Mondel Prabath Kumar, Anand Sunanda, Limaye Uday S
Department of Interventional Neuroradiology, Lilavati Hospital and Research Centre, A-791, Bandra Reclamation, Bandra(W), Mumbai, 400 050, Maharashtra, India,
Cardiovasc Intervent Radiol. 2015 Aug;38(4):1015-20. doi: 10.1007/s00270-014-0983-1. Epub 2014 Sep 12.
Crouzon's syndrome is the commonest variety of syndromic craniosynostosis. Life-threatening ear bleed due to ruptured jugular venous diverticulum in Crouzon's syndrome has not been described previously. In patients with syndromic craniosynostosis, definitive repair of jugular diverticulum by open surgery is fraught with high risk of bleeding, poor functional outcomes, and even death. A 24-year-old woman with Crouzon's syndrome presented with conductive hearing loss and recurrent episodes of torrential bleeding from her left ear. On computed tomography, a defect in the roof of jugular fossa containing jugular venous diverticulum immediately inferior to the bony external auditory canal was seen. The clinical presentation, imaging features, and endovascular management of Crouzon's syndrome due to a ruptured jugular venous diverticulum is described.
克鲁宗综合征是综合征性颅缝早闭最常见的类型。此前尚未有关于克鲁宗综合征患者因颈静脉憩室破裂导致危及生命的耳部出血的描述。在患有综合征性颅缝早闭的患者中，通过开放手术对颈静脉憩室进行确定性修复存在出血风险高、功能预后差甚至死亡的问题。一名患有克鲁宗综合征的24岁女性出现传导性听力损失，左耳反复出现大量出血。计算机断层扫描显示，在骨性外耳道正下方的颈静脉窝顶部有一个包含颈静脉憩室的缺损。本文描述了因颈静脉憩室破裂导致的克鲁宗综合征的临床表现、影像学特征及血管内治疗。
