Case Reports / Journal of Clinical Neuroscience 21 (2014) 1259–1261
1259
The use of adenosine in the treatment of a high-flow vein of Galen malformation in an adult Asterios Tsimpas a, Nohra Chalouhi a, Jonathan D. Halevy b, Stavropoula Tjoumakaris a, L. Fernando Gonzalez a, Stephen J. Monteith a, Aaron S. Dumont a, Robert Rosenwasser a, Pascal Jabbour a,⇑ a Division of Neurovascular Surgery and Endovascular Neurosurgery, Department of Neurosurgery, Thomas Jefferson University Hospital, 909 Walnut Street, Philadelphia, PA 19107, USA b Rancocas Valley Anesthesia, Cinnaminson, NJ, USA
a r t i c l e
i n f o
Article history: Received 23 October 2013 Accepted 9 November 2013
Keywords: Adenosine Endovascular therapy Flow arrest High-flow arteriovenous shunt Vein of Galen aneurysmal malformation
a b s t r a c t The treatment of high-flow vein of Galen aneurysmal malformations (VGAM) remains a therapeutic challenge for the neurosurgeon and the neurointerventionalist, as it is associated with high morbidity and mortality rates despite recent advances in open cranial surgery and interventional neuroradiology. A 37-year-old patient presented with a history of non-specific headaches. He had a history of heart failure since birth that was caused by an untreated VGAM. Intravenous boluses of adenosine were injected as an attempt to slow down the arteriovenous shunting of a VGAM prior to endovascular treatment. Adenosine can be a very useful adjunct in patients with extremely high arteriovenous shunting. n-butyl cyanoacrylate (n-BCA) should be the embolic material of choice due to its quick polymerization and adhesive properties. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Vein of Galen aneurysmal malformations (VGAM) are complex, high-flow arteriovenous malformations of the brain that have been described since the late 19th century, and they are diagnosed almost exclusively in children [1]. The operative mortality approximates 90% [2–4]. They are now treated almost exclusively via the endovascular route. The intravenous (IV) use of adenosine has been described in the literature when temporary flow arrest needs to be achieved, such as in embolization of high-flow arteriovenous malformations, operative treatment of intracranial aneurysms, and various open cardiothoracic and systemic interventional procedures [5–8]. To our knowledge this is the first report describing the use of IV adenosine in the treatment of a very high-flow VGAM in an adult patient. 2. Case report A 37-year-old patient presented with a history of non-specific headaches. He had a history of heart failure since birth that was caused by an untreated VGAM. His cardiac disease was managed conservatively with digoxin and beta-blockers. He was neurologically intact on physical examination. An MRI/magnetic resonance angiography of the brain and a digital subtraction angiography showed a complex, mural lesion with rapid arteriovenous (AV) shunting. Multiple arterial feeders, which originated from anterior (ACA), middle and posterior cerebral arteries bilaterally, as well as anterior and posterior choroidal branches, terminated at a largely dilated venous pouch that drained into a falcine sinus (Fig. 1). The patient was monitored with electrocardiogram leads, two oxygen saturation probes, a capnograph, a non-invasive blood pressure cuff, and a central and a radial arterial line. Transcutaneous pacing pads were placed on the patient’s chest, and the pacer
⇑ Corresponding author. Tel.: +1 215 503 7008; fax: +1 215 503 7038. E-mail address: [email protected] (P. Jabbour).
