Clinical Imaging 38 (2014) 884–887
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
Central venous occlusion mimics carotid cavernous fistula: a case report and review of the literature☆,☆☆ Gayle R. Salama a,⁎,1, Joaquim M. Farinhas a,1, David D. Pasquale a,1, Christian Wertenbaker b,1, Jacqueline A. Bello a,1 a
Department of Neuroradiology, Montefiore Medical Center, 111 East 210th St., Bronx, NY 10647, USA Department of Ophthalmology, Montefiore Medical Center, 111 East 210th St., Bronx, NY 10647, USA
b
a r t i c l e
i n f o
Article history: Received 23 February 2014 Received in revised form 21 June 2014 Accepted 30 June 2014 Keywords: Central venous occlusion Carotid cavernous fistula Digital subtraction angiography Recanalization Interventional radiology
a b s t r a c t A patient presented with signs and symptoms of a left carotid cavernous fistula (CCF). Computed tomography angiography confirmed filling of the cavernous sinus in the arterial phase. Cerebral digital subtraction angiography demonstrated no evidence of CCF. The workup, diagnosis, and treatment of this patient are discussed, and the literature is reviewed. Published by Elsevier Inc.
1. Case description A 40-year-old female with diabetes mellitus type 1, hypertension, hypothyroidism, end-stage renal disease on hemodialysis, and binocular total blindness from proliferative diabetic retinopathy and tractional retinal detachments presented with 3three days of worsening “whooshing in the left ear” and painful “bulging” of her left eye. Three months prior to admission, she had noted a “bump” on her left forehead, which progressed to left facial edema and nasal congestion. She also noted pulsatile tinnitus if she lay on her left ear and variable pain in the left eye and face for about 4 months. She reported that her symptoms improved during dialysis treatments. Her hemodialysis fistula was in the left upper arm. On the day prior to admission, she was seen by her ophthalmologist, who noted prominent left conjunctival and episcleral vessels and left conjunctival chemosis, and sent her to the emergency department for evaluation of a possible carotid cavernous fistula (CCF). ☆ Advances in Knowledge:1. Retrograde filling of the external and internal jugular veins during the intracapillary phase of a vertebral artery injection may suggest central venous occlusion. ☆☆ Implications for Patient Care:1. Diabetic patients on dialysis with aneurysmal fistulae and signs and symptoms of carotid cavernous fistula (CCF) should be evaluated for central venous occlusions.2. Nephrologists and peripheral interventional radiologists should be consulted earlier in the care of patients with fistula sites and signs and symptoms of CCF because they commonly see central venous occlusions and can facilitate a more rapid diagnosis. ⁎ Corresponding author. Weill Cornell Medical Center–NYPH, 525 East 68th Street, Box 141, New York, NY 10065, USA. E-mail address: [email protected] (G.R. Salama). 1 Tel.:+1 718 798 5449; fax: +1 718 798 5376. http://dx.doi.org/10.1016/j.clinimag.2014.06.018 0899-7071/Published by Elsevier Inc.
On admission, her blood pressure was 142/88. She was in no acute distress. Physical exam was remarkable for the following: conjunctival and episcleral injection and mild proptosis of the left eye, a bruit over the left temple near the eye but not over the eye itself, normal extraocular movements, left-sided eyelid and facial edema extending to the forehead, and decreased sensation over the left face (Fig. 1). There was no left facial weakness and tongue protrusion was midline. Laboratory values were remarkable for blood urea nitrogen and creatinine of 29.0 and 6.4, respectively. Computed tomography angiography (CTA) of the head (Fig. 2) showed asymmetric opacification of the left cavernous sinus, left jugular bulb, left facial vein, and left retromandibular vein, as well as the left internal jugular vein in the arterial phase. The venous plexus within the left masticator space and left parotid space was also asymmetrically prominent. These findings were suspicious for a CCF; however, the left superior ophthalmic vein was neither enlarged nor opacified in the arterial phase of the CTA. Magnetic resonance angiogram (MRA; Fig. 3) showed mildly increased signal in the left cavernous sinus. Intracranial digital subtraction angiography (DSA) showed no evidence of CCF. However, the left vertebral artery injection showed filling of the left internal and external jugular veins in a caudal–cephalad direction during the intracranial capillary phase (Fig. 4) due to reflux of contrast into the left subclavian artery and into the left neck veins via the left arm fistula. Additionally, right and left external carotid artery injections further ruled out a dural CCF. Given the lack of an intracranial cause for a CCF and unexplained caudal-to-cephalad filling of the left neck veins, peripheral venous injections of the upper extremities were performed. The right upper
G.R. Salama et al. / Clinical Imaging 38 (2014) 884–887
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Fig. 1. Photograph of the face showing asymmetric swelling of the left side and of the left eye shows episcleral and conjunctival injection.
