526
Case Reports / Journal of Clinical Neuroscience 21 (2014) 526–529
pressure around the aneurysmal wall can lead to aneurysm rupture [4]. Decompression of type I Chiari malformations can rapidly resolve abnormal CSF flow hydrodynamics, affecting the pressure equilibrium between the sides of the aneurysm wall significantly [1,2]. In our patient, SAH occurred during bony decompression before dural opening. One plausible explanation is that the significant CSF flow changes at the foramen magnum decreased the CSF pressure against the outer aneurysm wall to such an extent that the intra-aneurysmal pressure rapidly and significantly exceeded the inward pressure from outside the aneurysm. Two previous patients with remote undiagnosed aneurysm rupture have been reported. A remote undiagnosed anterior communicating artery aneurysm ruptured during transsphenoidal resection of a pituitary adenoma. After tumor removal, the arachnoid abruptly collapsed into the sella turcica, applying a traction force on the wall of the aneurysm similar to that which may have been created by the sudden large increase in caudal CSF flow in our patient. Unlike our patient, however, the authors could not exclude intraoperative trauma as a cause of aneurysm formation [3]. The other case coincided with subdural drain insertion after urgent evacuation of a chronic subdural hematoma via frontoparietal craniotomy. CSF drainage may have affected the CSF flow dynamics and aneurysm pressure equilibrium enough to cause rupture [4]. Although our case did not involve draining excessive CSF, the decompression may have caused excessive caudal flow of CSF to the spinal region, which may have precipitated aneurysm rupture. To our knowledge this is the first report of intraoperative rupture of an undiagnosed cerebral aneurysm immediately after dural
opening during Chiari decompression. Only one prior undiagnosed cerebral aneurysm rupture without the possibility of surgical trauma has been reported and that case also occurred when CSF hydrodynamics were affected by CSF drainage with a subdural drain. The significant and rapid changes of previously abnormal CSF physiology noted with decompression of type I Chiari malformation can provide a pressure gradient that may predispose to rupture of undiagnosed cerebral aneurysms.
Conflict of interest/disclosure The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
References [1] Sivaramakrishnan A, Alperin N, Surapaneni S, et al. Evaluating the effect of decompression surgery on cerebrospinal fluid flow and intracranial compliance in patients with chiari malformation with magnetic resonance imaging flow studies. Neurosurgery 2004;55:1344–50. [2] Dolar MT, Haughton VM, Iskandar BJ, et al. Effect of craniocervical decompression on peak CSF velocities in symptomatic patients with Chiari I malformation. AJNR Am J Neuroradiol 2004;25:142–5. [3] Tsuchida T, Tanaka R, Yokoyama M, et al. Rupture of anterior communicating artery aneurysm during transsphenoidal surgery for pituitary adenoma. Surg Neurol 1983;20:67–70. [4] Stefini R, Ghitti F, Bergomi R, et al. Uncommon presentation of ruptured intracranial aneurysm during surgical evacuation of chronic subdural hematoma: case report. Surg Neurol 2008;69:89–92. [5] Ferguson GG. Physical factors in the initiation, growth, and rupture of human intracranial saccular aneurysms. J Neurosurg 1972;37:666–77.
http://dx.doi.org/10.1016/j.jocn.2013.04.031
Intraventricular tissue plasminogen activator for intraventricular hemorrhage caused by an arteriovenous malformation Kelley Keefe ⇑, Meysam Kebriaei, Andrew Gard, Arun-Angelo Patil Division of Neurosurgery, Department of Surgery, 982035 University of Nebraska Medical Center, Omaha, NE 68198-2035, USA
a r t i c l e
i n f o
Article history: Received 21 December 2012 Accepted 19 April 2013
Keywords: Intracranial arteriovenous malformation Intracranial hemorrhage Intraventricular injections Thrombolytic therapy
a b s t r a c t The use of thrombolytics delivered through an external ventricular drain has improved outcomes in intraventricular hemorrhage, a disease with a poor prognosis; however, presence of an arteriovenous malformation is generally considered a contraindication to thrombolytic use. Due do the high mortality with the current standard of care, thrombolytics should be considered as an acceptable treatment option despite the presence of an arteriovenous malformation in certain clinical situations. We review the available literature and present an additional patient to make the case for the use of thrombolytics for intraventricular hemorrhage from an arteriovenous malformation. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Intracranial hemorrhage (ICH) is the most common and most dangerous presentation of arteriovenous malformations (AVM), as it has a 10–30% mortality rate [1]. Up to 30–50% of ICH will be complicated by extension of blood into the ventricular space, forming an intraventricular hemorrhage (IVH) [2]. When this occurs, the
⇑ Corresponding author. Tel.: +1 402 559 9605; fax: +1 402 559 7779. E-mail address: [email protected] (K. Keefe).
