Case Report Cerebral Infarction due to Central Vein Occlusion in a Hemodialysis Patient Vikram Prasad, MD, Shahine Baghai, MD, Dheeraj Gandhi, MD, Fred Moeslein, MD, PhD, Gaurav Jindal, MD From the Department of Diagnostic / Interventional Radiology and Nuclear Medicine, University of Maryland Medical Center, 22 S. Greene St., Baltimore, Maryland, USA.
ABSTRACT Venous congestive encephalopathy is a rare complication of central venous occlusion in hemodialysis patients with upper extremity dialysis created shunts. We describe the clinical presentation and endovascular management of an end-stage renal disease patient with a left upper extremity arteriovenous graft who developed intracranial venous hypertension, left-sided subdural and subarachnoid intracranial hemorrhage, and left-sided cerebral infarcts related to a left brachiocephalic vein occlusion. Keywords: Venous congestive encephalopathy, cerebral infarction, hemodialysis. Acceptance: Received September 4, 2013, and in revised form May 20, 2014. Accepted for publication June 1, 2014. Correspondence: Address correspondence to Gaurav Jindal, MD, Department of Diagnostic / Interventional Radiology and Nuclear Medicine University of Maryland Medical Center 22 S. Greene St. Baltimore, Maryland, USA. E-mail: [email protected]. Conflicts of Interest: None. J Neuroimaging 2015;25:494-496. DOI: 10.1111/jon.12152
Introduction Venous congestive encephalopathy (VCE) is a rare complication of central venous occlusion in hemodialysis patients with upper extremity dialysis created shunts.1 It can present with a variety of nonspecific neurological signs and symptoms, mimicking a range of differing neurological diagnoses.2 As a result, diagnosis is often delayed and can lead to irreversible neurological deficits from cerebral infarction. We report an institutional review board-approved case of an end-stage renal disease (ESRD) patient with a left upper extremity arteriovenous (AV) graft who developed a left brachiocephalic vein occlusion, resulting in flow reversal within the left internal jugular vein (IJV). Intracranial venous hypertension ensued leading to multiple left-sided cerebral infarcts in addition to left-sided subdural and subarachnoid intracranial hemorrhage.
Case Report A 47-year-old African American gentleman with ESRD developed altered mental status. A left subdural hematoma was discovered and successfully treated by surgical evacuation. The patient’s mental status failed to improve, and he was transferred to our institution for further evaluation. On admission, general examination demonstrated an AV graft in the left upper extremity with positive thrill and bruit. Subtle left upper extremity and facial swelling was also present in retrospect but not seen on initial physical exam. Cranial nerve examination revealed diminished reactivity of the left pupil, horizontal nystagmus,
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diminished corneal and gag reflexes, and symmetric bilateral facial muscles. Motor examination revealed right hypertonia and withdrawal to nail bed pressure in all four extremities, though less briskly in the right upper and right lower extremity. Strength was 1/5 in the left upper and lower extremity and 0/5 in the right upper and lower extremities. Sensation was grossly intact. Magnetic resonance imaging (MRI) of the brain demonstrated the left-sided subdural hematoma and multiple new patchy areas of restricted diffusion throughout the left cerebral hemisphere in multiple vascular territories, compatible with infarctions (Fig 1A–B). Time-of-flight (TOF) noncontrast magnetic resonance angiography (MRA) of the head and neck demonstrated arterialized signal intensity similar to arteries in the left IJV extending into the left sigmoid and transverse sinuses, left superior and inferior petrosal venous sinuses and several left parietal cortical veins, findings compatible with flow reversal (Fig 2). Gadolinium enhanced MR venography over the head, however, was not obtained at this time. A conventional cerebral angiogram was performed. Delayed, digital subtracted angiographic (DSA) images from a left internal carotid artery (ICA) injection demonstrated venous drainage through the right dural venous sinuses into the right IJV with lack of opacification of the left sigmoid sinus or the left IJV, suggestive of flow reversal or thrombosis of these vessels. A small filling defect in the left transverse sinus was also seen, compatible with thrombus; this was confirmed to represent a
◦ 2014 by the American Society of Neuroimaging C
Fig 1. Axial FLAIR MRI images of the brain demonstrate (A) left sided subdural hemorrhage (thin arrows) and small subarachnoid hemorrhage (block arrow) and (B) abnormal signal intensity throughout multiple vascular territories (open circles) in the left cerebral hemisphere a few days later. These findings are compatible with venous outflow congestion related hemorrhage and parenchymal infarctions. Corresponding diffusion restriction on diffusion weighted imaging (not shown) in these locations was consistent with infarcted brain tissue.
