Venous Congestive Myelopathy Due to Chronic Inferior Vena Cava Thrombosis Treated with Endovascular Stenting: Case Report and Review of the Literature

Venous Congestive Myelopathy due to Chronic Inferior Vena Cava Thrombosis Treated with Endovascular Stenting: Case Report and Review of the Literature

1 2 3 4 Diego Z. Carvalho, MD , Joshua D. Hughes, MD , Greta B. Liebo, MD , Emily C. Bendel, MD , 4 1 Haraldur Bjarnason, MD , and James P. Klaas, MD 1Department of Neurology, Mayo Clinic, Rochester, MN, USA. 2Department of Neurosurgery, Mayo Clinic, Rochester, MN, USA 3Department of Neuroradiology, Mayo Clinic, Rochester, MN, USA 4Department of Vascular & Interventional Radiology, Mayo Clinic, Rochester, MN, USA Journal of Vascular and Interventional Neurology, Vol. 8 Abstract Authors have no conflict of interest to disclose. Objective—Impaired inferior vena cava (IVC) outflow can lead to collateralization of blood to the valveless epidural venous plexus, causing epidural venous engorgement and venous congestion. Herein we describe a case of chronic IVC thrombosis presenting as venous congestive myelopathy treated with angioplasty and endovascular stenting. The pathophysiological mechanisms of cord injury are hypothesized, and IVC stenting application is evaluated. Methods—Case report and review of the literature. Results—IVC outflow obstruction has only rarely been associated with neurologic dysfunction, with reports of lumbosacral nerve root compression in the cases of IVC agenesis, compression, or occlusion. Although endovascular angioplasty with stenting is emerging as a leading treatment option for chronic IVC thrombosis, its use to treat neurologic complications is limited to one case report for intractable sciatica. Our case is the first description of IVC thrombosis presenting with venous congestive myelopathy, and treated successfully with IVC stenting. Conclusion—Venous congestive myelopathy should be seen as a broader clinical condition, including not only typical dural arteriovenous fistulas, but also disorders of venous outflow. Therefore, identifying a rare, but potentially treatable, etiology is important to avoid permanent neurologic deficits. IVC stenting is proposed as a novel and effective treatment approach.

Keywords Myelopathy; Inferior vena cava; Thrombosis; Endovascular; Stenting

Introduction Venous hypertension from inferior vena cava (IVC) out- dural venous engorgement and venous congestion (Fig- flow obstruction causes increased blood flow into the ure 1). collateral azygous–hemiazygous system and lumbar-lat- Rarely, abnormalities of venous return through the IVC eral sacral venous plexus. This venous network commu- have been associated with lumbosacral radiculopathy, nicates with the epidural venous plexus, providing opti- presenting as sciatica [1,2], neurogenic claudication [3– mal venous drainage of the spinal cord in physiological 5], and cauda equina syndrome [6–10] due to IVC agen- circumstances. However, as the epidural venous plexus esis [6,10] compression by pregnant uterus [1] or malig- is destitute of valves, outflow obstruction leads to epi- nancy [1,3], as well as occlusion due to stenosis [1,2] or

Published February, 2015. All Rights Reserved by JVIN. Unauthorized reproduction of this article is prohibited Corresponding Author: Diego Z. Carvalho, MD. Department of Neurology Mayo Clinic 200 First Street SW Rochester, MN 55905 car- [email protected] Phone: 507-538-0138 Carvalho et al. 50 Journal of Vascular and Interventional Neurology, Vol. 8

Figure 1. Spinal Venous Drainage. Α: in the setting of normal venous drainage, blood from the spinal cord passes from the capillaries into the intrinsic venous system, the extrinsic (pial) veins, and then exits the dura via the valveless radicular veins. It subsequently joins with the extradural (vertebral) system and is carried away by efferent veins into the thoracic and lumbar intervertebral veins, finally reaching the azygous system and IVC, respectively (see inset). B: in the setting of IVC occlusion, venous drainage from the lower extremities, as well as the abdomen and pelvis, is redirec- ted into the azygous system by way of paravertebral collaterals and the vertebral plexus. the vertebral plexus becomes engorged. Transmission of elevated venous pressure into the valveless radicular veins may result in congestive myelopathy due to slowed, static, or retrograde flow.