extremity venous injection demonstrated normal flow to a patent superior vena cava. Direct injection of the patient's left arm fistula, which was aneurismal, demonstrated complete obstruction of the left proximal brachiocephalic vein (BCV; Fig. 5). Retrograde venous flow was noted first in the left external jugular and facial veins followed by retrograde flow in the left internal jugular vein. Recanalization of the chronic left BCV occlusion was performed using an 18-gauge Colapinto needle. Angioplasty and stenting of the left BCV were performed using a 10×40mm S.M.A.R.T. Stent (Cordis Corporation, Bridgewater, NJ, USA; Fig. 6). Her symptoms improved within 24 h (Fig. 7).
2. Literature review We reviewed the literature conjoining CCF, central venous stenosis or occlusion, and dialysis. To do so, we systematically searched PubMed and the World Wide Web through Google's search engine in January 2014 for studies investigating patients who demonstrate symptoms and imaging findings of a CCF and who are ultimately diagnosed with central venous occlusion. Only two PubMed publications and one conference presentation, independently published,
Fig. 2. Axial contrast-enhanced CT image of the brain demonstrates asymmetric enhancement of the left cavernous sinus (horizontal arrow) adjacent to the left cavernous carotid artery. Opacification of left facial veins (vertical arrow) and left hemi-facial swelling (oblique arrow) are also seen.
satisfied our inclusion criteria of central venous occlusion mimicking CCF clinically and by either CTA or MRA imaging. The first report, by Paksoy et al. [1], described three cases. All three cases were women ranging in age from 48 to 70 years. Presenting symptoms varied among the patients and included vertebrobasilar insufficiency, fluctuating left cephalalgia, and diplopia. Each patient underwent intracranial three-dimensional (3D) time-of-flight (TOF) MRA showing hyperintense vascular signal from the left cavernous sinus to the proximal internal jugular vein at the jugular bulb. Flow was in a caudal–cephalad direction. There was no contralateral signal abnormality. Left brachiocephalic stenosis was confirmed by magnetic resonance venography (MRV) of the neck. In the first two cases, DSA was performed to rule out CCF. However, knowledge of the prior two cases allowed the authors to forgo DSA in the third case and perform less invasive imaging, a central contrast-enhanced MRV, instead. In the second report, Watson and Russo [2] described a 36-yearold woman on dialysis with a left arm hemodialysis fistula with intermittent visual blurriness and headaches over a period of 3 weeks. Noncontrast magnetic resonance imaging (MRI) of the brain revealed prominence of the left superior ophthalmic vein and mild left proptosis. Intracranial MRA showed increased signal abnormality within the left cavernous sinus, left transverse sinus, left inferior petrosal sinus, and the ipsilateral superior ophthalmic vein,
Fig. 3. Axial MRA of the brain demonstrates signal secondary to flow (arrow) in the left cavernous sinus adjacent to the left cavernous carotid artery.
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Fig. 6. DSA spot film of the chest during injection of arteriovenous fistula in left arm demonstrates recanalization, stenting, and patency of the left BCV.
Fig. 4. Left vertebral injection, anteroposterior projection demonstrates early, asymmetric filling of left facial and external jugular veins (arrow) in the late arterial phase.
suggesting CCF. Cerebral DSA showed no evidence of CCF. Venous phase images during injection of the aortic arch demonstrated left BCV occlusion. Left-sided venous return occurred retrograde through the left internal jugular vein. Recanalization with Wallstent placement was performed, and 5-month follow-up noted resolution of symptoms. The most recent example was described in a case conference. A 64year-old male who suffered from end-stage renal disease on hemodialysis complained of 3 weeks of right periorbital and facial swelling. Ophthalmologic exam demonstrated a poorly reactive/ dilated pupil, conjunctival injection, chemosis, and elevated intraocular pressure. His symptoms worsened despite treatment for periorbital cellulitis with antibiotics. Once again, 3D TOF MRA showed abnormal signal in the right superior ophthalmic vein, right cavernous, inferior petrosal, and cavernous sinuses. Additional collateral facial and scalp veins demonstrated abnormal flow on MRV. Intracranial DSA failed to demonstrate a CCF. Injection into the patient's right AV graft demonstrated complete occlusion of the right BCV with retrograde flow into the internal jugular vein. The BCV
Fig. 5. DSA spot film of the chest during injection of arteriovenous fistula in left arm demonstrates occlusion of the left mid-BCV (arrow) and cephalad flow of contrast in the left neck veins.