the right IJV (Fig 3B). DSA venography of the head, however, was not obtained at this time. Over the next several days, the patient’s left upper extremity edema improved with decreased hypertonia and improved motor strength throughout the right upper and right lower extremities (3/5 strength bilaterally). Follow-up TOF MRA of the head and neck demonstrated complete interval resolution of the arterialized signal within the left IJV and left dural venous sinuses consistent with restoration of normal flow direction (Fig 4).
Discussion Fig 2. TOF MRA images. Intracranial vasculature demonstrates arterialized signal abnormality within the left IJV (thin arrow), left sigmoid sinus (thick arrow) and left transverse sinus (medium arrow) suggestive of flow reversal.
small focal thrombus demonstrated by focal T1-weighted signal abnormality on MRI. Given the constellation of symptoms and distribution of infarcts, central venous stenosis was suspected and a left upper extremity fistulogram was performed. DSA images demonstrated occlusion of the left brachiocephalic vein with brisk reflux of contrast into the left IJV and cross-filling of the contralateral IJV via numerous cervical collateral veins (Fig 3A). Percutaneous venoplasty and endovascular stent repair of the brachiocephalic vein was performed, resulting in improved central venous flow. On immediate post-stent venography, only mild contrast reflux into the left IJV was noted with resolution of cross filling into
Chronic hemodialysis patients are at higher risk than others for developing central venous stenosis and occlusion due to chronic indwelling catheters and arterialized blood flow within the central veins from dialysis created shunts.3,4 Symptoms of central venous stenosis include ipsilateral upper extremity edema, dilation of the superficial veins, and pain. Rarely, neurological signs and symptoms secondary to intracranial venous congestion may be a manifestation of central vein occlusion.1 Brachiocephalic vein stenosis or occlusion in particular has been shown to be associated with flow reversal within the left IJV, facial edema, and rarely cerebral venous hypertension.5,6 Signs and symptoms of VCE are nonspecific and may be reversible if the underlying cause is treated early.1,5,6 A delay in diagnosis, however, can lead to parenchymal infarction or hemorrhage.7,8 Cases such as ours are not commonly seen given that, in the early stages of central venous occlusion, collateral blood flow into superficial and deep veins of the chest and neck may develop prior to venous reflux into intracranial
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Fig 3. Left upper extremity fistulogram. (A) Pre-stent repair DSA image demonstrates level of the stenosis (short thin arrow) with reflux of contrast into the left IJV (long thin arrow) and cross-filling of the right IJV via numerous cervical venous collaterals (short thick arrow) with drainage into the SVC (long thick arrow). (B) Post-stent repair DSA image shows contrast opacification within the stent (long arrow) and contrast entering the SVC with only mild residual reflux of contrast into the proximal left IJV (short arrow) and no evidence of cross-filling of the right IJV.
would not likely have accounted for the extensive cerebral infarcts throughout the left cerebral hemisphere.
Conclusion VCE is a rare and potentially life-threatening condition in a dialysis patient with a dialysis created shunt and central venous occlusion. Clinicians treating these patients should maintain a high index of suspicion for this condition in a patient presenting with neurological symptoms, since it may be treated with balloon angioplasty and stent repair or surgical ligation of the shunt.