thrombosis [1,4,7–9]. Signs of myelopathy in IVC dis- athy (VCM) is hypothesized as the primary pathophy- ease have been described only once, in a female patient siological mechanism underlying this condition. who presented with spastic monoplegia due to IVC compression from a pregnant uterus [11]. Neural foramen Case report and spinal canal compression secondary to engorged epidural and paravertebral collaterals have been pro- A 20-year-old male with no significant past medical his- posed as the major pathophysiological mechanism in tory, apart from delayed umbilical cord healing, presen- radicular involvement [1,2,12,13]. ted to our institution with urinary retention and bilateral lower extremity weakness (right worse than left). Four Endovascular angioplasty and stenting is a treatment days prior to admission, while digging a hole for a pool, option in some cases of IVC thrombosis. However, to he developed sudden midline low back pain, which the best of our knowledge, there has been only one case worsened with movement and straining. By the next where chronic IVC thrombosis with neurologic presen- day, he could barely walk. He noticed gradual ascending tation (intractable sciatica) was treated successfully with numbness and tingling that affected his whole body endovascular stenting [2]. Herein we report the novel below the waist. He also reported constipation and case of a patient with conus medullaris syndrome pre- numbness over the perineum when he wiped himself. senting as a complication of chronic IVC thrombosis, successfully managed with endovascular stenting. As Neurologic examination was significant for mildly opposed to external compression of nerve roots or spine reduced strength in lower extremities. This was best by epidural venous plexus, venous congestive myelop- appreciated when the patient attempted to walk on his 51 Journal of Vascular and Interventional Neurology, Vol. 8 Figure 2. A: Sagittal T2 weighted images demonstrate nonspecific T2 hyperintensity through the length of the thoracic spine, particularly inferiorly. B: Axial T2 weighted images through the lumbar spine demonstrate engorgement of the anterior internal venous plexus in the epidural space (white arrows) and basivertebral plexus (black arrow). C: Axial T2 weighted images show that the signal hyperintensity involves the central cord in the characteristic “snake eye” or “owl eye” appearance, representing injury of the gray matter. D: Postcontrast sagittal T1 images again demonstrate prominent epidural flow voids from the engorged spinal venous plexuses.

“tip-toes.” The right lower extremity was weaker than cular intervention was performed on the fourth day of his left, and affected muscle groups included the iliop- hospitalization. Briefly, catheterization from the right soas, hamstrings, anterior tibialis, and gastrocnemius common femoral vein and right internal jugular led to and soleus. Gait was wide-based. Reflexes were hyper- the intrahepatic IVC, and a stump was noted, which active in lower extremity with bilateral Babinski signs. appeared fibrotic in nature. The IVC was dilated with Sensory was abnormal to pinprick in the perineal region 10-, 14-, and 18-mm balloons. Three 22 mm × 45 mm only. Patient was unable to void and required urinary wall stents were placed from the bifurcation of the com- catheterization, draining initially 2.5 L. Clinically his mon iliac veins up to the upper portion of the IVC. A exam was most consistent with conus medullaris syn- follow up venogram revealed preferential flow in the drome. stented IVC (Figure 3C). The procedure was well toler- ated, and the patient was started on systemic anticoagu- MRI of the spine revealed epidural venous engorgement, lation. T2 signal hyperintensity through the length of the thoracic spine, and signs of spinal cord injury with distinct Over the next two days, the patient’s strength gradually central gray matter involvement (Figure 2A and C). No improved. His lower back pain resolved, and weakness signs of compression of the spinal cord or nerve roots was undetectable on examination. He reported that the were identified. DWI and ADC map showed no restric- lower extremity numbness also improved, roughly 80% tion to suggest acute infarct. Postcontrast sagittal T1 normal at time of discharge, and he was able to void images showed no associated enhancement, suggesting a spontaneously. After 3 weeks, patient reported complete more chronic process. MRA of the thoracolumbar spine resolution of his neurologic symptoms. demonstrated normal opacification of the paraspinal and spinal arteries without shunting to or enlargement of Discussion intradural spinal veins to suggest a spinal dural AVF. Contrast-enhanced CT of abdomen and pelvis revealed To the best of our knowledge, this is the first reported extensive retroperitoneal collateralization and intrahe- case of myelopathy due to IVC occlusion. The cause of patic IVC occlusion (Figure 3A and B). Work up was this patient’s IVC occlusion is not entirely clear. The negative for thrombophilias. history of delayed healing of umbilical cord stump, fibrotic occlusion, and age of presentation, suggests a The patient was initially given methylprednisone 1 g IV congenital thrombosis of the IVC. Most congenital IVC and mannitol IV on presentation without significant abnormalities remain asymptomatic due to development improvement, although he did report some improvement of collaterals, but collateral flow can become over- in his back pain following mannitol infusion. Endovas- whelmed with aging, leading to venous insufficiency,

