Do Angelique Sao-Mai, Kapurch Joseph, Kumar Ravi, Port John, Miller Jeffrey W, Van Gompel Jamie J
Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA; Case Western Reserve University, School of Medicine, Cleveland, Ohio, USA.
Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA.
World Neurosurg. 2017 Dec;108:998.e7-998.e16. doi: 10.1016/j.wneu.2017.09.073. Epub 2017 Sep 29.
Spinal dural arteriovenous fistulas (DAVFs) have diverse presentations. Magnetic resonance imaging (MRI) reveals spinal cord swelling in only 45% to 74% of cases. We present an unusual case of a 57-year old man with a craniocervical junction DAVF in which the edema appeared in the thoracolumbar region, skipping the cervical spinal cord.
A case report and literature review from a tertiary referral center are provided.
In our patient, symptoms progressed over 3 months from low back and radicular pain to weakness, saddle anesthesia, and urinary retention. MRI showed T2 hyperintensity from T3-4 to the conus medullaris. The results from initial brain and spine angiograms were negative. Repeated angiography with the patient under anesthesia revealed a DAVF in the craniocervical junction composed of a cervical branch arising from the right vertebral artery and entering the nidus at the foramen magnum. A prominent intradural draining vein extended inferiorly along the posterior aspect of the cervical spinal cord. The patient was taken to the operating room, where the extradural vertebral artery was dissected and a suboccipital craniectomy was performed. After the dura was opened, the arterialized vein was visualized and clipped. Somatosensory evoked potentials and motor evoked potentials remained at baseline throughout the occlusion. The dura and fistula were removed en bloc, and the draining vein was confirmed to lose flow on indocyanine green. Postoperatively, the patient's symptoms completely resolved, and he experienced full strength in the lower extremities and perineal sensation. A review of 19 cases of DAVF at the craniocervical junction with cord edema shows that >90% involve the brainstem or cervical cord. Only 1 other patient had a similar "skip lesion," in which the cord edema first appeared in the thoracic cord.
This rare presentation highlights the importance of diagnostic persistence when unexplained cord findings are seen on MRI. Repeated angiography and the inclusion of proximal vascular lesions may be considered in the evaluation of isolated thoracic edema.
脊髓硬脊膜动静脉瘘（DAVF）有多种表现形式。磁共振成像（MRI）仅在45%至74%的病例中显示脊髓肿胀。我们报告一例不寻常的病例，一名57岁男性患有颅颈交界区DAVF，其水肿出现在胸腰段，而颈髓未受累。
提供一份来自三级转诊中心的病例报告及文献综述。
在我们的患者中，症状在3个月内从腰背部和神经根性疼痛进展为无力、鞍区麻醉和尿潴留。MRI显示从T3 - 4至脊髓圆锥T2高信号。最初的脑和脊髓血管造影结果为阴性。在麻醉下对患者进行重复血管造影显示颅颈交界区有一个DAVF，由右椎动脉发出的一个颈部分支在枕骨大孔处进入瘘口。一条明显的硬脊膜内引流静脉沿颈髓后方下行。患者被送往手术室，在那里解剖硬膜外椎动脉并进行枕下颅骨切除术。打开硬脑膜后，可见动脉化静脉并予以夹闭。在整个闭塞过程中，体感诱发电位和运动诱发电位保持在基线水平。将硬脑膜和瘘管整块切除，通过吲哚菁绿证实引流静脉失去血流。术后，患者症状完全缓解，下肢肌力和会阴感觉完全恢复。对19例伴有脊髓水肿的颅颈交界区DAVF病例的回顾显示，>90%累及脑干或颈髓。只有另外1例患者有类似的“跳跃性病变”，其脊髓水肿首先出现在胸髓。
这种罕见的表现突出了在MRI上出现无法解释的脊髓表现时持续进行诊断的重要性。在评估孤立的胸段水肿时，可考虑重复血管造影并检查近端血管病变。