mortality drastically increases to 50–80%, which is related to the amount of blood in the ventricles and presence of blood in the third and fourth ventricles [3]. Thus, IVH necessitates urgent treatment to prevent neurological deterioration and death. The use of intraventricular thrombolytics (IVT) began in an effort to improve outcomes and has been successful [2–4]. In a recent meta-analysis by Staykov et al., IVT were found to decrease mortality in the setting of IVH from 53% to 16% compared to external ventricular drain (EVD) alone [2]. Despite this promising application of thrombolytics to treat IVH, the presence of an AVM is often considered an absolute con-
Case Reports / Journal of Clinical Neuroscience 21 (2014) 526–529
527
Fig. 1. Axial slices of four non-contrast CT scans at presentation (A–C), post bleed day (PBD) 1 after one dose of recombinant tissue plasminogen activator (rt-PA) (D–F), PBD 2 after three doses of rt-PA (G–I), and PBD 3 after the fifth and final dose of rt-PA (J–L). The left column illustrates the resolution of blood in the fourth ventricle. The fourth ventricle is filled with blood at presentation (A), and is stable 12 hours after the first rt-PA dose (D). After three doses there is significant resolution (G), and minimal blood left after five doses (J). The middle column shows that the thalamic hemorrhage remained stable with no evidence of rebleeding during the treatment period with rt-PA (E, H, K). It also shows the removal of blood from the frontal horns of the lateral ventricles (B, E, H, K). The right column depicts evacuation of the lateral ventricles, first in the right side (F) and then in the left (I). Finally after the last dose of rt-PA, there is layering of minimal residual blood in the occipital horns of the lateral ventricles (L).
528
Case Reports / Journal of Clinical Neuroscience 21 (2014) 526–529
traindication to thrombolytic use due to the risk of rebleeding [5]. A few case reports in the literature have documented successful use of IVT to treat IVH caused by an AVM [4,6–8]. In this paper, we present additional evidence and discuss the potential use of IVT for IVH despite the presence of an AVM. 2. Case report A 20-year-old woman, with a past medical history significant for a febrile seizure at 1 year of age, presented to the emergency department after suddenly collapsing. At presentation, the patient was found to be in a decerebrate posture with a dilated, nonreactive left pupil and a mid-position, non-reactive right pupil. Initial non-contrast CT scan showed a large ICH originating in the left thalamus with blood extending into all four ventricles and the subarachnoid space. The lateral ventricles were enlarged, suggestive of obstructive hydrocephalus (Fig. 1A–C). The patient was immediately intubated, an EVD was placed, levetiracetam and mannitol were started, and her blood pressure was kept to less than 140/90 mmHg. A CT angiogram shortly after was suspicious for an AVM anterior and superior to the left thalamic hemorrhage with stable hydrocephalus despite EVD placement. The possible risk of rehemorrhage and subsequent death was discussed with the family but the decision was made to give IVT due to the high rate of mortality associated with the patient’s condition. Specifically, the large volume of blood in all four ventricles, obstructive hydrocephalus, and poor clinical status worsened her prognosis. We used 2 mg of recombinant tissue plasminogen activator (rt-PA) through the EVD, then flushed the EVD with 1 cc of normal saline, and clamped the EVD for 30 minutes. rt-PA was given twice daily for a total of five doses. Almost 14 hours after the first dose of rt-PA, repeat CT scan showed decreased ventriculomegaly and decreased blood in the right temporal horn with no evidence of new hemorrhage (Fig. 1D–F). After three doses, CT scan on post-bleed day (PBD) 2 showed decreased ventriculomegaly, and removal of blood from the lateral and fourth ventricles (Fig. 1G–I). The fifth and final dose of rt-PA was given almost 48 hours after the initial dose. Twelve hours later the CT scan showed near-resolution of the IVH with no evidence of new hemorrhage (Fig. 1J–L). Cerebral angiogram performed on PBD 6 confirmed a left Spetzler–Martin Grade 4 AVM (Fig. 2). Throughout its duration, the EVD remained patent and functioning. On PBD 10, the EVD was clamped but not tolerated due to subsequent increase in intracranial pressure. Finally, on PBD
13 the EVD was successfully removed without placement of a shunt. The patient’s hospitalization was complicated by pneumonia and Clostridium difficile, which were treated. She ultimately required a tracheostomy for prolonged ventilation. The patient was in the intensive care unit for 20 days and discharged to a skilled rehabilitation center on PBD 29. Since her hospitalization, her right-sided hemiparesis and dysphasia have improved.