subsequently underwent angioplasty after a glide wire was passed through the occlusion and a Cordis S.M.A.R.T. Stent was deployed to maintain patency [3]. Our case is the sixth reported case of central venous occlusion mimicking a CCF and only the third reported case of this presentation in a dialysis patient. Additionally, it is the second reported case of using a S.M.A.R.T. stent for recanalization of the BCV. It differs from previously reported cases in a number of ways. Our patient was completely blind, secondary to complications of long-standing diabetes, and thus could not report changes in vision. Additionally, while suspicion of CCF was based on early opacification of the left cavernous sinus, the observation of retrograde flow in the left external jugular vein during the left vertebral artery injection was a unique observation made during this case, which facilitated the correct diagnosis. For the studies discussed in this paper, a summary of clinical presentations, imaging findings, interventions, and outcomes is provided in Table 1. 3. Discussion This case demonstrates a central venous occlusion that mimicked CCF on clinical presentation as well as on axial imaging. Opacification of the cavernous sinus during angiography may suggest direct or dural CCF, arteriovenous malformations, or other less common etiologies [4,5]. Symptoms of CCF in our patient included exophthalmos, conjunctival chemosis, orbital pain, and dilated conjunctival and episcleral vessels. Findings in our patient atypical for CCF included facial swelling and lack of ipsilateral superior ophthalmic vein enlargement. In addition, this patient's bruit was not heard over the eye itself, as is typical in CCF, but only over the temple near the eye. During cerebral DSA, the left vertebral artery injection with secondary reflux into the left subclavian artery led us to the correct diagnosis. Of note, the left vertebral artery was not dominant in our
Fig. 7. Photograph of the left eye after S.M.A.R.T. stent placement showing resolution of chemosis and conjunctival injection.
G.R. Salama et al. / Clinical Imaging 38 (2014) 884–887
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Table 1 Summary and comparison of clinical presentations, imaging findings, interventions, and outcomes Case report
Symptoms
[1]—Patient #1
Vertebrobasilar insufficiency, 3D TOF MRA Head: abnormal vascular fluctuating unilateral head pain signal intensity in the unilateral jugular bulb, inferior petrosal sinus, and cavernous sinus Diplopia 3D TOF MRA Head: hyperintense vascular signal in the unilateral inferior petrosal sinus and cavernous sinus
[1]—Patient #2
[1]—Patient Vertebrobasilar insufficiency #3
[2]
Intermittent visual blurriness and headaches
[3]
Unilateral periorbital/facial swelling
Salama et al. Unilateral facial swelling, 2014 pulsatile tinnitus, bulging eye
Findings suggesting CCF
Diagnostic study
Intervention
Intracranial DSA: no CCF. 3D neck MRV: N/A ipsilatel BCV stenosis
Intracranial DSA: no CCF. MRV head: flow direction was cranial and flowrelated enhancement was seen in the ipsilateral internal jugular vein. MRV neck: BCV stenosis 3D TOF MRA Head: hyperintense vascular MRV head: cranial-directed flow and signal in unilateral inferior petrosal sinus flow-related enhancement in the ipsilateral internal jugular vein. MRV and cavernous sinus neck: BCV stenosis Intracranial DSA: arterial phase—no MRI brain: prominence of unilateral CCF; venous phase—retrograde flow superior ophthalmic vein and mild through the ipsilateral internal jugular ipsilateral proptosis. 3D TOF MRA: increased signal intensity in the ipsilateral vein cavernous sinus, transverse sinus, inferior petrosal sinus, and ipsilateral superior ophthalmic vein Intracranial DSA: no CCF. DSA with AV 3D TOF MRA Head: increased flow in graft injection: brachiocephalic ipsilateral cavernous sinus, opthlamic vein, signmoid and transverse sinuses and occlusion with retrograde flow in inferior petrosal sinus and internal jugular internal jugular vein vein with abnormal facial and scalp veins CTA head: opacification of the ipsilateral Intracranial DSA: no CCF. Retrograde cavernous sinus, jugular bulb, facial vein, flow in ipsilateral neck veins. DSA with retromandibular vein, and internal AV fistula injection: retrograde flow in jugular vein in the arterial phase. 3D TOF external jugular vein. Occlusion of BCV MRA: mildly increased signal in the ipsilateral cavernous sinus
Outcome of intervention N/A
N/A
N/A
N/A
N/A
Racanalization DSA: normal antegrade and Wallstent flow returned to the left internal jugular vein. Resolution of visual symptoms
Angioplasty and S.M.A.R.T. stent
DSA: normal antegrade flow returned to the left internal jugular vein
Angioplasty and S.M.A.R.T. stent
DSA: normal antegrade flow returned to the left internal jugular vein. Resolution of facial swelling
AV: arteriovenous; N/A: not available.
