, and LL projections, sometimes supplemented with tangential projections. Plain films may outline bony defects of the cranial vault, including parietal foramina and cranium bifidum. Computed tomography (CT), especially if supplemented with high-resolution techniques, delineates the bony defects in all areas of the skull, including those that remain inaccessible to conventional radiography, such as the frontobasal area. Three-dimensional CT reconstructions are best suited to complex malformations of the facial skeleton and skull base, being extremely helpful in the presurgical workup. MR imaging elegantly displays occult fistulas, the anatomy of the herniated masses, and the underlying brain (very helpful in basal cephaloceles).

1. Well-marginated, oval areas of radiolucency in the parietal bones straddling the sagittal suture; max. diameter >5 mm.; separated by a median, thin bony bridge (foramina parietalia permagna)

2. Foramina parietalia permagna; multiple exostoses; occasionally, craniofacial dysostosis (Potocki-Shaffer syndrome)

3. Midline ossification defect at various skull locations; most commonly, a single small (<2 cm), well-marginated oval defect at the parieto-occipi-tal junction; more rarely, single or double ostium at or straddling the foramen caecum, respectively; other signs of craniofacial dysraphism often associated with frontobasal cephaloceles; in cran-ioschisis, wide opening of the skull with external exposure of malformed brain (cranium bifidum)

4. Cranium bifidum occultum frontalis; hypertelorism; maxillo-naso-frontal cleft (frontonasal dys-plasia)

5. Frontal cranium bifidum; brachycephaly; bilateral cleft lip/palate; hypertelorism; severe midface hypoplasia (fronto-facio-nasal dysostosis)

6. Occipital encephalocele; polydactyly; polycystic kidney (Meckel syndrome)

7. Scalp/skull defects (aplasia cutis congenita)

8. Scalp/skull defects; transverse limb defects, most common and severe in lower limbs (Adams-Oliver syndrome)

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