was programmed to fire manually. A 7 French (F) sheath was placed in the right common femoral artery and a 6F sheath was placed in the left common femoral vein. The blood pressure was monitored additionally via the arterial groin sheath. A 6F Envoy catheter (Codman Neurovascular, Raynham, MA, USA) was placed over a 0.038 inch guide wire into the distal cervical segment of the right internal carotid artery (ICA). A Marathon flow-directed microcatheter (ev3 Neurovascular, Irvine, CA, USA) was advanced over a Synchro-10 microwire (Stryker Neurovascular, Fremont, CA, USA) into the distal right ACA, just proximal to one of the fistulous points. A microinjection with contrast made obvious that embolization of the arterial feeder without slowing down the AV shunt would be extremely dangerous. A 70% mixture of n-butyl cyanoacrylate (TRUFILL n-BCA, Codman Neurovascular) in ethiodized oil was chosen due to its adhesive properties and rapid polymerization. A nicardipine infusion dropped the mean arterial pressure to 40. A repeat right ICA angiogram did not demonstrate any significant slowing of the AV shunt. A slow injection of n-BCA was attempted, but it was rapidly shunted to the venous side. An attempt to further decrease the flow with multiple coils through the venous side without detaching them was unsuccessful, since the strong AV shunt pushed them out of the venous pouch and into the torcula. They were pulled out before becoming detached. The nicardipine infusion was stopped. A test dose of 21 mg adenosine (0.3 mg/kg) produced only a 3–4 second flow arrest. A subsequent dose of 39 mg (0.55 mg/kg) produced an approximately 10 second long near asystole, and the n-BCA was injected into the right ACA feeder. A repeat angiographic run showed significant slowing of the AV shunt without substantial obstruction of the venous outflow (Fig. 2). The patient was then extubated and was transferred to the intensive care unit. He was neurologically intact. During the first postoperative night the patient became progressively lethargic and stopped following commands. A CT scan of the brain revealed clotting of the venous pouch and formation of a right basal ganglia and a left frontal venous infarction with early hemorrhagic transformation. Embolic material was seen inside the falcine sinus and the torcula, causing at least partial outflow obstruction. The patient was eventually discharged to a reha-
1260
Case Reports / Journal of Clinical Neuroscience 21 (2014) 1259–1261
Fig. 1. Digital subtraction angiography showing (A) early anteroposterior (AP) view of the right internal carotid artery (ICA) injection before treatment, (B) late AP view of the right ICA injection before treatment, (C) early lateral view of the right ICA injection before treatment and (D) late lateral view of the right ICA injection before treatment.
Fig. 2. Digital subtraction angiography showing (A) anteroposterior (AP) view of the right internal carotid artery (ICA) injection after treatment with n-butyl cyanoacrylate (n-BCA), (B) early lateral view of the right ICA injection after treatment with n-BCA and (C) late lateral view of right ICA injection after treatment with n-BCA.
bilitation facility and his clinical condition progressively improved to the point where he was neurologically intact at his 6 month clinical follow-up. Follow-up angiography also demonstrated 100% occlusion of the lesion (Fig. 3).
3. Discussion Adenosine is an endogenous purine nucleoside that, among other effects, suppresses the sinoatrial and atrioventricular
Case Reports / Journal of Clinical Neuroscience 21 (2014) 1259–1261
1261
may have detached from the arterial wall and partially obstructed the outflow zone, which triggered retrograde thrombosis of the venous pouch and the deep venous system. Although the patient was kept normotensive after the embolization, this maneuver may have been insufficient due to the rapid AV shunting present in our patient. Gonzalez et al. suggested that systemic anticoagulation might improve the slow venous flow caused by partial outflow obstruction [10]. Our patient received a bolus of 5000 units of heparin during the procedure, and he was placed on a heparin drip after his decline, but both infarcts progressed to hemorrhagic transformation that further complicated his postoperative course. Despite this complication, an excellent clinical and angiographic outcome was achieved in this patient.
Conflicts of Interest/Disclosures
Fig. 3. Follow-up digital subtraction angiography, anteroposterior view right internal carotid artery injection showing complete occlusion of the malformation.
conduction, thereby causing a transient heart block. It also promotes endothelium-dependent smooth muscle relaxation with consequent arterial dilatation. Both effects may contribute to a transient flow arrest with profound hypotension. Adenosine is used as an antiarrhythmic in supraventricular tachycardia as a 3– 12 mg bolus every 1–2 minutes. It has a short half-life of less than 10 seconds. Therefore, it should be administered as a quick IV push followed by a saline flush via a central line, in order to maximize its cardiac effect. The use of a transdermal or transvenous pacer is highly recommended; however, the pacer settings should be turned to manual, if flow arrest is desired. Hashimoto et al. described the dose-response characteristics of adenosine when used in endovascular therapy [9]. Much higher doses, such as 1 mg/kg or even higher may be used when prolonged and profound hypotension needs to be achieved. In our patient, only two test doses were given due to the current shortage of adenosine at a national level. Nevertheless, the flow arrest achieved after the second dose was long enough to allow us to inject n-BCA, while minimizing the risk of distal embolization. We decided to use n-BCA due to its rapid polymerization time and adhesive properties. The angiographic run after the embolization showed no obvious outflow obstruction and the patient remained neurologically intact for several hours after the procedure. However, the CT scan that was obtained after neurological decline revealed embolic material in the persistent falcine sinus and the torcula. Part of the glue cast http://dx.doi.org/10.1016/j.jocn.2013.11.014