case but sufficient in caliber and easier to access than the right vertebral artery. Had we injected the dominant, right vertebral artery, we might have missed the retrograde left subclavian flow of contrast passing through the left arm fistula and into the left external jugular vein, which occurred due to the left BCV occlusion. The patient's left arm and left neck veins drained in a retrograde direction into the left cavernous sinus, across the intercavernous sinus to the right cavernous sinus, and returned to the heart via right neck veins. Our patient had two significant risk factors for central venous occlusion: diabetes and high flow venous return from the left upper extremity via the hemodialysis fistula. Diabetics have accelerated proliferation, adhesion, and migration of vascular smooth muscle cells as well as abnormal contact inhibition [6], placing them at increased risk for intimal hyperplasia. In addition, venous stenosis is promoted by the high flow rates seen with arteriovenous fistulas [7]. Central venous stenosis is also commonly associated with placement of central venous catheters and devices [8]. Of note, diabetics are more prone to develop venous intimal hyperplasia and thrombosis in response to endothelial trauma induced by HD catheters [9]. However, our patient had no history of ipsilateral central venous catheterization. Salgado et al. [10] report that elective ligation of hemodialysis access sites secondary to central venous occlusion is more frequent in patients with previous left jugular venous catheterization. Left jugular venous catheters follow a longer course to the SVC via the left BCV and are associated with higher rates of occlusion than right-sided venous catheters. Therefore, they recommend evaluation of the venous system prior to left-sided dialysis access creation for diabetic ESRD patients who have had previous left-sided internal jugular central venous catheters. This is the third reported case of a dialysis patient with central venous occlusion of the BCV presenting with signs and symptoms mimicking CCF. Nephrologists and peripheral interventional radiol-
ogists commonly diagnose this entity. In our case, the patient's fistula was aneurysmal, which is common in the setting of proximal venous stenosis. Had nephrology been consulted and more focus given to this patient's fistula site, the diagnosis of central venous occlusion might have been made sooner. Therefore, diabetic dialysis patients with aneurysmal fistulae and signs and symptoms of CCF should be evaluated for central venous occlusions to avoid unnecessarily invasive diagnostic procedures and expedite the delivery of effective treatment.
References [1] Paksoy Y, Oguz Gen B, Genc E. Retrograde flow in the left inferior petrosal sinus and blood steal of the cavernous sinus associated with central vein stenosis: MR angiographic findings. Am J Neuroradiol 2003;24:1364–8. [2] Watson R, Russo C. Upper extremity arteriovenous dialysis fistula resulting in cavernous sinus arterialized blood flow. Am J Neuroradiol 2007;28:1155–6. [3] Gibbs Jerry M. “Case Report #0437” Houston Angiography Club Meeting. PowerPoint 2007; 2008 [bhttp://www.uth.tmc.edu/radiology/ICF/2007_2008/ 0437.ppsN]. [4] Glaser J. Neuro-ophthalmology, Hagerstown, Harper and Row; 1978 336–8. [5] Stark D, Bradley W, editors. Magnetic resonance imaging, 1308. St Louis: C.V. Mosby; 1999. p. 1660–1. [6] Faries PL, Rohan DI, Takahara H, Wyers MC, Contreras MA, Quist WC, King GL, Logerfo FW. Human vascular smooth muscle cells of diabetic origin exhibit increased proliferation, adhesion, and migration. J Vasc Surg 2001;33(3):601–7. [7] Jaberi A, Schwartz D, Marticorena R, Dacouris N, Prabhudesai V, McFarlane P, Donnelly S. Risk factors for the development of cephalic arch stenosis. J Vasc Access 2007;8(4):287–95. [8] Agarwal A, Patel B, Haddad N. Central vein stenosis: a nephrologist's perspective. Semin Dial 2007;20:53–62. [9] Mompeõ B, Ortega F, Sarmiento L, Castaño I. Ultrastructual analogies between intimal alterations in veins from diabetic patents and animals with STZ-induced diabetics. Ann Vasc Surg 1999;13:294–301. [10] Salgado OJ, Urdaneta B, Colmenares B, García R, Flores C. Right versus left internal jugular vein catheterization for hemodialysis: complications and impact on ipsilateral access creation. Artif Organs 2004;28(8):728–33.
