Emerg Radiol DOI 10.1007/s10140-014-1264-7
CASE REPORT
Can arteriovenous malformation prevent the diagnosis of brain death? Onur Ergun & Erdem Birgi & Idil Gunes Tatar & Mehmet Fevzi Oztekin & Baki Hekimoglu
Received: 21 July 2014 / Accepted: 15 August 2014 # American Society of Emergency Radiology 2014
Abstract We present a case of a 14-year-old boy with spontaneous intracranial hemorrhage that was referred to us to confirm the diagnosis of brain death with cerebral angiography. In the left carotid angiogram, there was no arterial flow above the craniovertebral junction. But in the right carotid angiogram, there was arterial flow up to the level of posterior communicating artery. Right posterior cerebral artery was filled with contrast medium via patent posterior communicating artery and later, an opacifying arteriovenous malformation (AVM) was detected which was also seen in the vertebral angiogram. Although the angiographic findings of the patient did not confirm the angiographic criteria for the diagnosis of brain death, it could not be also excluded because the only cerebral flow was the filling of the AVM and no other cerebral perfusion was detected. Keywords Angiography . Arteriovenous malformations . Brain death
Background Nontraumatic intracranial hemorrhages are uncommon in children and accepted as important causes of sudden and O. Ergun : E. Birgi (*) : I. G. Tatar : B. Hekimoglu Department of Radiology, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey e-mail: [email protected] M. F. Oztekin Department of Neurology, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey
unexplained death. Arteriovenous malformation (AVM) is one of the important reasons for spontaneous hemorrhage in childhood. Although brain death is a clinical diagnosis, confirmation by ancillary tests is often required in protocols. Four-vessel angiography is accepted as the golden standard. We present a case of a 14-year-old boy with spontaneous intracranial hemorrhage which was referred to us to confirm the diagnosis of brain death with cerebral angiography. In selective four-vessel angiography, although there was no cerebral perfusion, an opacifying cerebral AVM was detected and it created confusion in the diagnosis of brain death.
Case presentation A 14-year-old boy was presented to the emergency department with epileptic seizure. Initial examination showed that the patient had a Glasgow Coma Score of 4 and had extensor response to painful stimuli. The cranial CT revealed right parietal intraparenchymal and intraventricular hemorrhage. During follow-up, the clinical criteria of brain death were fulfilled at the emergency room. He developed a deep coma with no spontaneous breathing, no brain stem reflexes, and no spontaneous motor activity. The patient was referred to confirm the diagnosis of brain death with cerebral angiography. A cerebral angiography was performed via right femoral approach, with selective catheterization of both carotid and vertebral arteries by injecting iodinated contrast medium under high pressure. Bilateral external carotid arteries were
Emerg Radiol
patent as expected. In the left carotid angiogram, there was no arterial flow in the internal carotid artery above the craniovertebral junction (Fig. 1a, b). But in the right carotid angiogram, there was arterial flow up to the level of posterior communicating artery and right posterior cerebral artery was filled with contrast via patent posterior communicating artery (Fig. 2). Later, an AVM was opacified which was also detected in the vertebral angiogram (Fig. 3a, b). There was no
Fig. 2 In the right carotid angiogram, the arterial flow up to the level of posterior communicating artery in the right internal carotid artery (black thick arrow) could be seen. Right posterior cerebral artery was filled with contrast via patent posterior communicating artery (white arrow). An opacifying AVM could be seen (black thin arrow)
opacification in the circle of Willis or anterior and middle cerebral arteries in any selective angiograms.