3. Discussion AVM are estimated to be present in 0.1% of the population and have an annual rate of hemorrhage of 2% [1]. The high flow and high pressure nature of AVM, in addition to intranidal aneurysms, make these vascular connections prone to hemorrhage and pose a challenge in the treatment of IVH caused by an AVM. The high mortality rate in IVH can be attributed to the obstructive hydrocephalus, inflammatory effects of blood products, and direct mass effect of the hematoma [2]. The most immediate threat to life during IVH is obstructive hydrocephalus. Current management of obstructive hydrocephalus due to IVH revolves around placement of an EVD; however, EVD alone has been estimated to decrease mortality by only 20% with no improvement in functional outcomes [2,5]. EVD are not able to remove the thrombus without additional intervention and often become occluded, due to the large amount of coagulated blood. In addition, EVD do not hasten clot resolution nor decrease the inflammatory effects caused by blood [2]. Thus, therapies directed at removing ventricular blood have been investigated, with IVT having the most success. IVT have been found to decrease mortality and improve outcome in IVH, and are currently under further investigation in phase III clinical trials, the Clot Lysis: Evaluating Accelerated Resolution of Intraventricular Hemorrhage (CLEAR) trial; however the presence of an AVM has been an exclusion criterion in these studies. IVT increase the rate of clot dissolution, help maintain patency of the EVD, and minimize exposure of blood products to neural tissue [2,5]. This, in turn, helps relieve the obstruction causing hydrocephalus, control intracranial pressure, and shorten the duration of EVD use. The risk of rebleeding should certainly not be overlooked, as it can be a fatal complication of using IVT regardless of the etiology of the IVH. Rebleeding is of greater concern in the setting of an AVM because of its immense vascularity and high pressure [1]. In addition, unlike other causes of IVH, such as hypertension, coagul-
Fig. 2. Left internal carotid artery angiography, anterior–posterior (A) and lateral (B) views showing a Spetzler-Martin Grade 4 thalamic arteriovenous malformation. Shunting was from the posterior communicating artery, posterior cerebral artery, and lenticulostriate branches of the middle cerebral artery. The feeding vessels were the lenticulostriate, anterior thalamic perforators, and posterior choroidal vessels. It drained into the vein of Galen and straight sinus.