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
Central venous occlusion mimics carotid cavernous fistula: a case report and review of the literature☆,☆☆ Gayle R. Salama a,⁎,1, Joaquim M. Farinhas a,1, David D. Pasquale a,1, Christian Wertenbaker b,1, Jacqueline A. Bello a,1 a
Department of Neuroradiology, Montefiore Medical Center, 111 East 210th St., Bronx, NY 10647, USA Department of Ophthalmology, Montefiore Medical Center, 111 East 210th St., Bronx, NY 10647, USA
b
a r t i c l e
i n f o
Article history: Received 23 February 2014 Received in revised form 21 June 2014 Accepted 30 June 2014 Keywords: Central venous occlusion Carotid cavernous fistula Digital subtraction angiography Recanalization Interventional radiology
a b s t r a c t A patient presented with signs and symptoms of a left carotid cavernous fistula (CCF). Computed tomography angiography confirmed filling of the cavernous sinus in the arterial phase. Cerebral digital subtraction angiography demonstrated no evidence of CCF. The workup, diagnosis, and treatment of this patient are discussed, and the literature is reviewed. Published by Elsevier Inc.
1. Case description A 40-year-old female with diabetes mellitus type 1, hypertension, hypothyroidism, end-stage renal disease on hemodialysis, and binocular total blindness from proliferative diabetic retinopathy and tractional retinal detachments presented with 3three days of worsening “whooshing in the left ear” and painful “bulging” of her left eye. Three months prior to admission, she had noted a “bump” on her left forehead, which progressed to left facial edema and nasal congestion. She also noted pulsatile tinnitus if she lay on her left ear and variable pain in the left eye and face for about 4 months. She reported that her symptoms improved during dialysis treatments. Her hemodialysis fistula was in the left upper arm. On the day prior to admission, she was seen by her ophthalmologist, who noted prominent left conjunctival and episcleral vessels and left conjunctival chemosis, and sent her to the emergency department for evaluation of a possible carotid cavernous fistula (CCF). ☆ Advances in Knowledge:1. Retrograde filling of the external and internal jugular veins during the intracapillary phase of a vertebral artery injection may suggest central venous occlusion. ☆☆ Implications for Patient Care:1. Diabetic patients on dialysis with aneurysmal fistulae and signs and symptoms of carotid cavernous fistula (CCF) should be evaluated for central venous occlusions.2. Nephrologists and peripheral interventional radiologists should be consulted earlier in the care of patients with fistula sites and signs and symptoms of CCF because they commonly see central venous occlusions and can facilitate a more rapid diagnosis. ⁎ Corresponding author. Weill Cornell Medical Center–NYPH, 525 East 68th Street, Box 141, New York, NY 10065, USA. E-mail address: [email protected] (G.R. Salama). 1 Tel.:+1 718 798 5449; fax: +1 718 798 5376. http://dx.doi.org/10.1016/j.clinimag.2014.06.018 0899-7071/Published by Elsevier Inc.