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Figure 3. Contrast-enhanced Ct of the abdomen and pelvis demonstrates Α: I V C narrowed and unopacified (black arrow) relative to the hepatic veins and B: large venous varices in the retroperitoneum (white arrows). C: Status post endovascular IVC angioplasty and stent placement, demonstrating preferential flow to the stented IVC.

venous congestion, and epidural venous engorgement. with IVC agenesis, wherein postexercise MRI showed a As neuroimaging did not reveal compression of nerve notable increase in the volume of the venous plexus roots or spinal cord, a mechanism of VCM can be [16]. hypothesized. This conclusion is supported with the patient’s clinical improvement following IVC recanali- In the 20 reported cases of patients with neurologic dis- zation and stenting. orders in the setting of IVC involvement, one or more likely etiologies were found in 16 patients. These inclu- Epidural venous congestion can reduce the arteriove- ded IVC agenesis (2 cases) [6,10]; and IVC thrombosis nous pressure gradient within the cord, decreasing tissue attributed to oral contraceptive use (6 cases) [1,7,10], perfusion and promoting chronic ischemia. This mecha- pregnancy (3 cases) [1,11], malignancy (2 cases) [1,3], nism is similar to that of spinal epidural arteriovenous Behcet’s disease (1 case) [1], Budd–Chiari syndrome (1 fistulas (AVF) [14,15]. In these cases, multiple small case) [2], and protein C deficiency (1 case) [1]. In cases arterial vessels feed one or more epidural veins, leading of pregnancy, malignancy (1 out of 2), and protein C to epidural venous plexus engorgement, which, after deficiency, treating the etiology with delivery, chemo- reaching a significant pressure gradient, can cause con- therapy, and anticoagulation, respectively, was associ- gestion or even reflux into the radiculomeningeal and ated with good outcomes. In other cases of acute IVC then perimedullary veins. In our case, the mechanism of thrombosis, other treatment approaches were performed venous congestion is different from that of typical dural with good outcomes reported, including venous throm- AVFs, so the classic imaging characteristics of VCM bectomy (3 cases) [1,7,9]; intracatheter thrombolysis with serpentine enhancing veins on the cord surface with balloon angioplasty (1 case) [7]; and systemic anti- (dilated perimedullary veins) were not present. Increased coagulation alone (10 cases) [1,8,10]. In the literature, intrathoracic pressure, which occurs with exertion, such there are only two reports of patients with chronic IVC as digging holes, could acutely worsen venous return, occlusion presenting with neurologic symptoms. In one and force pooling of blood into an already overwhelmed case of IVC agenesis, a patient presented acutely with epidural plexus, increasing congestion or reflux to peri- cauda equina syndrome and underwent laminectomy, medullary veins with ensuing spinal cord ischemia. This bilateral foraminotomies, and coagulation of epidural is an accord with Kamerath and Morgan’s report of veins [6]. In another case, a patient with Budd–Chiari exercise-induced lower extremity numbness in a patient syndrome presented with intractable sciatica and under-

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