Tadpole-shaped lateralized parietal atretic cephalocele associated with an ipsilateral lacrimal gland fistula and schizencephalic clefts

Background Parietal atretic cephalocele (AC) and its associated intracranial venous anomalies, such as vertical embryonic positioning of the straight sinus (VEP of SS), have, in previous reports, been exclusively restricted to the midline. Clinical presentation We report a patient with lateralized parietal AC on the right side. The AC was in the shape of a tadpole, with a large head and a long tail, extending to the proximity of the right external canthus, where a lacrimal gland fistula was observed. The superior sagittal sinus and VEP of SS were also displaced to the right side, although the sagittal suture was located at the midline. Schizencephalic clefts in the right posterior cortex were also observed. Conclusion The parietal AC, which was initially located in the midline, could conceivably have been displaced to the right side by other developmental processes. However, the relationship between lateralized AC and associated multiple anomalies on the ipsilateral side is difficult to explain monogenetically. Our case study indicates that AC might have a broader spectrum of clinical symptoms than was once thought to be the case.