On admission, her blood pressure was 142/88. She was in no acute distress. Physical exam was remarkable for the following: conjunctival and episcleral injection and mild proptosis of the left eye, a bruit over the left temple near the eye but not over the eye itself, normal extraocular movements, left-sided eyelid and facial edema extending to the forehead, and decreased sensation over the left face (Fig. 1). There was no left facial weakness and tongue protrusion was midline. Laboratory values were remarkable for blood urea nitrogen and creatinine of 29.0 and 6.4, respectively. Computed tomography angiography (CTA) of the head (Fig. 2) showed asymmetric opacification of the left cavernous sinus, left jugular bulb, left facial vein, and left retromandibular vein, as well as the left internal jugular vein in the arterial phase. The venous plexus within the left masticator space and left parotid space was also asymmetrically prominent. These findings were suspicious for a CCF; however, the left superior ophthalmic vein was neither enlarged nor opacified in the arterial phase of the CTA. Magnetic resonance angiogram (MRA; Fig. 3) showed mildly increased signal in the left cavernous sinus. Intracranial digital subtraction angiography (DSA) showed no evidence of CCF. However, the left vertebral artery injection showed filling of the left internal and external jugular veins in a caudal–cephalad direction during the intracranial capillary phase (Fig. 4) due to reflux of contrast into the left subclavian artery and into the left neck veins via the left arm fistula. Additionally, right and left external carotid artery injections further ruled out a dural CCF. Given the lack of an intracranial cause for a CCF and unexplained caudal-to-cephalad filling of the left neck veins, peripheral venous injections of the upper extremities were performed. The right upper
G.R. Salama et al. / Clinical Imaging 38 (2014) 884–887
885
Fig. 1. Photograph of the face showing asymmetric swelling of the left side and of the left eye shows episcleral and conjunctival injection.
extremity venous injection demonstrated normal flow to a patent superior vena cava. Direct injection of the patient's left arm fistula, which was aneurismal, demonstrated complete obstruction of the left proximal brachiocephalic vein (BCV; Fig. 5). Retrograde venous flow was noted first in the left external jugular and facial veins followed by retrograde flow in the left internal jugular vein. Recanalization of the chronic left BCV occlusion was performed using an 18-gauge Colapinto needle. Angioplasty and stenting of the left BCV were performed using a 10×40mm S.M.A.R.T. Stent (Cordis Corporation, Bridgewater, NJ, USA; Fig. 6). Her symptoms improved within 24 h (Fig. 7).
2. Literature review We reviewed the literature conjoining CCF, central venous stenosis or occlusion, and dialysis. To do so, we systematically searched PubMed and the World Wide Web through Google's search engine in January 2014 for studies investigating patients who demonstrate symptoms and imaging findings of a CCF and who are ultimately diagnosed with central venous occlusion. Only two PubMed publications and one conference presentation, independently published,
Fig. 2. Axial contrast-enhanced CT image of the brain demonstrates asymmetric enhancement of the left cavernous sinus (horizontal arrow) adjacent to the left cavernous carotid artery. Opacification of left facial veins (vertical arrow) and left hemi-facial swelling (oblique arrow) are also seen.
satisfied our inclusion criteria of central venous occlusion mimicking CCF clinically and by either CTA or MRA imaging. The first report, by Paksoy et al. [1], described three cases. All three cases were women ranging in age from 48 to 70 years. Presenting symptoms varied among the patients and included vertebrobasilar insufficiency, fluctuating left cephalalgia, and diplopia. Each patient underwent intracranial three-dimensional (3D) time-of-flight (TOF) MRA showing hyperintense vascular signal from the left cavernous sinus to the proximal internal jugular vein at the jugular bulb. Flow was in a caudal–cephalad direction. There was no contralateral signal abnormality. Left brachiocephalic stenosis was confirmed by magnetic resonance venography (MRV) of the neck. In the first two cases, DSA was performed to rule out CCF. However, knowledge of the prior two cases allowed the authors to forgo DSA in the third case and perform less invasive imaging, a central contrast-enhanced MRV, instead. In the second report, Watson and Russo [2] described a 36-yearold woman on dialysis with a left arm hemodialysis fistula with intermittent visual blurriness and headaches over a period of 3 weeks. Noncontrast magnetic resonance imaging (MRI) of the brain revealed prominence of the left superior ophthalmic vein and mild left proptosis. Intracranial MRA showed increased signal abnormality within the left cavernous sinus, left transverse sinus, left inferior petrosal sinus, and the ipsilateral superior ophthalmic vein,
Fig. 3. Axial MRA of the brain demonstrates signal secondary to flow (arrow) in the left cavernous sinus adjacent to the left cavernous carotid artery.