Case Reports / Journal of Clinical Neuroscience 21 (2014) 529–531
opathies, and aneurysms that may be acutely corrected, it is not always feasible or recommended to secure an AVM during an acute hemorrhage [1]. Initial data from the CLEAR trials suggests that IVT have an acceptable safety profile, with an estimated rebleeding rate of 8% when 1 mg of rt-PA is given every 8 hours. However, AVM have been an exclusion criterion in this study and a contraindication to thrombolytic use due to the risk of rebleeding [3]. Nevertheless, given the grim prognosis of IVH irrespective of etiology, the benefits of IVT may outweigh the risks in selected clinical situations. Our patient presented with panventricular hemorrhage, obstructive hydrocephalus, and a poor clinical state despite placement of an EVD. Without adjunctive use of IVT, the panventricular hemorrhage and hydrocephalus would be fatal. The compassionate use of IVT in this patient helped to rapidly clear the ventricular hematoma while maintaining a patent ventricular catheter. No rebleeding was noted throughout this treatment. Perhaps AVM should not be considered an absolute contraindication to IVT. Further evidence is needed to adequately assess the safety profile of IVT in this specific clinical scenario. 4. Conclusion IVT have been used successfully and safely to treat IVH secondary to an AVM as shown in this case report and a few others in the literature [4,6–8]. Despite the risk of rebleeding, IVT should be considered a reasonable adjunctive treatment due to the high rate of neurologic deterioration and death that may ensue with current standard of care. Further studies should be conducted to assess
529
the safety and efficacy of IVT in the setting of IVH caused by an AVM. Conflicts of interest/disclosure The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Zacharia BE, Vaughan KA, Jacoby A, et al. Management of ruptured brain arteriovenous malformations. Curr Atheroscler Rep 2012;14:335–42. [2] Staykov D, Bardutzky J, Huttner HB, et al. Intraventricular fibrinolysis for intracerebral hemorrhage with severe ventricular involvement. Neurocrit Care 2011;15:194–209. [3] Naff N, Williams MA, Keyl PM, et al. Low-dose recombinant tissue-type plasminogen activator enhances clot resolution in brain hemorrhage: the intraventricular hemorrhage thrombolysis trial. Stroke 2011;42:3009–16. [4] Goh KY, Poon WS. Recombinant tissue plasminogen activator for the treatment of spontaneous adult intraventricular hemorrhage. Surg Neurol 1998;50:526, 31 [discussion 531–2]. [5] Morgan T, Awad I, Keyl P, et al. Preliminary report of the clot lysis evaluating accelerated resolution of intraventricular hemorrhage (CLEAR-IVH) clinical trial. Acta Neurochir Suppl 2008;105:217–20. [6] Jorens PG, Menovsky TM, Voormolen MH, et al. Intraventricular thrombolysis for massive intraventricular hemorrhage due to periventricular arteriovenous malformations: no absolute contraindications as rescue therapy prior to surgical repair or embolization? Clin Neurol Neurosurg 2009;111:544–50. [7] Kumar K, Demeria DD, Verma A. Recombinant tissue plasminogen activator in the treatment of intraventricular hemorrhage secondary to periventricular arteriovenous malformation before surgery: case report. Neurosurgery 2003;52:964, 8 [discussion 968–9]. [8] Mayfrank L, Rohde V, Gilsbach JM. Fibrinolytic treatment of intraventricular haemorrhage preceding surgical repair of ruptured aneurysms and arteriovenous malformations. Br J Neurosurg 1999;13:128–31.
http://dx.doi.org/10.1016/j.jocn.2013.04.031
Pharyngo-occipital artery variant arising proximal to occluded internal carotid artery: The risk of an unnecessary endarterectomy José E. Cohen a,c,⇑, Ronen R. Leker b, J. Moshe Gomori c, Eyal Itshayek a a
Department of Neurosurgery, Hadassah-Hebrew University Medical Center, POB 12000, Jerusalem 91120, Israel Department of Neurology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel c Department of Radiology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel b
a r t i c l e
i n f o
Article history: Received 16 June 2013 Accepted 18 June 2013
Keywords: Anatomic variant Carotid endarterectomy Carotid stenosis Occipital artery Stent Stroke
a b s t r a c t Collateral branches originating from the cervical internal carotid artery (ICA) are rare but can have significant clinical and surgical implications. We present a case of pharyngo-occipital artery arising proximal from an occluded ICA that was missed and confused for severe stenosis of the ICA, leading to the misguided indication for carotid endarterectomy. Advanced preoperative studies allowed timely recognition of this anomaly and reconsideration of the therapeutic plan. We stress the importance of recognizing these variants by careful examination of multimodal pre-surgical exams. Awareness of these variants will allow a more precise diagnosis, and more appropriate management of patients with carotid artery disease. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Collateral branches originating from the cervical internal carotid artery (ICA) are rare and thus have scarcely been reported [1– ⇑ Corresponding author. Tel.: +972 2 677 7092; fax: +972 2 641 6281. E-mail address: [email protected] (J.E. Cohen).