References
Fig 4. Three days poststent repair. TOF MRA of the head demonstrates complete resolution of the previously seen arterialized signal abnormality in the expected region of the left dural venous sinuses (arrows).
veins. Rapid development of central venous stenosis or occlusion, however, may not allow for the development of collateral venous channels, which could result in intracranial shunting and acute neurological manifestations.5 It is also postulated that cerebral venous reflux can be tolerated if alterative intracranial routes of drainage are present via the dural sinuses into the contralateral IJV and/or if cerebral venous hypertension is mild, although these ideas have not been proven.8,9 Although gadolinium enhanced MR venography and DSA venography over the head were not obtained in this patient, the complete resolution of arterialized signal intensity in the left dural venous sinuses after angioplasty of the central venous occlusion suggests that the left-sided cerebral infarcts were due to both retrograde flow and partial venous thrombosis. On its own, the small focal thrombus within the left transverse sinus
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1. Hartmann A, Mast H, Stapf C, et al. Peripheral hemodialysis shunt with intracranial venous congestion. Stroke 2001;32(12):2945-2946. 2. Samaniego E, Abrams K, Dabus G, et al. Severe venous congestive encephalopathy secondary to a dialysis arteriovenous graft. J Neurointerv Surg 2012;5(5):e37. 3. Kundu S. Central venous disease in hemodialysis patients: prevalence, etiology and treatment. J Vasc Access 2010;11(1):1-7. 4. Oguzkurt L, Tercan F, Yildirim S, et al. Central venous stenosis in haemodialysis patients without a previous history of catheter placement. Eur J Radiol 2005;55(2):237-242. 5. Nishimoto H, Ogasawara K, Miura K, et al. Acute intracranial hypertension due to occlusion of the brachiocephalic vein in a patient undergoing hemodialysis. Cerebrovasc Dis 2005;20(3):207208. 6. Eames P, Senthil L, Thomas A, et al. Brachiocephalic vein stenosis in association with ipsilateral hyperdynamic brachio-basilic fistula causing ipsilateral facial swelling and contralateral papilledema and visual loss. A case report. Interv Neuroradiol 2010;16(3):322-325. 7. Nishijima H, Tomiyama M, Haga R, et al. Venous cerebral infarction in a patient with peripheral hemodialysis shunt and occlusion of the left brachiocephalic vein. J Stroke Cerebrovasc Dis 2011;20(4):381-383. 8. Saha MK, Hamieh T, Larkin B, et al. Cerebral hemorrhage due to internal jugular vein stenosis in a hemodialysis patient. Clin Exp Nephrol 2011;16(2):345-349. 9. Schaller B, Graf R. Cerebral Venous Infarction: The pathophysiological concept. Cerebrovasc Dis 2004;18(3):179-188.
Copyright of Journal of Neuroimaging is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
ABSTRACT Venous congestive encephalopathy is a rare complication of central venous occlusion in hemodialysis patients with upper extremity dialysis created shunts. We describe the clinical presentation and endovascular management of an end-stage renal disease patient with a left upper extremity arteriovenous graft who developed intracranial venous hypertension, left-sided subdural and subarachnoid intracranial hemorrhage, and left-sided cerebral infarcts related to a left brachiocephalic vein occlusion. Keywords: Venous congestive encephalopathy, cerebral infarction, hemodialysis. Acceptance: Received September 4, 2013, and in revised form May 20, 2014. Accepted for publication June 1, 2014. Correspondence: Address correspondence to Gaurav Jindal, MD, Department of Diagnostic / Interventional Radiology and Nuclear Medicine University of Maryland Medical Center 22 S. Greene St. Baltimore, Maryland, USA. E-mail: [email protected]. Conflicts of Interest: None. J Neuroimaging 2015;25:494-496. DOI: 10.1111/jon.12152
Introduction Venous congestive encephalopathy (VCE) is a rare complication of central venous occlusion in hemodialysis patients with upper extremity dialysis created shunts.1 It can present with a variety of nonspecific neurological signs and symptoms, mimicking a range of differing neurological diagnoses.2 As a result, diagnosis is often delayed and can lead to irreversible neurological deficits from cerebral infarction. We report an institutional review board-approved case of an end-stage renal disease (ESRD) patient with a left upper extremity arteriovenous (AV) graft who developed a left brachiocephalic vein occlusion, resulting in flow reversal within the left internal jugular vein (IJV). Intracranial venous hypertension ensued leading to multiple left-sided cerebral infarcts in addition to left-sided subdural and subarachnoid intracranial hemorrhage.