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G.R. Salama et al. / Clinical Imaging 38 (2014) 884–887
Fig. 6. DSA spot film of the chest during injection of arteriovenous fistula in left arm demonstrates recanalization, stenting, and patency of the left BCV.
Fig. 4. Left vertebral injection, anteroposterior projection demonstrates early, asymmetric filling of left facial and external jugular veins (arrow) in the late arterial phase.
suggesting CCF. Cerebral DSA showed no evidence of CCF. Venous phase images during injection of the aortic arch demonstrated left BCV occlusion. Left-sided venous return occurred retrograde through the left internal jugular vein. Recanalization with Wallstent placement was performed, and 5-month follow-up noted resolution of symptoms. The most recent example was described in a case conference. A 64year-old male who suffered from end-stage renal disease on hemodialysis complained of 3 weeks of right periorbital and facial swelling. Ophthalmologic exam demonstrated a poorly reactive/ dilated pupil, conjunctival injection, chemosis, and elevated intraocular pressure. His symptoms worsened despite treatment for periorbital cellulitis with antibiotics. Once again, 3D TOF MRA showed abnormal signal in the right superior ophthalmic vein, right cavernous, inferior petrosal, and cavernous sinuses. Additional collateral facial and scalp veins demonstrated abnormal flow on MRV. Intracranial DSA failed to demonstrate a CCF. Injection into the patient's right AV graft demonstrated complete occlusion of the right BCV with retrograde flow into the internal jugular vein. The BCV
Fig. 5. DSA spot film of the chest during injection of arteriovenous fistula in left arm demonstrates occlusion of the left mid-BCV (arrow) and cephalad flow of contrast in the left neck veins.
subsequently underwent angioplasty after a glide wire was passed through the occlusion and a Cordis S.M.A.R.T. Stent was deployed to maintain patency [3]. Our case is the sixth reported case of central venous occlusion mimicking a CCF and only the third reported case of this presentation in a dialysis patient. Additionally, it is the second reported case of using a S.M.A.R.T. stent for recanalization of the BCV. It differs from previously reported cases in a number of ways. Our patient was completely blind, secondary to complications of long-standing diabetes, and thus could not report changes in vision. Additionally, while suspicion of CCF was based on early opacification of the left cavernous sinus, the observation of retrograde flow in the left external jugular vein during the left vertebral artery injection was a unique observation made during this case, which facilitated the correct diagnosis. For the studies discussed in this paper, a summary of clinical presentations, imaging findings, interventions, and outcomes is provided in Table 1. 3. Discussion This case demonstrates a central venous occlusion that mimicked CCF on clinical presentation as well as on axial imaging. Opacification of the cavernous sinus during angiography may suggest direct or dural CCF, arteriovenous malformations, or other less common etiologies [4,5]. Symptoms of CCF in our patient included exophthalmos, conjunctival chemosis, orbital pain, and dilated conjunctival and episcleral vessels. Findings in our patient atypical for CCF included facial swelling and lack of ipsilateral superior ophthalmic vein enlargement. In addition, this patient's bruit was not heard over the eye itself, as is typical in CCF, but only over the temple near the eye. During cerebral DSA, the left vertebral artery injection with secondary reflux into the left subclavian artery led us to the correct diagnosis. Of note, the left vertebral artery was not dominant in our
Fig. 7. Photograph of the left eye after S.M.A.R.T. stent placement showing resolution of chemosis and conjunctival injection.
