Esfahani Darian R, Alaraj Ali, Birk Daniel M, Thulborn Keith R, Charbel Fady T
Department of Neurosurgery, University of Illinois at Chicago, Chicago, Illinois, USA.
Center for Magnetic Resonance Research, University of Illinois at Chicago, Chicago, Illinois, USA.
World Neurosurg. 2018 Jan;109:129-133. doi: 10.1016/j.wneu.2017.09.106. Epub 2017 Sep 23.
Venous outflow obstructions are rare anatomic findings that can appear with symptoms of elevated intracranial pressure, including headache and vision loss, and can be mistaken for more common diagnoses, such as idiopathic intracranial hypertension (IIH) or cerebral venous sinus thrombosis (CVST). Although venous outflow obstructions have been reported in rare bone dysplasias and congenital abnormalities, to date they have not been reported in renal osteodystrophy (ROD), a relatively common disorder seen in patients with chronic kidney disease.
In this case, the authors describe a patient with marked intracranial hypertension from jugular foramen stenosis secondary to ROD. After diagnosis by CT and magnetic resonance venography, catheter venography confirmed an osseus band around the left jugular bulb, and a 40-mm Hg pressure gradient across the stenotic foramen. The patient subsequently underwent ventriculoperitoneal shunting and optic nerve sheath fenestration with symptom improvement. The postoperative course was significant for development of CVST, necessitating treatment.
This report reviews the presentation, pathology, and neurosurgical treatment of patients with ROD and venous outflow obstructions, and explores the differential diagnoses of outflow obstructions, IIH, and CVST. To our knowledge, this is the first report of intracranial hypertension from jugular foramen stenosis secondary to renal osteodystrophy.
静脉流出道梗阻是一种罕见的解剖学发现，可出现颅内压升高的症状，包括头痛和视力丧失，并且可能被误诊为更常见的疾病，如特发性颅内高压（IIH）或脑静脉窦血栓形成（CVST）。虽然静脉流出道梗阻已在罕见的骨发育异常和先天性异常中报道，但迄今为止，尚未在肾性骨营养不良（ROD）中报道，肾性骨营养不良是慢性肾病患者中相对常见的疾病。
在本病例中，作者描述了一名因ROD继发颈静脉孔狭窄而患有明显颅内高压的患者。通过CT和磁共振静脉造影诊断后，导管静脉造影证实左颈静脉球周围有一条骨带，狭窄孔两端的压力梯度为40 mmHg。患者随后接受了脑室腹腔分流术和视神经鞘开窗术，症状得到改善。术后病程中出现了CVST，需要进行治疗。
本报告回顾了ROD和静脉流出道梗阻患者的临床表现、病理和神经外科治疗，并探讨了流出道梗阻、IIH和CVST的鉴别诊断。据我们所知，这是第一例关于肾性骨营养不良继发颈静脉孔狭窄导致颅内高压的报告。