3]. However, these vascular variants can have significant clinical and surgical implications [4–6]. Considering that surgical procedures involving the cervical carotid artery are frequently performed interventions, awareness of such anatomic variants and anomalies is essential for adequate diagnosis, therapeutic planning, and safety of interventions. We present a patient with their pharyngo-occipital artery (POA) arising proximal from an occluded
Case Reports / Journal of Clinical Neuroscience 21 (2014) 526–529
pressure around the aneurysmal wall can lead to aneurysm rupture [4]. Decompression of type I Chiari malformations can rapidly resolve abnormal CSF flow hydrodynamics, affecting the pressure equilibrium between the sides of the aneurysm wall significantly [1,2]. In our patient, SAH occurred during bony decompression before dural opening. One plausible explanation is that the significant CSF flow changes at the foramen magnum decreased the CSF pressure against the outer aneurysm wall to such an extent that the intra-aneurysmal pressure rapidly and significantly exceeded the inward pressure from outside the aneurysm. Two previous patients with remote undiagnosed aneurysm rupture have been reported. A remote undiagnosed anterior communicating artery aneurysm ruptured during transsphenoidal resection of a pituitary adenoma. After tumor removal, the arachnoid abruptly collapsed into the sella turcica, applying a traction force on the wall of the aneurysm similar to that which may have been created by the sudden large increase in caudal CSF flow in our patient. Unlike our patient, however, the authors could not exclude intraoperative trauma as a cause of aneurysm formation [3]. The other case coincided with subdural drain insertion after urgent evacuation of a chronic subdural hematoma via frontoparietal craniotomy. CSF drainage may have affected the CSF flow dynamics and aneurysm pressure equilibrium enough to cause rupture [4]. Although our case did not involve draining excessive CSF, the decompression may have caused excessive caudal flow of CSF to the spinal region, which may have precipitated aneurysm rupture. To our knowledge this is the first report of intraoperative rupture of an undiagnosed cerebral aneurysm immediately after dural
opening during Chiari decompression. Only one prior undiagnosed cerebral aneurysm rupture without the possibility of surgical trauma has been reported and that case also occurred when CSF hydrodynamics were affected by CSF drainage with a subdural drain. The significant and rapid changes of previously abnormal CSF physiology noted with decompression of type I Chiari malformation can provide a pressure gradient that may predispose to rupture of undiagnosed cerebral aneurysms.
Conflict of interest/disclosure The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
References [1] Sivaramakrishnan A, Alperin N, Surapaneni S, et al. Evaluating the effect of decompression surgery on cerebrospinal fluid flow and intracranial compliance in patients with chiari malformation with magnetic resonance imaging flow studies. Neurosurgery 2004;55:1344–50. [2] Dolar MT, Haughton VM, Iskandar BJ, et al. Effect of craniocervical decompression on peak CSF velocities in symptomatic patients with Chiari I malformation. AJNR Am J Neuroradiol 2004;25:142–5. [3] Tsuchida T, Tanaka R, Yokoyama M, et al. Rupture of anterior communicating artery aneurysm during transsphenoidal surgery for pituitary adenoma. Surg Neurol 1983;20:67–70. [4] Stefini R, Ghitti F, Bergomi R, et al. Uncommon presentation of ruptured intracranial aneurysm during surgical evacuation of chronic subdural hematoma: case report. Surg Neurol 2008;69:89–92. [5] Ferguson GG. Physical factors in the initiation, growth, and rupture of human intracranial saccular aneurysms. J Neurosurg 1972;37:666–77.
http://dx.doi.org/10.1016/j.jocn.2013.04.031
Intraventricular tissue plasminogen activator for intraventricular hemorrhage caused by an arteriovenous malformation Kelley Keefe ⇑, Meysam Kebriaei, Andrew Gard, Arun-Angelo Patil Division of Neurosurgery, Department of Surgery, 982035 University of Nebraska Medical Center, Omaha, NE 68198-2035, USA
a r t i c l e
i n f o
Article history: Received 21 December 2012 Accepted 19 April 2013
Keywords: Intracranial arteriovenous malformation Intracranial hemorrhage Intraventricular injections Thrombolytic therapy
a b s t r a c t The use of thrombolytics delivered through an external ventricular drain has improved outcomes in intraventricular hemorrhage, a disease with a poor prognosis; however, presence of an arteriovenous malformation is generally considered a contraindication to thrombolytic use. Due do the high mortality with the current standard of care, thrombolytics should be considered as an acceptable treatment option despite the presence of an arteriovenous malformation in certain clinical situations. We review the available literature and present an additional patient to make the case for the use of thrombolytics for intraventricular hemorrhage from an arteriovenous malformation. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Intracranial hemorrhage (ICH) is the most common and most dangerous presentation of arteriovenous malformations (AVM), as it has a 10–30% mortality rate [1]. Up to 30–50% of ICH will be complicated by extension of blood into the ventricular space, forming an intraventricular hemorrhage (IVH) [2]. When this occurs, the
⇑ Corresponding author. Tel.: +1 402 559 9605; fax: +1 402 559 7779. E-mail address: [email protected] (K. Keefe).