G.R. Salama et al. / Clinical Imaging 38 (2014) 884–887
887
Table 1 Summary and comparison of clinical presentations, imaging findings, interventions, and outcomes Case report
Symptoms
[1]—Patient #1
Vertebrobasilar insufficiency, 3D TOF MRA Head: abnormal vascular fluctuating unilateral head pain signal intensity in the unilateral jugular bulb, inferior petrosal sinus, and cavernous sinus Diplopia 3D TOF MRA Head: hyperintense vascular signal in the unilateral inferior petrosal sinus and cavernous sinus
[1]—Patient #2
[1]—Patient Vertebrobasilar insufficiency #3
[2]
Intermittent visual blurriness and headaches
[3]
Unilateral periorbital/facial swelling
Salama et al. Unilateral facial swelling, 2014 pulsatile tinnitus, bulging eye
Findings suggesting CCF
Diagnostic study
Intervention
Intracranial DSA: no CCF. 3D neck MRV: N/A ipsilatel BCV stenosis
Intracranial DSA: no CCF. MRV head: flow direction was cranial and flowrelated enhancement was seen in the ipsilateral internal jugular vein. MRV neck: BCV stenosis 3D TOF MRA Head: hyperintense vascular MRV head: cranial-directed flow and signal in unilateral inferior petrosal sinus flow-related enhancement in the ipsilateral internal jugular vein. MRV and cavernous sinus neck: BCV stenosis Intracranial DSA: arterial phase—no MRI brain: prominence of unilateral CCF; venous phase—retrograde flow superior ophthalmic vein and mild through the ipsilateral internal jugular ipsilateral proptosis. 3D TOF MRA: increased signal intensity in the ipsilateral vein cavernous sinus, transverse sinus, inferior petrosal sinus, and ipsilateral superior ophthalmic vein Intracranial DSA: no CCF. DSA with AV 3D TOF MRA Head: increased flow in graft injection: brachiocephalic ipsilateral cavernous sinus, opthlamic vein, signmoid and transverse sinuses and occlusion with retrograde flow in inferior petrosal sinus and internal jugular internal jugular vein vein with abnormal facial and scalp veins CTA head: opacification of the ipsilateral Intracranial DSA: no CCF. Retrograde cavernous sinus, jugular bulb, facial vein, flow in ipsilateral neck veins. DSA with retromandibular vein, and internal AV fistula injection: retrograde flow in jugular vein in the arterial phase. 3D TOF external jugular vein. Occlusion of BCV MRA: mildly increased signal in the ipsilateral cavernous sinus
Outcome of intervention N/A
N/A
N/A
N/A
N/A
Racanalization DSA: normal antegrade and Wallstent flow returned to the left internal jugular vein. Resolution of visual symptoms
Angioplasty and S.M.A.R.T. stent
DSA: normal antegrade flow returned to the left internal jugular vein
Angioplasty and S.M.A.R.T. stent
DSA: normal antegrade flow returned to the left internal jugular vein. Resolution of facial swelling
AV: arteriovenous; N/A: not available.
case but sufficient in caliber and easier to access than the right vertebral artery. Had we injected the dominant, right vertebral artery, we might have missed the retrograde left subclavian flow of contrast passing through the left arm fistula and into the left external jugular vein, which occurred due to the left BCV occlusion. The patient's left arm and left neck veins drained in a retrograde direction into the left cavernous sinus, across the intercavernous sinus to the right cavernous sinus, and returned to the heart via right neck veins. Our patient had two significant risk factors for central venous occlusion: diabetes and high flow venous return from the left upper extremity via the hemodialysis fistula. Diabetics have accelerated proliferation, adhesion, and migration of vascular smooth muscle cells as well as abnormal contact inhibition [6], placing them at increased risk for intimal hyperplasia. In addition, venous stenosis is promoted by the high flow rates seen with arteriovenous fistulas [7]. Central venous stenosis is also commonly associated with placement of central venous catheters and devices [8]. Of note, diabetics are more prone to develop venous intimal hyperplasia and thrombosis in response to endothelial trauma induced by HD catheters [9]. However, our patient had no history of ipsilateral central venous catheterization. Salgado et al. [10] report that elective ligation of hemodialysis access sites secondary to central venous occlusion is more frequent in patients with previous left jugular venous catheterization. Left jugular venous catheters follow a longer course to the SVC via the left BCV and are associated with higher rates of occlusion than right-sided venous catheters. Therefore, they recommend evaluation of the venous system prior to left-sided dialysis access creation for diabetic ESRD patients who have had previous left-sided internal jugular central venous catheters. This is the third reported case of a dialysis patient with central venous occlusion of the BCV presenting with signs and symptoms mimicking CCF. Nephrologists and peripheral interventional radiol-
ogists commonly diagnose this entity. In our case, the patient's fistula was aneurysmal, which is common in the setting of proximal venous stenosis. Had nephrology been consulted and more focus given to this patient's fistula site, the diagnosis of central venous occlusion might have been made sooner. Therefore, diabetic dialysis patients with aneurysmal fistulae and signs and symptoms of CCF should be evaluated for central venous occlusions to avoid unnecessarily invasive diagnostic procedures and expedite the delivery of effective treatment.
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