Case Report A 47-year-old African American gentleman with ESRD developed altered mental status. A left subdural hematoma was discovered and successfully treated by surgical evacuation. The patient’s mental status failed to improve, and he was transferred to our institution for further evaluation. On admission, general examination demonstrated an AV graft in the left upper extremity with positive thrill and bruit. Subtle left upper extremity and facial swelling was also present in retrospect but not seen on initial physical exam. Cranial nerve examination revealed diminished reactivity of the left pupil, horizontal nystagmus,
494
Copyright
diminished corneal and gag reflexes, and symmetric bilateral facial muscles. Motor examination revealed right hypertonia and withdrawal to nail bed pressure in all four extremities, though less briskly in the right upper and right lower extremity. Strength was 1/5 in the left upper and lower extremity and 0/5 in the right upper and lower extremities. Sensation was grossly intact. Magnetic resonance imaging (MRI) of the brain demonstrated the left-sided subdural hematoma and multiple new patchy areas of restricted diffusion throughout the left cerebral hemisphere in multiple vascular territories, compatible with infarctions (Fig 1A–B). Time-of-flight (TOF) noncontrast magnetic resonance angiography (MRA) of the head and neck demonstrated arterialized signal intensity similar to arteries in the left IJV extending into the left sigmoid and transverse sinuses, left superior and inferior petrosal venous sinuses and several left parietal cortical veins, findings compatible with flow reversal (Fig 2). Gadolinium enhanced MR venography over the head, however, was not obtained at this time. A conventional cerebral angiogram was performed. Delayed, digital subtracted angiographic (DSA) images from a left internal carotid artery (ICA) injection demonstrated venous drainage through the right dural venous sinuses into the right IJV with lack of opacification of the left sigmoid sinus or the left IJV, suggestive of flow reversal or thrombosis of these vessels. A small filling defect in the left transverse sinus was also seen, compatible with thrombus; this was confirmed to represent a
◦ 2014 by the American Society of Neuroimaging C
Fig 1. Axial FLAIR MRI images of the brain demonstrate (A) left sided subdural hemorrhage (thin arrows) and small subarachnoid hemorrhage (block arrow) and (B) abnormal signal intensity throughout multiple vascular territories (open circles) in the left cerebral hemisphere a few days later. These findings are compatible with venous outflow congestion related hemorrhage and parenchymal infarctions. Corresponding diffusion restriction on diffusion weighted imaging (not shown) in these locations was consistent with infarcted brain tissue.
the right IJV (Fig 3B). DSA venography of the head, however, was not obtained at this time. Over the next several days, the patient’s left upper extremity edema improved with decreased hypertonia and improved motor strength throughout the right upper and right lower extremities (3/5 strength bilaterally). Follow-up TOF MRA of the head and neck demonstrated complete interval resolution of the arterialized signal within the left IJV and left dural venous sinuses consistent with restoration of normal flow direction (Fig 4).
Discussion Fig 2. TOF MRA images. Intracranial vasculature demonstrates arterialized signal abnormality within the left IJV (thin arrow), left sigmoid sinus (thick arrow) and left transverse sinus (medium arrow) suggestive of flow reversal.
small focal thrombus demonstrated by focal T1-weighted signal abnormality on MRI. Given the constellation of symptoms and distribution of infarcts, central venous stenosis was suspected and a left upper extremity fistulogram was performed. DSA images demonstrated occlusion of the left brachiocephalic vein with brisk reflux of contrast into the left IJV and cross-filling of the contralateral IJV via numerous cervical collateral veins (Fig 3A). Percutaneous venoplasty and endovascular stent repair of the brachiocephalic vein was performed, resulting in improved central venous flow. On immediate post-stent venography, only mild contrast reflux into the left IJV was noted with resolution of cross filling into
Chronic hemodialysis patients are at higher risk than others for developing central venous stenosis and occlusion due to chronic indwelling catheters and arterialized blood flow within the central veins from dialysis created shunts.3,4 Symptoms of central venous stenosis include ipsilateral upper extremity edema, dilation of the superficial veins, and pain. Rarely, neurological signs and symptoms secondary to intracranial venous congestion may be a manifestation of central vein occlusion.1 Brachiocephalic vein stenosis or occlusion in particular has been shown to be associated with flow reversal within the left IJV, facial edema, and rarely cerebral venous hypertension.5,6 Signs and symptoms of VCE are nonspecific and may be reversible if the underlying cause is treated early.1,5,6 A delay in diagnosis, however, can lead to parenchymal infarction or hemorrhage.7,8 Cases such as ours are not commonly seen given that, in the early stages of central venous occlusion, collateral blood flow into superficial and deep veins of the chest and neck may develop prior to venous reflux into intracranial
Prasad et al: Cerebral Infarction due to Central Vein Occlusion
495
Fig 3. Left upper extremity fistulogram. (A) Pre-stent repair DSA image demonstrates level of the stenosis (short thin arrow) with reflux of contrast into the left IJV (long thin arrow) and cross-filling of the right IJV via numerous cervical venous collaterals (short thick arrow) with drainage into the SVC (long thick arrow). (B) Post-stent repair DSA image shows contrast opacification within the stent (long arrow) and contrast entering the SVC with only mild residual reflux of contrast into the proximal left IJV (short arrow) and no evidence of cross-filling of the right IJV.