The industry affiliations of the authors are as follows: Pascal Jabbour: Consultant for ev3, Codman, Mizuho; Stavropoula Tjoumakaris: Consultant for Stryker; L. Fernando Gonzalez: Consultant for ev3; Aaron S. Dumont: Consultant for ev3, Stryker; and Robert Rosenwasser: Consultant for Boston Scientific. References [1] Dandy WE. Experimental hydrocephalus. Ann Surg 1919;70:129–42. [2] Hoffman HJ, Chuang S, Hendrick EB, et al. Aneurysms of the vein of Galen. Experience at the Hospital for Sick Children, Toronto. J Neurosurg 1982;57:316–22. [3] Morgan MK, Johnston IH, Sundt Jr TM. Normal perfusion pressure breakthrough complicating surgery for the vein of Galen malformation: report of three cases. Neurosurgery 1989;24:406–10. [4] Johnston IH, Whittle IR, Besser M, et al. Vein of Galen malformation: diagnosis and management. Neurosurgery 1987;20:747–58. [5] Bendok BR, Gupta DK, Rahme RJ, et al. Adenosine for temporary flow arrest during intracranial aneurysm surgery: a single-center retrospective review. Neurosurgery 2011;69:815–20 [discussion 820–811]. [6] Dorros G, Cohn JM. Adenosine-induced transient cardiac asystole enhances precise deployment of stent-grafts in the thoracic or abdominal aorta. J Endovasc Surg 1996;3:270–2. [7] Guinn NR, McDonagh DL, Borel CO, et al. Adenosine-induced transient asystole for intracranial aneurysm surgery: a retrospective review. J Neurosurg Anesthesiol 2011;23:35–40. [8] Pile-Spellman J, Young WL, Joshi S, et al. Adenosine-induced cardiac pause for endovascular embolization of cerebral arteriovenous malformations: technical case report. Neurosurgery 1999;44:881–6 [discussion 886–887]. [9] Hashimoto T, Young WL, Aagaard BD, et al. Adenosine-induced ventricular asystole to induce transient profound systemic hypotension in patients undergoing endovascular therapy. Dose–response characteristics. Anesthesiology 2000;93:998–1001. [10] Gonzalez LF, Chalouhi N, Jabbour P, et al. Rapid and progressive venous thrombosis after occlusion of high-flow arteriovenous fistula. World Neurosurg 2013;80:e359–365.
1259
The use of adenosine in the treatment of a high-flow vein of Galen malformation in an adult Asterios Tsimpas a, Nohra Chalouhi a, Jonathan D. Halevy b, Stavropoula Tjoumakaris a, L. Fernando Gonzalez a, Stephen J. Monteith a, Aaron S. Dumont a, Robert Rosenwasser a, Pascal Jabbour a,⇑ a Division of Neurovascular Surgery and Endovascular Neurosurgery, Department of Neurosurgery, Thomas Jefferson University Hospital, 909 Walnut Street, Philadelphia, PA 19107, USA b Rancocas Valley Anesthesia, Cinnaminson, NJ, USA
a r t i c l e
i n f o
Article history: Received 23 October 2013 Accepted 9 November 2013
Keywords: Adenosine Endovascular therapy Flow arrest High-flow arteriovenous shunt Vein of Galen aneurysmal malformation
a b s t r a c t The treatment of high-flow vein of Galen aneurysmal malformations (VGAM) remains a therapeutic challenge for the neurosurgeon and the neurointerventionalist, as it is associated with high morbidity and mortality rates despite recent advances in open cranial surgery and interventional neuroradiology. A 37-year-old patient presented with a history of non-specific headaches. He had a history of heart failure since birth that was caused by an untreated VGAM. Intravenous boluses of adenosine were injected as an attempt to slow down the arteriovenous shunting of a VGAM prior to endovascular treatment. Adenosine can be a very useful adjunct in patients with extremely high arteriovenous shunting. n-butyl cyanoacrylate (n-BCA) should be the embolic material of choice due to its quick polymerization and adhesive properties. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Vein of Galen aneurysmal malformations (VGAM) are complex, high-flow arteriovenous malformations of the brain that have been described since the late 19th century, and they are diagnosed almost exclusively in children [1]. The operative mortality approximates 90% [2–4]. They are now treated almost exclusively via the endovascular route. The intravenous (IV) use of adenosine has been described in the literature when temporary flow arrest needs to be achieved, such as in embolization of high-flow arteriovenous malformations, operative treatment of intracranial aneurysms, and various open cardiothoracic and systemic interventional procedures [5–8]. To our knowledge this is the first report describing the use of IV adenosine in the treatment of a very high-flow VGAM in an adult patient. 2. Case report A 37-year-old patient presented with a history of non-specific headaches. He had a history of heart failure since birth that was caused by an untreated VGAM. His cardiac disease was managed conservatively with digoxin and beta-blockers. He was neurologically intact on physical examination. An MRI/magnetic resonance angiography of the brain and a digital subtraction angiography showed a complex, mural lesion with rapid arteriovenous (AV) shunting. Multiple arterial feeders, which originated from anterior (ACA), middle and posterior cerebral arteries bilaterally, as well as anterior and posterior choroidal branches, terminated at a largely dilated venous pouch that drained into a falcine sinus (Fig. 1). The patient was monitored with electrocardiogram leads, two oxygen saturation probes, a capnograph, a non-invasive blood pressure cuff, and a central and a radial arterial line. Transcutaneous pacing pads were placed on the patient’s chest, and the pacer
⇑ Corresponding author. Tel.: +1 215 503 7008; fax: +1 215 503 7038. E-mail address: [email protected] (P. Jabbour).