mortality drastically increases to 50–80%, which is related to the amount of blood in the ventricles and presence of blood in the third and fourth ventricles [3]. Thus, IVH necessitates urgent treatment to prevent neurological deterioration and death. The use of intraventricular thrombolytics (IVT) began in an effort to improve outcomes and has been successful [2–4]. In a recent meta-analysis by Staykov et al., IVT were found to decrease mortality in the setting of IVH from 53% to 16% compared to external ventricular drain (EVD) alone [2]. Despite this promising application of thrombolytics to treat IVH, the presence of an AVM is often considered an absolute con-
Case Reports / Journal of Clinical Neuroscience 21 (2014) 526–529
527
Fig. 1. Axial slices of four non-contrast CT scans at presentation (A–C), post bleed day (PBD) 1 after one dose of recombinant tissue plasminogen activator (rt-PA) (D–F), PBD 2 after three doses of rt-PA (G–I), and PBD 3 after the fifth and final dose of rt-PA (J–L). The left column illustrates the resolution of blood in the fourth ventricle. The fourth ventricle is filled with blood at presentation (A), and is stable 12 hours after the first rt-PA dose (D). After three doses there is significant resolution (G), and minimal blood left after five doses (J). The middle column shows that the thalamic hemorrhage remained stable with no evidence of rebleeding during the treatment period with rt-PA (E, H, K). It also shows the removal of blood from the frontal horns of the lateral ventricles (B, E, H, K). The right column depicts evacuation of the lateral ventricles, first in the right side (F) and then in the left (I). Finally after the last dose of rt-PA, there is layering of minimal residual blood in the occipital horns of the lateral ventricles (L).
528
Case Reports / Journal of Clinical Neuroscience 21 (2014) 526–529
traindication to thrombolytic use due to the risk of rebleeding [5]. A few case reports in the literature have documented successful use of IVT to treat IVH caused by an AVM [4,6–8]. In this paper, we present additional evidence and discuss the potential use of IVT for IVH despite the presence of an AVM. 2. Case report A 20-year-old woman, with a past medical history significant for a febrile seizure at 1 year of age, presented to the emergency department after suddenly collapsing. At presentation, the patient was found to be in a decerebrate posture with a dilated, nonreactive left pupil and a mid-position, non-reactive right pupil. Initial non-contrast CT scan showed a large ICH originating in the left thalamus with blood extending into all four ventricles and the subarachnoid space. The lateral ventricles were enlarged, suggestive of obstructive hydrocephalus (Fig. 1A–C). The patient was immediately intubated, an EVD was placed, levetiracetam and mannitol were started, and her blood pressure was kept to less than 140/90 mmHg. A CT angiogram shortly after was suspicious for an AVM anterior and superior to the left thalamic hemorrhage with stable hydrocephalus despite EVD placement. The possible risk of rehemorrhage and subsequent death was discussed with the family but the decision was made to give IVT due to the high rate of mortality associated with the patient’s condition. Specifically, the large volume of blood in all four ventricles, obstructive hydrocephalus, and poor clinical status worsened her prognosis. We used 2 mg of recombinant tissue plasminogen activator (rt-PA) through the EVD, then flushed the EVD with 1 cc of normal saline, and clamped the EVD for 30 minutes. rt-PA was given twice daily for a total of five doses. Almost 14 hours after the first dose of rt-PA, repeat CT scan showed decreased ventriculomegaly and decreased blood in the right temporal horn with no evidence of new hemorrhage (Fig. 1D–F). After three doses, CT scan on post-bleed day (PBD) 2 showed decreased ventriculomegaly, and removal of blood from the lateral and fourth ventricles (Fig. 1G–I). The fifth and final dose of rt-PA was given almost 48 hours after the initial dose. Twelve hours later the CT scan showed near-resolution of the IVH with no evidence of new hemorrhage (Fig. 1J–L). Cerebral angiogram performed on PBD 6 confirmed a left Spetzler–Martin Grade 4 AVM (Fig. 2). Throughout its duration, the EVD remained patent and functioning. On PBD 10, the EVD was clamped but not tolerated due to subsequent increase in intracranial pressure. Finally, on PBD
13 the EVD was successfully removed without placement of a shunt. The patient’s hospitalization was complicated by pneumonia and Clostridium difficile, which were treated. She ultimately required a tracheostomy for prolonged ventilation. The patient was in the intensive care unit for 20 days and discharged to a skilled rehabilitation center on PBD 29. Since her hospitalization, her right-sided hemiparesis and dysphasia have improved.
