Supplementary Material for: 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 are rare entities. Only few cases having both the features are reported. Sinus pericranii most commonly drain 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 sinus pericranii at a sub-occipital location with termination of left transverse sinus into the sinus pericranii draining further through the extracranial suboccipital and extravertebral cervical venous plexi into external jugular veins. Distal transverse sinus and sigmoid sinus on left side were absent. Discussion/Conclusion - Crouzon’s syndrome with sinus pericranii is an extremely rare entity. Sinus pericranii with communication to major posterior dural venous sinuses are 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 sinus pericranii is essential for proper surgical planning as it needs to be preserved mandatorily to prevent cerebral venous infarction.

引言——克鲁宗综合征（Crouzon’s syndrome）与颅骨骨膜窦（sinus pericranii）均为罕见病症，目前同时兼具两种病症特征的病例报道极少。颅骨骨膜窦最常见的引流关联区域为上矢状窦（superior sagittal sinus），其与主要颅后硬脑膜窦的交通通路极为罕见。病例报告——本文报道1例极为罕见的合并枕下区颅骨骨膜窦的克鲁宗综合征病例：患者左侧横窦（left transverse sinus）终止于该颅骨骨膜窦，并进一步通过颅枕下及椎外颈静脉丛（extravertebral cervical venous plexi）引流至颈外静脉（external jugular veins）；左侧远端横窦与乙状窦（sigmoid sinus）缺如。讨论与结论——合并颅骨骨膜窦的克鲁宗综合征属于极罕见病症。与主要颅后硬脑膜窦交通的颅骨骨膜窦同样少见，且多伴随多缝颅缝早闭（multi-suture craniosynostosis）。治疗方案需根据其引流的静脉血量制定，此类病例大多为优势型静脉窦，无法实施闭塞手术。术前明确诊断优势型颅骨骨膜窦对制定合理手术方案至关重要，因术中必须保留该静脉窦以避免脑静脉梗死。