Discussion
Fig. 1 In anteroposterior (a) and lateral (b) projections of the left carotid angiogram, no arterial flow in the internal carotid artery above the craniovertebral junction could be seen
Nontraumatic spontaneous intracranial hemorrhages are uncommon in children and accepted as important causes of sudden and unexplained death. It is generally secondary to underlying structural vascular abnormalities such as AVMs and aneurysms [1]. AVM is an abnormal connection of arteries and veins with the absence of connecting capillaries between them so the blood flow drains directly from arteries to venous system without perfusing the brain parenchyma. It is generally accepted as congenital but symptoms may occur at any age; mostly at 20–40 years. In a study of young patients presenting to the emergency department with spontaneous intraparenchymal hemorrhage, AVM was found as the most common pathology with a rate of 39.2 % among the patients with vascular etiologies [2]. The overall risk of bleeding of an AVM is about 2–4 % per year and re-bleeding within the first year is reported as 15–20 % [1]. The patients may present with brain hemorrhage, seizures, headache, or progressive neurological deficit. Cerebral AVMs are commonly presented with intracranial hemorrhage that is usually seen as parenchymal and/or intraventricular and is associated with 10 % mortality and 30 % morbidity [1]. The diagnosis of brain death is primarily considered to be clinical. Brain death determination consists of absent cerebral function, absent brainstem function, and apnea with well-
Emerg Radiol
Angiographic findings in brain death are no visualization of anterior circulation beyond level of supraclinoid internal carotid arteries, no posterior circulation filling beyond dural penetration of vertebral arteries, and absence of internal cerebral vein filling [4]. In brain death, intracranial perfusion is absent with the exception of an occasional opacification of the superior sagittal sinus [5]. In brain death, the major phenomenon is the complete arrest of cerebral perfusion due to increased intracranial pressure above the blood pressure that leads to the cytotoxic death of the cerebral neurons. However, rare case reports and studies demonstrated that there may be stasis of blood in the intracranial vessels in clinically apparent brain death [6, 7]. In such confusing conditions, as in our case, radionuclide imaging of the brain parenchyma with brain-specific lipophilic agent Tc99m HMPAO (hexamethyl propylene amine oxime) may support the diagnosis of brain death by demonstrating no parenchymal uptake [8]. We believe that the arteriovenous shunting prevented the complete cessation of blood flow in our case. It should be kept in mind that visualization of an arteriovenous malformation should not preclude the diagnosis of brain death in the absence of cerebral perfusion.
Conflict of interest The authors declare that they have no conflict of interest.
References
Fig. 3 In anteroposterior (a) and lateral (b) projections of the left vertebral angiogram, the opacifying AVM could be seen (black thin arrow). Black thick arrow Left vertebral artery. White arrow Basilar artery
defined etiology that is irreversible. Sometimes confounding factors make this clinical evaluation impossible so the ancillary tests become necessary [3]. Ancillary tests include cerebral angiography, radionuclide scanning, transcranial Doppler ultrasonography, and other imaging studies (MRI, CT) [4]. A four-vessel angiography is accepted as the golden standard.
1. Renowden S (2005) Interventional neuroradiology. J Neurol Neurosurg Psychiatry 76(3):48–63 2. Romero JM, Artunduaga M, Forero NP, Delgado J, Sarfaraz K, Goldstein JN et al (2009) Accuracy of CT angiography for the diagnosis of vascular abnormalities causing intraparenchymal hemorrhage in young patients. Emerg Radiol 16(3):195–201 3. Martin S, Alexis F, Jean-Luc G, François T, Stephan L (2012) Selective 4 vessels angiography in brain death: a retrospective study. Can J Neurol Sci 37:492–497 4. Manraj KSH, Navraj SH. Potential ancillary tests in the evaluation of brain death: the value of cerebral blood flow assessment. Canadian Council for Donation and Transplantation 5. Canadian Neurocritical Care Group (1999) Guidelines for the diagnosis of brain death. Can J Neurol Sci 26:64–66 6. Sawicki M, Bohatyrewicz R, Safranow K, Walecka A, Walecki J, Rowinski O et al (2013) Dynamic evaluation of stasis filling phenomenon with computed tomography in diagnosis of brain death. Neuroradiology 55(9):1061–1069 7. Ala TA, Kuhn MJ, Johnson AJ (2006) A case meeting clinical brain death criteria with residual cerebral perfusion. Am J Neuroradiol 27(9):1805–1806 8. Sinha P, Conrad GR (2012) Scintigraphic confirmation of brain death. Semin Nucl Med 42:27–32