3. Discussion AVM are estimated to be present in 0.1% of the population and have an annual rate of hemorrhage of 2% [1]. The high flow and high pressure nature of AVM, in addition to intranidal aneurysms, make these vascular connections prone to hemorrhage and pose a challenge in the treatment of IVH caused by an AVM. The high mortality rate in IVH can be attributed to the obstructive hydrocephalus, inflammatory effects of blood products, and direct mass effect of the hematoma [2]. The most immediate threat to life during IVH is obstructive hydrocephalus. Current management of obstructive hydrocephalus due to IVH revolves around placement of an EVD; however, EVD alone has been estimated to decrease mortality by only 20% with no improvement in functional outcomes [2,5]. EVD are not able to remove the thrombus without additional intervention and often become occluded, due to the large amount of coagulated blood. In addition, EVD do not hasten clot resolution nor decrease the inflammatory effects caused by blood [2]. Thus, therapies directed at removing ventricular blood have been investigated, with IVT having the most success. IVT have been found to decrease mortality and improve outcome in IVH, and are currently under further investigation in phase III clinical trials, the Clot Lysis: Evaluating Accelerated Resolution of Intraventricular Hemorrhage (CLEAR) trial; however the presence of an AVM has been an exclusion criterion in these studies. IVT increase the rate of clot dissolution, help maintain patency of the EVD, and minimize exposure of blood products to neural tissue [2,5]. This, in turn, helps relieve the obstruction causing hydrocephalus, control intracranial pressure, and shorten the duration of EVD use. The risk of rebleeding should certainly not be overlooked, as it can be a fatal complication of using IVT regardless of the etiology of the IVH. Rebleeding is of greater concern in the setting of an AVM because of its immense vascularity and high pressure [1]. In addition, unlike other causes of IVH, such as hypertension, coagul-
Fig. 2. Left internal carotid artery angiography, anterior–posterior (A) and lateral (B) views showing a Spetzler-Martin Grade 4 thalamic arteriovenous malformation. Shunting was from the posterior communicating artery, posterior cerebral artery, and lenticulostriate branches of the middle cerebral artery. The feeding vessels were the lenticulostriate, anterior thalamic perforators, and posterior choroidal vessels. It drained into the vein of Galen and straight sinus.
