Crouzon's Syndrome with a Dominant Sinus Pericranii Draining Transverse Sinus: Report of a Rare Association and Review of Literature.

INTRODUCTION

Crouzon's syndrome and sinus pericranii (SP) are rare entities. Only few cases having both the features are reported. SP most commonly drains in relation to superior sagittal sinus and their communication to major posterior dural sinuses is rare.

CASE REPORT

We report a rare case of Crouzon's syndrome with SP at a suboccipital location with termination of left transverse sinus into the SP draining further through the extracranial suboccipital and extravertebral cervical venous plexi into external jugular veins. Distal transverse sinus and sigmoid sinus on the left side were absent.

CONCLUSION

Crouzon's syndrome with SP is an extremely rare entity. SP with communication to major posterior dural venous sinuses is also rare and mostly associated with multi-suture craniosynostosis. Management depends on the volume of venous blood they are draining. Most of them are dominant type and their occlusion is not feasible. Preoperative diagnosis of a dominant SP is essential for proper surgical planning as it needs to be preserved mandatorily to prevent cerebral venous infarction.