was programmed to fire manually. A 7 French (F) sheath was placed in the right common femoral artery and a 6F sheath was placed in the left common femoral vein. The blood pressure was monitored additionally via the arterial groin sheath. A 6F Envoy catheter (Codman Neurovascular, Raynham, MA, USA) was placed over a 0.038 inch guide wire into the distal cervical segment of the right internal carotid artery (ICA). A Marathon flow-directed microcatheter (ev3 Neurovascular, Irvine, CA, USA) was advanced over a Synchro-10 microwire (Stryker Neurovascular, Fremont, CA, USA) into the distal right ACA, just proximal to one of the fistulous points. A microinjection with contrast made obvious that embolization of the arterial feeder without slowing down the AV shunt would be extremely dangerous. A 70% mixture of n-butyl cyanoacrylate (TRUFILL n-BCA, Codman Neurovascular) in ethiodized oil was chosen due to its adhesive properties and rapid polymerization. A nicardipine infusion dropped the mean arterial pressure to 40. A repeat right ICA angiogram did not demonstrate any significant slowing of the AV shunt. A slow injection of n-BCA was attempted, but it was rapidly shunted to the venous side. An attempt to further decrease the flow with multiple coils through the venous side without detaching them was unsuccessful, since the strong AV shunt pushed them out of the venous pouch and into the torcula. They were pulled out before becoming detached. The nicardipine infusion was stopped. A test dose of 21 mg adenosine (0.3 mg/kg) produced only a 3–4 second flow arrest. A subsequent dose of 39 mg (0.55 mg/kg) produced an approximately 10 second long near asystole, and the n-BCA was injected into the right ACA feeder. A repeat angiographic run showed significant slowing of the AV shunt without substantial obstruction of the venous outflow (Fig. 2). The patient was then extubated and was transferred to the intensive care unit. He was neurologically intact. During the first postoperative night the patient became progressively lethargic and stopped following commands. A CT scan of the brain revealed clotting of the venous pouch and formation of a right basal ganglia and a left frontal venous infarction with early hemorrhagic transformation. Embolic material was seen inside the falcine sinus and the torcula, causing at least partial outflow obstruction. The patient was eventually discharged to a reha-
1260
Case Reports / Journal of Clinical Neuroscience 21 (2014) 1259–1261
Fig. 1. Digital subtraction angiography showing (A) early anteroposterior (AP) view of the right internal carotid artery (ICA) injection before treatment, (B) late AP view of the right ICA injection before treatment, (C) early lateral view of the right ICA injection before treatment and (D) late lateral view of the right ICA injection before treatment.
Fig. 2. Digital subtraction angiography showing (A) anteroposterior (AP) view of the right internal carotid artery (ICA) injection after treatment with n-butyl cyanoacrylate (n-BCA), (B) early lateral view of the right ICA injection after treatment with n-BCA and (C) late lateral view of right ICA injection after treatment with n-BCA.
bilitation facility and his clinical condition progressively improved to the point where he was neurologically intact at his 6 month clinical follow-up. Follow-up angiography also demonstrated 100% occlusion of the lesion (Fig. 3).
