Atretic parietal cephaloceles revisited: an enlarging clinical and imaging spectrum?

PURPOSE We describe imaging features that are clues to the diagnosis of atretic cephaloceles and discuss clinical findings and a possible mechanism by which these lesions develop. METHODS Eight children (five girls and three boys) ranging in age from 1 day to 3 years 4 months with midline subscalp lesions underwent radiologic examination with CT or MR imaging. In all cases, the lesions were surgically excised and subjected to pathologic examination. Imaging studies and medical records were reviewed retrospectively. RESULTS Six of eight children had vertical embryonic positioning of the straight sinus with a prominent superior cerebellar cistern. A "spinning-top" configuration of the tentorial incisura, a "cigar-shaped" CSF tract within the interhemispheric fissure, fenestration of the superior sagittal sinus, and "peaking" of the tentorium were associated findings helpful in making this diagnosis. Two of the eight children had findings indistinguishable from focal dermoid, six were developmentally normal, one had mild motor delay, and one died at the age of 3 years. Pathologic examination revealed glial, meningeal (arachnoid), fibrous, and dermal elements. CONCLUSION Characteristic findings on MR images and CT scans provide clues to the diagnosis of atretic cephalocele. However, even in the presence of abnormal imaging findings, these children may be developmentally normal.