CASE REPORT
Can arteriovenous malformation prevent the diagnosis of brain death? Onur Ergun & Erdem Birgi & Idil Gunes Tatar & Mehmet Fevzi Oztekin & Baki Hekimoglu
Received: 21 July 2014 / Accepted: 15 August 2014 # American Society of Emergency Radiology 2014
Abstract We present a case of a 14-year-old boy with spontaneous intracranial hemorrhage that was referred to us to confirm the diagnosis of brain death with cerebral angiography. In the left carotid angiogram, there was no arterial flow above the craniovertebral junction. But in the right carotid angiogram, there was arterial flow up to the level of posterior communicating artery. Right posterior cerebral artery was filled with contrast medium via patent posterior communicating artery and later, an opacifying arteriovenous malformation (AVM) was detected which was also seen in the vertebral angiogram. Although the angiographic findings of the patient did not confirm the angiographic criteria for the diagnosis of brain death, it could not be also excluded because the only cerebral flow was the filling of the AVM and no other cerebral perfusion was detected. Keywords Angiography . Arteriovenous malformations . Brain death
Background Nontraumatic intracranial hemorrhages are uncommon in children and accepted as important causes of sudden and O. Ergun : E. Birgi (*) : I. G. Tatar : B. Hekimoglu Department of Radiology, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey e-mail: [email protected] M. F. Oztekin Department of Neurology, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey
unexplained death. Arteriovenous malformation (AVM) is one of the important reasons for spontaneous hemorrhage in childhood. Although brain death is a clinical diagnosis, confirmation by ancillary tests is often required in protocols. Four-vessel angiography is accepted as the golden standard. We present a case of a 14-year-old boy with spontaneous intracranial hemorrhage which was referred to us to confirm the diagnosis of brain death with cerebral angiography. In selective four-vessel angiography, although there was no cerebral perfusion, an opacifying cerebral AVM was detected and it created confusion in the diagnosis of brain death.
Case presentation A 14-year-old boy was presented to the emergency department with epileptic seizure. Initial examination showed that the patient had a Glasgow Coma Score of 4 and had extensor response to painful stimuli. The cranial CT revealed right parietal intraparenchymal and intraventricular hemorrhage. During follow-up, the clinical criteria of brain death were fulfilled at the emergency room. He developed a deep coma with no spontaneous breathing, no brain stem reflexes, and no spontaneous motor activity. The patient was referred to confirm the diagnosis of brain death with cerebral angiography. A cerebral angiography was performed via right femoral approach, with selective catheterization of both carotid and vertebral arteries by injecting iodinated contrast medium under high pressure. Bilateral external carotid arteries were
Emerg Radiol
patent as expected. In the left carotid angiogram, there was no arterial flow in the internal carotid artery above the craniovertebral junction (Fig. 1a, b). But in the right carotid angiogram, there was arterial flow up to the level of posterior communicating artery and right posterior cerebral artery was filled with contrast via patent posterior communicating artery (Fig. 2). Later, an AVM was opacified which was also detected in the vertebral angiogram (Fig. 3a, b). There was no
Fig. 2 In the right carotid angiogram, the arterial flow up to the level of posterior communicating artery in the right internal carotid artery (black thick arrow) could be seen. Right posterior cerebral artery was filled with contrast via patent posterior communicating artery (white arrow). An opacifying AVM could be seen (black thin arrow)
opacification in the circle of Willis or anterior and middle cerebral arteries in any selective angiograms.
