CASE 14

Clinical Presentation

A 7-month-old male presents with triangular appearance of the forehead and flattened temporal fossae, giving a “keel-shaped” appearance to the head.

Figure 14A

Figure 14B

Figure 14C

Figure 14D

Radiologic Findings

The AP plain x-ray of the skull demonstrates hypotelorism, with medial elevation of the superior orbital rims giving the face a “quizzical” appearance (Fig. 14A). Selected images from 3D CT volumerendered reconstructions: frontal (Fig. 14B1) and oblique (Fig. 14B2) show a prominent, ridged metopic suture. The frontal squama is poorly expanded, in contrast with the temporoparietal widening of the skull. The inside view of the skull (Fig. 14C) demonstrates its triangular shape (trigonocephaly). Note narrowing of the anterior cranial fossa, lateral shortening of the orbital roofs, a deep and narrow cribriform plate groove, and the bilateral bulging and widening of the middle cranial fossa. Axial imaging illustrates the trigonocephaly with the metopic beak (Fig. 14D1) and the endocranial metopic notch in the region of the anterior portion of the superior sagittal sinus (Fig. 14D2).

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