would not likely have accounted for the extensive cerebral infarcts throughout the left cerebral hemisphere.
Conclusion VCE is a rare and potentially life-threatening condition in a dialysis patient with a dialysis created shunt and central venous occlusion. Clinicians treating these patients should maintain a high index of suspicion for this condition in a patient presenting with neurological symptoms, since it may be treated with balloon angioplasty and stent repair or surgical ligation of the shunt.
References
Fig 4. Three days poststent repair. TOF MRA of the head demonstrates complete resolution of the previously seen arterialized signal abnormality in the expected region of the left dural venous sinuses (arrows).
veins. Rapid development of central venous stenosis or occlusion, however, may not allow for the development of collateral venous channels, which could result in intracranial shunting and acute neurological manifestations.5 It is also postulated that cerebral venous reflux can be tolerated if alterative intracranial routes of drainage are present via the dural sinuses into the contralateral IJV and/or if cerebral venous hypertension is mild, although these ideas have not been proven.8,9 Although gadolinium enhanced MR venography and DSA venography over the head were not obtained in this patient, the complete resolution of arterialized signal intensity in the left dural venous sinuses after angioplasty of the central venous occlusion suggests that the left-sided cerebral infarcts were due to both retrograde flow and partial venous thrombosis. On its own, the small focal thrombus within the left transverse sinus
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Journal of Neuroimaging Vol 25 No 3 May 2015
1. Hartmann A, Mast H, Stapf C, et al. Peripheral hemodialysis shunt with intracranial venous congestion. Stroke 2001;32(12):2945-2946. 2. Samaniego E, Abrams K, Dabus G, et al. Severe venous congestive encephalopathy secondary to a dialysis arteriovenous graft. J Neurointerv Surg 2012;5(5):e37. 3. Kundu S. Central venous disease in hemodialysis patients: prevalence, etiology and treatment. J Vasc Access 2010;11(1):1-7. 4. Oguzkurt L, Tercan F, Yildirim S, et al. Central venous stenosis in haemodialysis patients without a previous history of catheter placement. Eur J Radiol 2005;55(2):237-242. 5. Nishimoto H, Ogasawara K, Miura K, et al. Acute intracranial hypertension due to occlusion of the brachiocephalic vein in a patient undergoing hemodialysis. Cerebrovasc Dis 2005;20(3):207208. 6. Eames P, Senthil L, Thomas A, et al. Brachiocephalic vein stenosis in association with ipsilateral hyperdynamic brachio-basilic fistula causing ipsilateral facial swelling and contralateral papilledema and visual loss. A case report. Interv Neuroradiol 2010;16(3):322-325. 7. Nishijima H, Tomiyama M, Haga R, et al. Venous cerebral infarction in a patient with peripheral hemodialysis shunt and occlusion of the left brachiocephalic vein. J Stroke Cerebrovasc Dis 2011;20(4):381-383. 8. Saha MK, Hamieh T, Larkin B, et al. Cerebral hemorrhage due to internal jugular vein stenosis in a hemodialysis patient. Clin Exp Nephrol 2011;16(2):345-349. 9. Schaller B, Graf R. Cerebral Venous Infarction: The pathophysiological concept. Cerebrovasc Dis 2004;18(3):179-188.
Copyright of Journal of Neuroimaging is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