Discussion
Fig. 1 In anteroposterior (a) and lateral (b) projections of the left carotid angiogram, no arterial flow in the internal carotid artery above the craniovertebral junction could be seen
Nontraumatic spontaneous intracranial hemorrhages are uncommon in children and accepted as important causes of sudden and unexplained death. It is generally secondary to underlying structural vascular abnormalities such as AVMs and aneurysms [1]. AVM is an abnormal connection of arteries and veins with the absence of connecting capillaries between them so the blood flow drains directly from arteries to venous system without perfusing the brain parenchyma. It is generally accepted as congenital but symptoms may occur at any age; mostly at 20–40 years. In a study of young patients presenting to the emergency department with spontaneous intraparenchymal hemorrhage, AVM was found as the most common pathology with a rate of 39.2 % among the patients with vascular etiologies [2]. The overall risk of bleeding of an AVM is about 2–4 % per year and re-bleeding within the first year is reported as 15–20 % [1]. The patients may present with brain hemorrhage, seizures, headache, or progressive neurological deficit. Cerebral AVMs are commonly presented with intracranial hemorrhage that is usually seen as parenchymal and/or intraventricular and is associated with 10 % mortality and 30 % morbidity [1]. The diagnosis of brain death is primarily considered to be clinical. Brain death determination consists of absent cerebral function, absent brainstem function, and apnea with well-
Emerg Radiol
Angiographic findings in brain death are no visualization of anterior circulation beyond level of supraclinoid internal carotid arteries, no posterior circulation filling beyond dural penetration of vertebral arteries, and absence of internal cerebral vein filling [4]. In brain death, intracranial perfusion is absent with the exception of an occasional opacification of the superior sagittal sinus [5]. In brain death, the major phenomenon is the complete arrest of cerebral perfusion due to increased intracranial pressure above the blood pressure that leads to the cytotoxic death of the cerebral neurons. However, rare case reports and studies demonstrated that there may be stasis of blood in the intracranial vessels in clinically apparent brain death [6, 7]. In such confusing conditions, as in our case, radionuclide imaging of the brain parenchyma with brain-specific lipophilic agent Tc99m HMPAO (hexamethyl propylene amine oxime) may support the diagnosis of brain death by demonstrating no parenchymal uptake [8]. We believe that the arteriovenous shunting prevented the complete cessation of blood flow in our case. It should be kept in mind that visualization of an arteriovenous malformation should not preclude the diagnosis of brain death in the absence of cerebral perfusion.
Conflict of interest The authors declare that they have no conflict of interest.
References
Fig. 3 In anteroposterior (a) and lateral (b) projections of the left vertebral angiogram, the opacifying AVM could be seen (black thin arrow). Black thick arrow Left vertebral artery. White arrow Basilar artery
defined etiology that is irreversible. Sometimes confounding factors make this clinical evaluation impossible so the ancillary tests become necessary [3]. Ancillary tests include cerebral angiography, radionuclide scanning, transcranial Doppler ultrasonography, and other imaging studies (MRI, CT) [4]. A four-vessel angiography is accepted as the golden standard.
1. Renowden S (2005) Interventional neuroradiology. J Neurol Neurosurg Psychiatry 76(3):48–63 2. Romero JM, Artunduaga M, Forero NP, Delgado J, Sarfaraz K, Goldstein JN et al (2009) Accuracy of CT angiography for the diagnosis of vascular abnormalities causing intraparenchymal hemorrhage in young patients. Emerg Radiol 16(3):195–201 3. Martin S, Alexis F, Jean-Luc G, François T, Stephan L (2012) Selective 4 vessels angiography in brain death: a retrospective study. Can J Neurol Sci 37:492–497 4. Manraj KSH, Navraj SH. Potential ancillary tests in the evaluation of brain death: the value of cerebral blood flow assessment. Canadian Council for Donation and Transplantation 5. Canadian Neurocritical Care Group (1999) Guidelines for the diagnosis of brain death. Can J Neurol Sci 26:64–66 6. Sawicki M, Bohatyrewicz R, Safranow K, Walecka A, Walecki J, Rowinski O et al (2013) Dynamic evaluation of stasis filling phenomenon with computed tomography in diagnosis of brain death. Neuroradiology 55(9):1061–1069 7. Ala TA, Kuhn MJ, Johnson AJ (2006) A case meeting clinical brain death criteria with residual cerebral perfusion. Am J Neuroradiol 27(9):1805–1806 8. Sinha P, Conrad GR (2012) Scintigraphic confirmation of brain death. Semin Nucl Med 42:27–32