Case Reports / Journal of Clinical Neuroscience 21 (2014) 529–531
opathies, and aneurysms that may be acutely corrected, it is not always feasible or recommended to secure an AVM during an acute hemorrhage [1]. Initial data from the CLEAR trials suggests that IVT have an acceptable safety profile, with an estimated rebleeding rate of 8% when 1 mg of rt-PA is given every 8 hours. However, AVM have been an exclusion criterion in this study and a contraindication to thrombolytic use due to the risk of rebleeding [3]. Nevertheless, given the grim prognosis of IVH irrespective of etiology, the benefits of IVT may outweigh the risks in selected clinical situations. Our patient presented with panventricular hemorrhage, obstructive hydrocephalus, and a poor clinical state despite placement of an EVD. Without adjunctive use of IVT, the panventricular hemorrhage and hydrocephalus would be fatal. The compassionate use of IVT in this patient helped to rapidly clear the ventricular hematoma while maintaining a patent ventricular catheter. No rebleeding was noted throughout this treatment. Perhaps AVM should not be considered an absolute contraindication to IVT. Further evidence is needed to adequately assess the safety profile of IVT in this specific clinical scenario. 4. Conclusion IVT have been used successfully and safely to treat IVH secondary to an AVM as shown in this case report and a few others in the literature [4,6–8]. Despite the risk of rebleeding, IVT should be considered a reasonable adjunctive treatment due to the high rate of neurologic deterioration and death that may ensue with current standard of care. Further studies should be conducted to assess
529
the safety and efficacy of IVT in the setting of IVH caused by an AVM. Conflicts of interest/disclosure The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Zacharia BE, Vaughan KA, Jacoby A, et al. Management of ruptured brain arteriovenous malformations. Curr Atheroscler Rep 2012;14:335–42. [2] Staykov D, Bardutzky J, Huttner HB, et al. Intraventricular fibrinolysis for intracerebral hemorrhage with severe ventricular involvement. Neurocrit Care 2011;15:194–209. [3] Naff N, Williams MA, Keyl PM, et al. Low-dose recombinant tissue-type plasminogen activator enhances clot resolution in brain hemorrhage: the intraventricular hemorrhage thrombolysis trial. Stroke 2011;42:3009–16. [4] Goh KY, Poon WS. Recombinant tissue plasminogen activator for the treatment of spontaneous adult intraventricular hemorrhage. Surg Neurol 1998;50:526, 31 [discussion 531–2]. [5] Morgan T, Awad I, Keyl P, et al. Preliminary report of the clot lysis evaluating accelerated resolution of intraventricular hemorrhage (CLEAR-IVH) clinical trial. Acta Neurochir Suppl 2008;105:217–20. [6] Jorens PG, Menovsky TM, Voormolen MH, et al. Intraventricular thrombolysis for massive intraventricular hemorrhage due to periventricular arteriovenous malformations: no absolute contraindications as rescue therapy prior to surgical repair or embolization? Clin Neurol Neurosurg 2009;111:544–50. [7] Kumar K, Demeria DD, Verma A. Recombinant tissue plasminogen activator in the treatment of intraventricular hemorrhage secondary to periventricular arteriovenous malformation before surgery: case report. Neurosurgery 2003;52:964, 8 [discussion 968–9]. [8] Mayfrank L, Rohde V, Gilsbach JM. Fibrinolytic treatment of intraventricular haemorrhage preceding surgical repair of ruptured aneurysms and arteriovenous malformations. Br J Neurosurg 1999;13:128–31.
http://dx.doi.org/10.1016/j.jocn.2013.04.031
Pharyngo-occipital artery variant arising proximal to occluded internal carotid artery: The risk of an unnecessary endarterectomy José E. Cohen a,c,⇑, Ronen R. Leker b, J. Moshe Gomori c, Eyal Itshayek a a
Department of Neurosurgery, Hadassah-Hebrew University Medical Center, POB 12000, Jerusalem 91120, Israel Department of Neurology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel c Department of Radiology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel b
a r t i c l e
i n f o
Article history: Received 16 June 2013 Accepted 18 June 2013
Keywords: Anatomic variant Carotid endarterectomy Carotid stenosis Occipital artery Stent Stroke
a b s t r a c t Collateral branches originating from the cervical internal carotid artery (ICA) are rare but can have significant clinical and surgical implications. We present a case of pharyngo-occipital artery arising proximal from an occluded ICA that was missed and confused for severe stenosis of the ICA, leading to the misguided indication for carotid endarterectomy. Advanced preoperative studies allowed timely recognition of this anomaly and reconsideration of the therapeutic plan. We stress the importance of recognizing these variants by careful examination of multimodal pre-surgical exams. Awareness of these variants will allow a more precise diagnosis, and more appropriate management of patients with carotid artery disease. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Collateral branches originating from the cervical internal carotid artery (ICA) are rare and thus have scarcely been reported [1– ⇑ Corresponding author. Tel.: +972 2 677 7092; fax: +972 2 641 6281. E-mail address: [email protected] (J.E. Cohen).
3]. However, these vascular variants can have significant clinical and surgical implications [4–6]. Considering that surgical procedures involving the cervical carotid artery are frequently performed interventions, awareness of such anatomic variants and anomalies is essential for adequate diagnosis, therapeutic planning, and safety of interventions. We present a patient with their pharyngo-occipital artery (POA) arising proximal from an occluded