3. Discussion Adenosine is an endogenous purine nucleoside that, among other effects, suppresses the sinoatrial and atrioventricular
Case Reports / Journal of Clinical Neuroscience 21 (2014) 1259–1261
1261
may have detached from the arterial wall and partially obstructed the outflow zone, which triggered retrograde thrombosis of the venous pouch and the deep venous system. Although the patient was kept normotensive after the embolization, this maneuver may have been insufficient due to the rapid AV shunting present in our patient. Gonzalez et al. suggested that systemic anticoagulation might improve the slow venous flow caused by partial outflow obstruction [10]. Our patient received a bolus of 5000 units of heparin during the procedure, and he was placed on a heparin drip after his decline, but both infarcts progressed to hemorrhagic transformation that further complicated his postoperative course. Despite this complication, an excellent clinical and angiographic outcome was achieved in this patient.
Conflicts of Interest/Disclosures
Fig. 3. Follow-up digital subtraction angiography, anteroposterior view right internal carotid artery injection showing complete occlusion of the malformation.
conduction, thereby causing a transient heart block. It also promotes endothelium-dependent smooth muscle relaxation with consequent arterial dilatation. Both effects may contribute to a transient flow arrest with profound hypotension. Adenosine is used as an antiarrhythmic in supraventricular tachycardia as a 3– 12 mg bolus every 1–2 minutes. It has a short half-life of less than 10 seconds. Therefore, it should be administered as a quick IV push followed by a saline flush via a central line, in order to maximize its cardiac effect. The use of a transdermal or transvenous pacer is highly recommended; however, the pacer settings should be turned to manual, if flow arrest is desired. Hashimoto et al. described the dose-response characteristics of adenosine when used in endovascular therapy [9]. Much higher doses, such as 1 mg/kg or even higher may be used when prolonged and profound hypotension needs to be achieved. In our patient, only two test doses were given due to the current shortage of adenosine at a national level. Nevertheless, the flow arrest achieved after the second dose was long enough to allow us to inject n-BCA, while minimizing the risk of distal embolization. We decided to use n-BCA due to its rapid polymerization time and adhesive properties. The angiographic run after the embolization showed no obvious outflow obstruction and the patient remained neurologically intact for several hours after the procedure. However, the CT scan that was obtained after neurological decline revealed embolic material in the persistent falcine sinus and the torcula. Part of the glue cast http://dx.doi.org/10.1016/j.jocn.2013.11.014
The industry affiliations of the authors are as follows: Pascal Jabbour: Consultant for ev3, Codman, Mizuho; Stavropoula Tjoumakaris: Consultant for Stryker; L. Fernando Gonzalez: Consultant for ev3; Aaron S. Dumont: Consultant for ev3, Stryker; and Robert Rosenwasser: Consultant for Boston Scientific. References [1] Dandy WE. Experimental hydrocephalus. Ann Surg 1919;70:129–42. [2] Hoffman HJ, Chuang S, Hendrick EB, et al. Aneurysms of the vein of Galen. Experience at the Hospital for Sick Children, Toronto. J Neurosurg 1982;57:316–22. [3] Morgan MK, Johnston IH, Sundt Jr TM. Normal perfusion pressure breakthrough complicating surgery for the vein of Galen malformation: report of three cases. Neurosurgery 1989;24:406–10. [4] Johnston IH, Whittle IR, Besser M, et al. Vein of Galen malformation: diagnosis and management. Neurosurgery 1987;20:747–58. [5] Bendok BR, Gupta DK, Rahme RJ, et al. Adenosine for temporary flow arrest during intracranial aneurysm surgery: a single-center retrospective review. Neurosurgery 2011;69:815–20 [discussion 820–811]. [6] Dorros G, Cohn JM. Adenosine-induced transient cardiac asystole enhances precise deployment of stent-grafts in the thoracic or abdominal aorta. J Endovasc Surg 1996;3:270–2. [7] Guinn NR, McDonagh DL, Borel CO, et al. Adenosine-induced transient asystole for intracranial aneurysm surgery: a retrospective review. J Neurosurg Anesthesiol 2011;23:35–40. [8] Pile-Spellman J, Young WL, Joshi S, et al. Adenosine-induced cardiac pause for endovascular embolization of cerebral arteriovenous malformations: technical case report. Neurosurgery 1999;44:881–6 [discussion 886–887]. [9] Hashimoto T, Young WL, Aagaard BD, et al. Adenosine-induced ventricular asystole to induce transient profound systemic hypotension in patients undergoing endovascular therapy. Dose–response characteristics. Anesthesiology 2000;93:998–1001. [10] Gonzalez LF, Chalouhi N, Jabbour P, et al. Rapid and progressive venous thrombosis after occlusion of high-flow arteriovenous fistula. World Neurosurg 2013;80:e359–365.
